44432 Federal Register / Vol. 71, No. 150 / Friday, August 4, 2006 / Proposed Rules 

SOCIAL SECURITY ADMINISTRATION 

20 CFR Part 404 

RIN 0960–AF33 

Revised Medical Criteria for Evaluating 

Immune System Disorders 

AGENCY: Social Security Administration. 

ACTION: Proposed rule. 

SUMMARY: We propose to revise the 

criteria in the Listing of Impairments 

(the listings) that we use to evaluate 

claims involving immune system 

disorders. We apply these criteria when 

you claim benefits based on disability 

under title II and title XVI of the Social 

Security Act (the Act). The proposed 

revisions reflect our adjudicative 

experience, as well as advances in 

medical knowledge, treatment, and 

methods of evaluating immune system 

disorders. 

DATES: To be sure your comments are 

considered, we must receive them by 

October 3, 2006. 

ADDRESSES: You may give us your 

comments by: using our Internet facility 

(i.e., Social Security Online) at http:// 

policy.ssa.gov/erm/rules.nsf/ 

Rules+Open+To+Comment or the 

Federal eRulemaking Portal at http:// 

www.regulations.gov; e-mail to 

regulations@ssa.gov; telefax to (410) 

966–2830; or, letter to the Commissioner 

of Social Security, P.O. Box 17703, 

Baltimore, MD 21235–7703. You may 

also deliver them to the Office of 

Regulations, Social Security 

Administration, 107 Altmeyer Building, 

6401 Security Boulevard, Baltimore, MD 

21235–6401, between 8 a.m. and 4:30 

p.m. on regular business days. 

Comments are posted on our Internet 

site, or you may inspect them physically 

on regular business days by making 

arrangements with the contact person 

shown in this preamble. 

FOR FURTHER INFORMATION CONTACT: Greg 

Zwitch, SSA Regulations Officer, Office 

of Regulations, Social Security 

Administration, 107 Altmeyer Building, 

6401 Security Boulevard, Baltimore, 

Maryland 21235–6401, (410) 965–1887 

or TTY (410) 966–5609. For information 

on eligibility or filing for benefits, call 

our national toll-free number, 1–800– 

772–1213 or TTY 1–800–325–0778, or 

visit our Internet Web site, Social 

Security Online, at http:// 

www.socialsecurity.gov/. 

SUPPLEMENTARY INFORMATION: Electronic 

Access: The electronic file of this 

document is available on the date of 

publication in the Federal Register at 

http://www.gpoaccess.gov/fr/ 

index.html. It is also available on the 

Internet site for SSA (i.e., Social 

Security Online) at http:// 

policy.ssa.gov/pnpublic.nsf/LawsRegs. 

What programs would these proposed 

regulations affect? 

These proposed regulations would 

affect disability determinations and 

decisions that we make for you under 

title II and title XVI of the Act. In 

addition, to the extent that Medicare 

entitlement and Medicaid eligibility are 

based on whether you qualify for 

disability benefits under title II and title 

XVI, these proposed regulations would 

also affect the Medicare and Medicaid 

programs. 

Who can get disability benefits? 

Under title II of the Act, we provide 

for the payment of disability benefits if 

you are disabled and belong to one of 

the following three groups: 

• Workers insured under the Act, 

• Children of insured workers, and 

• Widows, widowers, and surviving 

divorced spouses (see § 404.336) of 

insured workers. 

Under title XVI of the Act, we provide 

for Supplemental Security Income (SSI) 

payments on the basis of disability if 

you are disabled and have limited 

income and resources. 

How do we define disability? 

Under both the title II and title XVI 

programs, disability must be the result 

of any medically determinable physical 

or mental impairment or combination of 

impairments that is expected to result in 

death or which has lasted or is expected 

to last for a continuous period of at least 

12 months. Our definitions of disability 

are shown in the following table: 

If you file a claim under * * * And you are * * * Disability means you have a medically determinable impairment(s) 

as described above that results in * * * 

Title II .............................................. an adult or a child ........................... the inability to do any substantial gainful activity (SGA). 

Title XVI .......................................... a person age 18 or older ................ the inability to do any SGA. 

Title XVI .......................................... a person under age 18 ................... marked and severe functional limitations. 

What are the listings? 

The listings are examples of 

impairments that we consider severe 

enough to prevent you as an adult from 

doing any gainful activity. If you are a 

child seeking SSI payments based on 

disability, the listings describe 

impairments that we consider severe 

enough to result in ‘‘marked and severe 

functional limitations.’’ Although we 

publish the listings only in appendix 1 

to subpart P of part 404 of our rules, we 

incorporate them by reference in the SSI 

program in § 416.925 of our regulations, 

and apply them to claims under both 

title II and title XVI of the Act. 

How do we use the listings? 

The listings are in two parts. There 

are listings for adults (part A) and for 

children (part B). If you are a person age 

18 or over, we apply the listings in part 

A when we assess your claim, and we 

never use the listings in part B. 

If you are a person under age 18, we 

first use the criteria in part B of the 

listings. If the listings in part B do not 

apply, and if the specific disease 

process(es) has a similar effect on adults 

and children, we then use the criteria in 

part A. (See §§ 404.1525 and 416.925.) 

If your impairment(s) does not meet 

any listing, we will also consider 

whether it medically equals any listing; 

that is, whether it is as medically severe. 

(See §§ 404.1526 and 416.926.) 

We use the listings only to decide that 

you are disabled or that you are still 

disabled. We will never deny your claim 

or decide that you no longer qualify for 

benefits because your impairment(s) 

does not meet or medically equal a 

listing. If you have a severe 

impairment(s) that does not meet or 

medically equal any listing, we may still 

find you disabled based on other rules 

in the ‘‘sequential evaluation process’’ 

that we use to evaluate all disability 

claims. (See §§ 404.1520, 416.920, and 

416.924.) 

Also, when we conduct reviews to 

determine whether your disability 

continues, we will not find that your 

disability has ended based only on any 

changes in the listings. Our regulations 

explain that, when we change our 

listings, we continue to use our prior 

listings when we review your case, if 

you qualified for disability benefits or 

SSI payments based on our 

determination or decision that your 

impairment(s) met or medically equaled 

the listings. In these cases, we 

determine whether you have 

experienced medical improvement and, 


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if so, whether the medical improvement 

is related to the ability to work. If your 

condition(s) has medically improved so 

that you no longer meet or medically 

equal the prior listing, we evaluate your 

case further to determine whether you 

are currently disabled. We may find that 

you are currently disabled, depending 

on the full circumstances of your case. 

See §§ 404.1594(c)(3)(i) and 

416.994(b)(2)(iv)(A). If you are a child 

who is eligible for SSI payments, we 

follow a similar rule after we decide that 

you have experienced medical 

improvement in your condition(s). See 

§ 416.994a(b)(2). 

Why are we proposing to revise the 

listings for immune system disorders? 

We are proposing these revisions to 

update the listings and to provide more 

information about how we evaluate 

immune system disorders. We have not 

updated these rules since we first 

published them in 1993 (58 FR 36008). 

At that time, we established body 

system listings for immune system 

disorders in part A and part B. We made 

those rules effective for 5 years from the 

date of publication, unless we extended 

them, or revised and issued them again 

(58 FR at 36051). Since that time, we 

have extended the expiration date of the 

immune body system listings but we 

have not comprehensively revised them. 

We have, however, made several 

changes to these listings over the years. 

On November 19, 2001, we also 

published final rules in the Federal 

Register adding listings 14.09 and 

114.09, for inflammatory arthritis, to 

these body system listings, including 

introductory text to those listings in 

sections 14.00B6 and 114.00E (66 FR 

58009). We published minor technical 

changes to these body system listings on 

February 24, 2002 (67 FR 20018). 

How did we develop these proposed 

rules? 

These proposed rules reflect our 

adjudicative experience and advances in 

medical knowledge, treatment, and 

methods of evaluating immune system 

disorders. They also reflect comments 

we asked you to provide to help us 

develop the proposals. 

We published an Advance Notice of 

Proposed Rulemaking (ANPRM) in the 

Federal Register on May 9, 2003 (68 FR 

24896). The purpose of the ANPRM was 

to inform the public that we were 

planning to update and revise the rules 

we use to evaluate immune system 

disorders and to invite interested 

individuals and organizations to send us 

comments and suggestions for updating 

and revising the immune system 

listings. In the ANPRM, we provided a 

60-day period for comments and 

suggestions; that period ended on July 8, 

2003. We received over 200 letters and 

e-mails in response to the notice, many 

from individuals who have immune 

system disorders or who have family 

members with such disorders. We also 

received comments from medical 

experts, advocates, and people who 

adjudicate claims for us. Although we 

are not summarizing or responding to 

the comments in this notice, we read 

and considered them carefully and are 

proposing changes in our rules based on 

some of the suggestions we received. 

We also hosted policy conferences on 

‘‘Immune System Disorders in the 

Disability Programs’’ in Philadelphia, 

PA, on December 15, 2003, and in San 

Francisco, CA, on February 18 and 19, 

2004. At these conferences, we heard 

comments and suggestions for updating 

and revising these rules from 

individuals who have immune system 

disorders and their family members, 

physicians who treat individuals with 

immune system disorders, other 

professionals who work with people 

who have immune system disorders, 

advocates who represent individuals 

with immune system disorders, and 

individuals who make disability 

determinations and decisions for us in 

the State agencies and the Office of 

Hearings and Appeals. Several of the 

changes we propose in these rules are 

based on information we obtained at 

these conferences. 

When will we start to use these rules? 

We will not use these proposed rules 

until we evaluate the public comments 

we receive on them, determine whether 

they should be issued as final rules, and 

issue final rules in the Federal Register. 

If we publish final rules, we will 

explain in the preamble how we will 

apply them, and we will summarize and 

respond to the public comments. Until 

the effective date of any final rules, we 

will continue to use our current rules. 

How long would these proposed rules 

be effective? 

If we publish these proposed rules as 

final rules, they will remain in effect for 

8 years after the date they become 

effective, unless we extend them, or 

revise and issue them again. 

What revisions are we proposing to 

make? 

We are proposing to: 

• Expand and reorganize the 

introductory text in proposed 14.00 and 

114.00 to provide more guidance for our 

adjudicators, to update it, and to reflect 

the revised listings. 

• Add paragraph headings to the 

introductory text in proposed 14.00 and 

114.00 for easier reference. 

• Add proposed 14.00C and 114.00C 

to explain the meaning of key terms. 

• Remove all reference listings. 

Reference listings are listings that are 

met by satisfying the criteria of another 

listing. For example, current listing 

14.08G1 for human immunodeficiency 

virus (HIV) infection with anemia is a 

reference listing that requires evaluation 

under current listing 7.02 for chronic 

anemia. Therefore, it is redundant. 

Instead of using a reference listing, we 

propose to provide general guidance in 

the introductory text to the immune 

system listings (proposed 14.00J2g) 

stating that hematologic abnormalities, 

such as anemia, may be evaluated under 

7.00ff. In some cases, we are also 

replacing reference listings with new 

specific listing criteria for the 

impairments. For example, current 

listing 14.06, for undifferentiated 

connective tissue disorders, is entirely a 

reference listing. In the proposed rules, 

we are replacing the reference listing 

criterion with criteria that are specific to 

these disorders. 

• Add proposed listings 14.10 and 

114.10 for evaluating Sjoo ̈gren’s 

syndrome. 

• Add criteria to the listings, similar 

to those in current HIV infection listings 

14.08N and 114.08O, for each of the 

other listed immune system disorders 

(for example, systemic lupus 

erythematosus and systemic vasculitis). 

• Make nonsubstantive editorial 

changes to update the medical 

terminology in the introductory text and 

the listings and to make their language 

simpler and clearer. 

How are we proposing to change the 

introductory text to the adult immune 

system listings? 

We propose to expand and reorganize 

the introductory text to these listings. 

There are four major sections in current 

14.00, and the longest of those sections, 

14.00D, addresses only the evaluation of 

HIV infection. In these proposed rules, 

we add more sections and expand the 

guidance we provide about evaluating 

other kinds of immune system 

disorders. 

Some of the guidance in current 

14.00D is useful for evaluating other 

kinds of immune system disorders in 

addition to HIV infection. We are 

proposing to move that guidance from 

current 14.00D to new sections that 

would have more general applicability 

to immune system disorders. We are not 

proposing to remove any substantive 

guidance about how we evaluate HIV 

infection, only to reorganize some of the 


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information now in 14.00D of the 

current rules and to give it broader 

applicability where appropriate. We are 

also proposing to update and expand 

some of the guidance we provide for 

evaluating HIV infection and its effects, 

as we describe in more detail below. 

The four sections in the current rules 

are: 

• Current 14.00A, a short paragraph 

that describes generally the kinds of 

disorders we include in this body 

system. 

• Current 14.00B, a lengthy section 

that discusses the evaluation of 

connective tissue disorders; that is, 

autoimmune disorders. It includes six 

undesignated paragraphs that primarily 

explain the kinds of evidence we need 

to document the existence and severity 

of these disorders, including how we 

evaluate loss of function. These 

paragraphs are followed by six 

numbered sections that provide 

guidance about specific impairments in 

the listings. 

• Current 14.00C, a single sentence 

that explains that we evaluate allergic 

disorders under the appropriate listing 

of the affected body system. 

• Current 14.00D, a lengthy section 

that explains how we document the 

existence and severity of HIV infection, 

including how we evaluate loss of 

function under listing 14.08N. It 

includes eight numbered subsections 

and many paragraphs that are not 

designated with letters or numbers 

within those subsections. 

In the proposed rules, there are 10 

sections in the introductory text. The 

first three sections (proposed 14.00A, B, 

and C) provide general information 

about this body system, including 

definitions of terms. Each of the next 

three sections describes a particular 

category or type of immune system 

disorder: Autoimmune disorders 

(proposed 14.00D); immune deficiency 

disorders, excluding HIV infection 

(proposed 14.00E); and HIV infection 

(proposed 14.00F). The next three 

sections explain how we consider the 

effects of your treatment (proposed 

14.00G), your symptoms (proposed 

14.00H), and the functional limitations 

from your immune system disorder 

under these listings (proposed 14.00I). 

The last section, proposed section 

14.00J, explains how we consider the 

effects of your immune system disorder 

when it does not meet the requirements 

of one of the proposed immune system 

listings. We are designating all 

paragraphs in the proposed rules with 

letters or numbers to make it easier to 

refer to them. We are also providing 

headings for all of the major sections 

and many of the subsections. 

The following are the names of the 

major sections in proposed 14.00. We 

describe each section in detail later in 

this preamble. 

• Proposed 14.00A: What disorders 

do we evaluate under the immune 

system listings? 

• Proposed 14.00B: What information 

do we need to show that you have an 

immune system disorder? 

• Proposed 14.00C: Definitions 

• Proposed 14.00D: What are the 

listed autoimmune disorders in these 

listings? 

• Proposed 14.00E: How do we 

evaluate immune deficiency disorders, 

excluding HIV infection (14.07)? 

• Proposed 14.00F: How do we 

evaluate human immunodeficiency 

virus (HIV) infection? 

• Proposed 14.00G: How will we 

consider the effect of treatment in 

evaluating your autoimmune disorder, 

immune deficiency disorder, or HIV 

infection? 

• Proposed 14.00H: How do we 

consider your symptoms, including your 

constitutional symptoms or pain? 

• Proposed 14.00I: How do we use the 

functional criteria in these listings? 

• Proposed 14.00J: How do we 

evaluate your immune system disorder 

when it does not meet one of these 

listings? 

The following is a detailed 

description of the proposed changes in 

the introductory text of these proposed 

rules. 

14.00 Immune System Disorders 

We propose to change the name of 

this body system from ‘‘Immune 

System’’ to ‘‘Immune System Disorders’’

to more accurately reflect that we use 

these listings to evaluate immune 

system disorders in accordance with the 

requirements of the disability program. 

Proposed 14.00A—What disorders do 

we evaluate under the immune system 

listings? 

In proposed 14.00A, we provide a 

brief overview of this body system. We 

explain the kinds of disorders we 

evaluate under the immune system 

listings and that we organize these 

impairments under the categories of 

‘‘autoimmune disorders,’’ ‘‘immune 

deficiency disorders, excluding HIV 

infection,’’ and ‘‘HIV infection.’’

Proposed 14.00A has four subsections. 

We incorporate current 14.00A in the 

opening sentence of proposed 14.00A1. 

We propose to revise the sentence, 

which explains the kinds of immune 

system dysfunction that immune system 

disorders may cause, to update and 

simplify it. In proposed 14.00A1a and 

14.00A1b, we incorporate the first 

sentence in the sixth paragraph of 

current 14.00B to explain that immune 

system disorders can cause dysfunction 

in one or more components of the 

immune system, and describe ways in 

which immune system disorders may 

result in loss of function. In the second 

sentence of 14.001b, we propose to add 

‘‘involuntary’’ as a descriptor of weight 

loss to clarify that we mean weight loss 

due to an immune system disorder(s) or 

its treatment. We are adding 

‘‘involuntary’’ as a descriptor of weight 

loss throughout the introductory text in 

part A and part B for this same reason. 

Proposed 14.00A1c is a new paragraph 

that explains how we have organized 

immune system disorders in the preface 

(introductory text) of these listings. 

In proposed 14.00A2, Autoimmune 

disorders, we incorporate the first 

paragraph in current 14.00B to provide 

a brief description of autoimmune 

disorders. We propose to add an 

explanation that these disorders are 

sometimes referred to as ‘‘rheumatic 

diseases,’’ ‘‘connective tissue 

disorders,’’ or ‘‘collagen vascular 

disorders’’ and that some of the features 

of these disorders in adults differ from 

the features of the same disorders in 

children. We provide a cross-reference 

to proposed 14.00D, the section of the 

introductory text that addresses 

autoimmune disorders in detail. We also 

propose to remove the last sentence of 

the first paragraph of current 14.00B, 

which explains that connective tissue 

disorders generally evolve and persist 

over time, may result in functional loss, 

and may require long-term, repeated 

evaluation and management, because it 

does not provide useful adjudicative 

guidance. However, we do explain in 

proposed 14.00A1b that immune system 

disorders can cause limitation(s) that 

result in an ‘‘extreme’’ loss of function. 

Proposed 14.00A3, Immune 

deficiency disorders, excluding HIV 

infection, is new. We explain that these 

disorders can be classified as ‘‘primary’’

or ‘‘acquired,’’ are characterized by 

recurrent or unusual infections, and are 

associated with an increased risk of 

malignancies and of other autoimmune 

disorders. We also provide a crossreference to proposed 14.00E, the 

introductory section that addresses 

immune deficiency disorders in detail. 

In proposed 14.00A4, Human 

immunodeficiency virus (HIV) infection, 

we provide a brief description of HIV 

infection. We propose to move the first 

sentence in current 14.00D1 to this 

section. The sentence explains that HIV 

infection is caused by a specific 

retrovirus and may be characterized by 

increased susceptibility to opportunistic 

infections, cancers, or other conditions. 


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We also provide a cross-reference to 

proposed 14.00F, the section of the 

introductory text that addresses HIV 

infection in detail. 

Proposed 14.00B—What information do 

we need to show that you have an 

immune system disorder? 

In proposed 14.00B, we incorporate 

the first sentence of the second 

paragraph of current 14.00B to explain 

what information we need to show that 

you have an immune system disorder. 

We moved the second and third 

sentences of the second paragraph of 

current 14.00B, which define our term 

‘‘appropriate medically acceptable 

imaging,’’ to proposed 14.00C, a new 

section that provides definitions of 

terms in these listings. We propose to 

remove the last two sentences of the 

current paragraph. They explain that we 

will not purchase tests that may involve 

significant risk; however, we already 

include this general policy in 

§§ 404.1519m and 416.919m of our 

regulations so it is not necessary to 

repeat them in this section. 

In the second sentence of proposed 

14.00B, we provide that ‘‘we will make 

every reasonable effort’’ to obtain your 

medical history, medical findings, and 

the results of laboratory tests in 

documenting whether you have an 

immune system disorder. We include 

this requirement in current 14.00D, for 

HIV infection, but we do not include 

similar guidance in current 14.00B, for 

connective tissue disorders. We propose 

to add this guidance under proposed 

14.00B because it is appropriate for all 

immune system disorders. 

We also propose to remove the third 

and fourth paragraphs of current 14.00B. 

The third paragraph of current 14.00B 

provides that we need a longitudinal 

clinical record of at least 3 months 

demonstrating active disease to assess 

the severity and duration of your 

impairment. However, this is not always 

the case, even under the current rules. 

For example, individuals with HIV 

infection and cryptococcal meningitis 

(current listing 14.08B4) or Kaposi’s 

sarcoma (current listing 14.08B8), and 

individuals with ankylosing spondylitis 

with fixation (ankylosis) of the 

dorsolumbar spine at 45° (current listing 

14.09B2) are disabled based on those 

findings alone. In that case, we do not 

need 3 months of evidence or evidence 

showing active disease. Other cases may 

be decided with less than 3 months of 

evidence, while others may require 

more than 3 months of evidence. 

Therefore, we are removing this 

guidance because each case should be 

decided on an individual basis. 

Proposed 14.00C—Definitions 

In proposed 14.00C, we define what 

we mean by important terms in these 

listings. As already noted, we include 

the definition of ‘‘appropriate medically 

acceptable imaging’’ from the second 

paragraph of current 14.00B. However, 

we propose to replace the word 

‘‘proper’’ in the second sentence of this 

definition with the phrase ‘‘generally 

accepted and consistent with the 

prevailing state of medical knowledge 

and clinical practice’’ to more clearly 

explain what we mean. We also propose 

to include in this new section the 

definitions of the terms ‘‘severe’’ from 

the sixth paragraph of current 14.00B, 

‘‘inability to ambulate effectively’’ and 

‘‘inability to perform fine and gross 

movements effectively’’ from current 

14.00B6b, and ‘‘resistant to treatment,’’

‘‘recurrent,’’ and ‘‘disseminated’’ from 

the second, third, and fourth paragraphs 

of current 14.00D2. All of these terms 

will apply to several, and sometimes all, 

of the proposed listings in this body 

system. 

In proposed 14.00C, we do not 

include the phrase ‘‘must have lasted, or 

be expected to last, for at least 12 

months’’ from the definitions of 

‘‘inability to ambulate effectively’’ and 

‘‘inability to perform fine and gross 

movements effectively’’ in current 

14.00B6b because we believe it is 

unnecessary. Unless an impairment is 

expected to result in death, it must have 

lasted or must be expected to last for a 

continuous period of at least 12 months 

to meet the definition of disability. This 

proposed change would also make the 

definitions of the terms consistent with 

the definitions of the same terms in 

1.00B2b and 1.00B2c in the 

musculoskeletal body system. 

We also propose to move and simplify 

the definitions of the terms ‘‘resistant to 

treatment,’’ ‘‘recurrent,’’ and 

‘‘disseminated’’ in current 14.00D2, 

primarily to remove language that we 

believe is unnecessary. For example, we 

removed the explanation that the terms 

‘‘have the same general meaning as used 

by the medical community.’’ These 

changes are only editorial. We do not 

intend the proposed definitions to be 

substantively different from the current 

rules. 

In proposed 14.00C8, we reference 

current 1.00F for the definition of 

‘‘major peripheral joints’’ instead of 

restating the definition as we do in 

current 14.00B6a. We also propose to 

add the definitions of several other 

important terms in these listings, 

including in proposed 14.00C2, the term 

‘‘constitutional symptoms or signs.’’ In 

proposed 14.00C2, we also provide brief 

definitions for the constitutional 

symptoms ‘‘severe fatigue’’ and 

‘‘malaise.’’ We propose to add these 

definitions in response to the many 

comments we received that indicated 

that the fatigue and malaise that people 

who have immune system disorders 

experience can be very limiting. 

Proposed 14.00D—What are the listed 

autoimmune disorders in these listings? 

In proposed 14.00D, we incorporate 

and expand upon the information in 

current 14.00B1 through 14.00B6, which 

describe features commonly associated 

with each of the listed autoimmune 

system disorders. Throughout these 

sections, we refer to ‘‘autoimmune 

disorders’’ instead of ‘‘connective tissue 

disorders’’ because the phrase 

‘‘autoimmune disorders’’ is more 

medically accurate and more frequently 

used. We also propose to add a new 

section 14.00D7 for Sjo ̈gren’s syndrome 

because we are proposing to add new 

listing 14.10 for that autoimmune 

disorder. 

In proposed 14.00D1, Systemic lupus 

erythematosus (14.02), we expand and 

clarify the information in current 

14.00B1. In proposed 14.00D1a, 

General, we explain that systemic lupus 

erythematosus (SLE) may involve any 

organ or body system and describe by 

body system some potential 

manifestations that may be involved. 

We expand our explanation of how SLE 

is frequently characterized clinically 

and propose to change ‘‘fatigability’’

used in current 14.00B1 to ‘‘fatigue’’ to 

be consistent with how we describe this 

symptom throughout the immune 

system listings. We also add 

‘‘involuntary’’ as a descriptor of weight 

loss to clarify that we mean weight loss 

due to SLE or its treatment. In proposed 

14.00D1b, Documentation of SLE, we 

propose to update our rules to explain 

that your medical evidence will 

generally, but not always, show that 

your SLE satisfies the criteria in the 

‘‘Criteria for the Classification of 

Systemic Lupus Erythematosus’’ by the 

American College of Rheumatology, 

found in the most recent edition of the 

Primer on the Rheumatic Diseases 

published by the Arthritis Foundation. 

This is a more up-to-date reference than 

the 1982 reference in the current rules. 

In proposed 14.00D2, Systemic 

vasculitis (14.03), we clarify the 

information in the current rule. 

Proposed 14.00D2a, General, 

corresponds to the first three sentences 

of current 14.00B2. In it, we explain that 

vasculitis is an inflammation of blood 

vessels that may occur acutely in 

association with adverse drug reactions, 

certain chronic infections, and 


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occasionally malignancies, and that it 

may also be associated with other 

autoimmune disorders. We also give 

examples of several clinical patterns in 

which it may occur. We propose to 

remove the fourth sentence of current 

14.00B2, which describes cutaneous 

vasculitis, because the impairment 

varies greatly in its manifestation, may 

not be associated with systemic 

involvement, and would not be 

expected to result in a listing-level 

impairment. 

Proposed 14.00D2b, Documentation 

of systemic vasculitis, corresponds to 

the last two sentences of current 

14.00B2. In it, we describe 

documentation that we use to confirm 

the diagnosis of systemic vasculitis. 

Proposed 14.00D3, Systemic sclerosis 

(scleroderma) (14.04), corresponds to 

current 14.00B3. We propose to revise 

the heading and to expand the 

information in the section. Proposed 

14.00D3a, General, corresponds to the 

first three sentences of current 14.00B3. 

We propose to change the term 

‘‘Raynaud’s phenomena,’’ which we use 

in the second and third sentences of 

current 14.00B3, to ‘‘Raynaud’s 

phenomenon’’ because the latter is the 

correct term. We make this same change 

in proposed listing 14.04C. In proposed 

14.00D3b, Diffuse cutaneous systemic 

sclerosis, we continue to explain that, in 

addition to skin or blood vessels, major 

organ or systemic involvement may 

include the gastrointestinal tract, lungs, 

heart, kidneys, and muscle. This 

guidance corresponds to the fourth 

sentence in the current rule. 

Proposed 14.00D3c, Localized 

scleroderma (linear scleroderma or 

morphea), is new. We propose to add 

this section and appropriate listings in 

proposed 14.04 for these disorders that 

originate in childhood because their 

disabling effects can persist into 

adulthood. Proposed 14.00D3c is 

essentially the same as proposed 

114.00D3c, which we describe in detail 

later in this preamble. 

Proposed 14.00D3d, Documentation 

of systemic sclerosis (scleroderma), is 

also new. In it, we explain what 

documenting systemic sclerosis 

(scleroderma) involves and that there 

may be an overlap with other 

autoimmune disorders. 

In proposed 14.00D4, Polymyositis 

and dermatomyositis (14.05), we clarify 

the information in current 14.00B4. 

Proposed 14.00D4a, General, 

corresponds to the first three sentences 

of current 14.00B4. It describes the 

characteristics of polymyositis and 

dermatomyositis. In proposed 14.00D4b, 

Documentation of polymyositis or 

dermatomyositis, we describe the 

findings that are generally used to 

document these impairments. The first 

sentence of the proposed rule 

corresponds to the last sentence of 

current 14.00B4. We propose minor 

editorial revisions, including the 

removal of the reference to ‘‘myositis’’

because there are multiple characteristic 

abnormalities on muscle biopsy that 

support the diagnosis of polymyositis or 

dermatomyositis. We also propose to 

add a sentence to explain that people 

with dermatomyositis have a 

characteristic skin rash. 

In proposed 14.00D4c, Additional 

information about how we evaluate 

polymyositis and dermatomyositis 

under the listings, we explain how we 

evaluate commonly occurring 

limitations associated with these 

disorders. Proposed 14.00D4c(i) 

corresponds to the fourth and fifth 

sentences of current 14.00B4. We 

propose to delete the example of 

weakness of the anterior neck flexor 

muscles in the sixth sentence of current 

14.00B4 because we are proposing to 

delete the reference to the cervical 

muscles from listing 14.05 for reasons 

we explain later in this preamble. We 

also propose to add an example of 

squatting. Squatting is a common means 

for evaluating weakness in the pelvic 

girdle muscles. 

In proposed 14.00D4c(ii), we explain 

that we will evaluate malignancies 

(which may be associated with these 

disorders) under the malignant 

neoplastic diseases listings (13.00ff). We 

do not provide this guidance in 

proposed 114.00D4c in the childhood 

section for polymyositis or 

dermatomyositis because malignancies 

are not commonly associated with these 

disorders in children. We also explain 

that we evaluate the involvement of 

other organs or body systems under the 

affected body system. 

In proposed 14.00D5, 

Undifferentiated and mixed connective 

tissue disease (14.06), we reorganize and 

clarify the information in current 

14.00B5. In the proposed rules, we are 

adding an explicit reference to mixed 

connective tissue disease (MCTD) to 

clarify what we mean in the current 

rules when we refer to ‘‘overlap’’

syndromes. This is not a substantive 

change, but a clarification of our current 

rules to update medical terminology. In 

proposed 14.00D5a, General, we 

describe what we mean by 

undifferentiated and mixed connective 

tissue disease. In proposed 14.00D5b, 

Documentation of undifferentiated and 

mixed connective tissue disease, we 

explain when clinical features and 

serologic findings may be used to 

diagnose undifferentiated and mixed 

connective tissue disease. These 

provisions in proposed 14.00D5a and 

14.00D5b are not substantively different 

from the provisions in the first three 

sentences of current 14.00B5. 

We propose to delete the last sentence 

of current 14.00B5. The current 

sentence indicates that the correct 

designation of an ‘‘overlap’’ disorder is 

important for the assessment of 

prognosis. We believe that this sentence, 

while useful in treatment settings, does 

not provide useful adjudicative 

guidance. 

In proposed 14.00D6, Inflammatory 

arthritis (14.09), we expand, reorganize, 

and clarify the rules in current 14.00B6. 

Proposed 14.00D6a, General, 

corresponds to the first and fourth 

sentences of current 14.00B6. We 

continue to explain that inflammatory 

arthritides include a vast array of 

disorders that differ in cause, course, 

and outcome and may result in 

difficulties of ambulation or fine and 

gross movements. We edited the fourth 

sentence of current 14.00B6 to break it 

up into three shorter sentences. 

However, we do not intend to change 

the meaning of the provision. 

Proposed 14.00D6b, Inflammatory 

arthritides involving the axial spine 

(spondyloarthropathies), and 14.00D6c, 

Inflammatory arthritides involving the 

peripheral joints, correspond to the 

second and third sentences of current 

14.00B6. In these sections, we list some 

disorders that may be associated with 

inflammatory spondyloarthropathies 

involving the axial spine (proposed 

14.00D6b) and inflammatory arthritides 

affecting the peripheral joints (proposed 

14.00D6c). We propose to add 

inflammatory bowel disease (IBD) to the 

lists of examples in both sections 

because arthritis is the most common 

extra-intestinal complication of IBD. In 

proposed 14.00D6b, we remove the 

examples of ‘‘other reactive 

arthropathies’’ and ‘‘undifferentiated 

spondylitis’’ now included in the 

second sentence of current 14.00D6 

because they are non-specific and the 

list is not intended to be complete, only 

to provide some examples. Finally, we 

propose to update some of the 

terminology in this section; for example, 

we refer to ‘‘psoriatic arthritis’’ instead 

of ‘‘psoriatic arthropathy.’’

Proposed 14.00D6d, Documentation 

of inflammatory arthritides, is new. In 

it, we explain that generally, but not 

always, the diagnosis of inflammatory 

arthritis is made by the clinical features 

and serologic findings described in the 

most recent edition of the Primer on the 

Rheumatic Diseases. 

Proposed 14.00D6e, How we evaluate 

the inflammatory arthritides under the 


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listings, corresponds to the information 

in the last two sentences of current 

14.00B6, current 14.00B6c, and current 

14.00B6d. We are reorganizing the text 

to reflect the proposed reorganization of 

listing 14.09, which we explain later in 

this preamble, and to clarify it. 

• Proposed 14.00D6e(i) explains that 

proposed listings 14.09A and 14.09C1 

(current listings 14.09A and 14.09B) are 

met by showing an impairment that 

results in an ‘‘extreme limitation.’’ This 

is how we describe ‘‘inability to 

ambulate effectively’’ in 1.00B2b in our 

musculoskeletal listings and, therefore, 

would only be a clarification of the 

current rule. In the proposed rule, we 

retain the provision from current 

14.00B6c that the inability to ambulate 

effectively is implicit in proposed 

listing 14.09C1 (current listing 14.09B), 

the listing for ankylosis of the spine 

with fixation at a 45° angle, even though 

individuals who have the degree of 

ankylosis described in the listing 

ordinarily do not require the use of 

bilateral upper limb assistance. 

• Proposed 14.00D6e(ii) explains 

proposed listings 14.09B (current listing 

14.09D), 14.09C2 (current listing 

14.09E), and 14.09D. These listings do 

not describe a single impairment 

manifestation that results in an 

‘‘extreme’’ limitation. Rather, they 

describe combinations of impairment 

manifestations that should result in an 

‘‘extreme’’ limitation or in ‘‘marked’’

limitations in at least two areas of 

functioning. We also incorporate the 

provision in the first sentence of current 

14.00B6d that extra-articular 

impairments may meet listings in other 

body systems. 

• Proposed 14.00D6e(iii) corresponds 

to the third and fourth sentences of 

current 14.00B6d. It explains that extraarticular features of inflammatory 

arthritis may involve any body system 

and lists examples of commonly 

occurring extra-articular impairments by 

body system. We propose to reorganize 

and expand the list of examples of such 

impairments and to clarify the body 

systems to which they belong. 

• Proposed 14.00D6e(iv) and 

14.00D6e(v) correspond to the last 

sentence of current 14.00B6. In 

proposed 14.00D6e(iv), we replace 

‘‘persistent’’ with ‘‘permanent’’ and 

remove ‘‘without ongoing 

inflammation’’ to clarify that we 

evaluate permanent deformity of a major 

peripheral joint under listing 1.02 when 

it is the dominant feature of your 

impairment. Proposed 14.00D6e(v) 

explains that we use listing 1.03 to 

evaluate surgical reconstruction of a 

major weight-bearing joint. 

• Proposed 14.00D6e(vi) would 

clarify that we evaluate your 

impairment under any appropriate 

listing when you have both 

inflammation and chronic deformities. 

We are not including the provisions of 

current 14.00B6e in proposed 14.00D6. 

Current 14.00B6e provides that the fact 

that an individual is dependent on 

steroids, or any other drug, for the 

control of inflammatory arthritis is 

insufficient in itself to establish 

disability. We added it to part A of our 

listings in 2002 for consistency with 

114.00E6, a provision we added to part 

B of the listings at the same time (66 FR 

58010, 58020 (2001)). We are proposing 

to remove that provision for reasons we 

explain below in our summary of the 

proposed rules in part B. Therefore, we 

are proposing to remove this provision 

in part A for consistency with that 

change. However, in proposed 14.00G3, 

we continue to state that we will 

consider the adverse side effects of 

treatment, including the adverse effects 

of corticosteroids, to ensure that our 

adjudicators remember to consider the 

side effects an individual might 

experience from steroids and any other 

treatment. 

Proposed 14.00D7, Sjo ̈ gren’s 

syndrome (14.10), is new. As already 

noted, we are proposing to add a new 

listing for Sjo ̈gren’s syndrome. In 

connection with that proposed listing, 

proposed 14.00D7a, General, explains 

the features of the disorder, including 

its resulting symptoms and possible 

complications. We also list organ 

systems that may be involved and note 

that Sjo ̈gren’s syndrome may be 

associated with other autoimmune 

disorders. In proposed 14.00D7b, 

Documentation of Sjo ̈ gren’s syndrome, 

we also explain that if you have 

Sjo ̈gren’s syndrome, your medical 

evidence will generally, but not always, 

show that your disease satisfies the 

criteria in the ‘‘Criteria for the 

Classification of Sjo ̈gren’s Syndrome’’

found in the most recent edition of the 

Primer on the Rheumatic Diseases. 

Proposed 14.00E—How do we evaluate 

immune deficiency disorders, excluding 

HIV infection (14.07)? 

In proposed 14.00E, we add a new 

section describing how immune 

deficiency disorders (excluding HIV 

infection) are classified, documented, 

and evaluated. This section has four 

subsections. 

In proposed 14.00E1, General, we 

explain that immune deficiency 

disorders are classified as either 

‘‘primary’’ or ‘‘acquired.’’ Primary 

disorders are mainly seen in children 

but, due to recent advances in 

treatment, many affected children 

survive into adulthood. 

In proposed 14.00E2, Documentation 

of immune deficiency disorders, we 

explain that documentation of these 

disorders may be made by laboratory 

evidence or by other generally 

acceptable methods consistent with the 

prevailing state of medical knowledge 

and clinical practice. 

In proposed 14.00E3, Immune 

deficiency disorders treated by stem cell 

transplantation, we explain how we 

evaluate immune deficiency disorders 

that are treated in this way. In proposed 

14.00E3a, Evaluation in the first 12 

months, we explain that if you undergo 

stem cell transplantation we will 

consider you disabled until at least 12 

months from the date of the transplant. 

This is the same provision that we use 

for most malignancies treated by bone 

marrow or stem cell transplants in the 

neoplastic listings. In 13.00L4 of those 

listings, we also included a special 

provision for autologous bone marrow 

transplants—transplants using your own 

stem cells (69 FR 67034). We do not 

include such an alternative provision in 

these proposed rules because people 

with immune deficiency disorders 

receive allogeneic transplants—that is, 

stem cells taken from other people. 

Also, we propose to use ‘‘stem cell 

transplantation’’ instead of ‘‘bone 

marrow or stem cell transplantation’’ in 

this proposed section and in proposed 

listing 14.07B because ‘‘stem cell 

transplantation’’ is a broader term that 

encompasses different sites for 

obtaining hematopoetic (blood-forming) 

stem cells, including bone marrow, 

peripheral blood, and umbilical cord 

blood. In proposed 14.00E3b, 

Evaluation after the 12-month period 

has elapsed, we explain that, after that 

period has elapsed, we consider any 

demonstrable residuals of your immune 

deficiency disorder including any 

residual impairment(s) resulting from 

your treatment. The provision also is 

based on 13.00L4 in our malignant 

neoplastic diseases listings. 

Proposed 14.00E4, Medicationinduced immune suppression, is new. 

We explain that medication effects can 

result in immune suppression that will 

usually resolve once the medication is 

ceased. However, if you take prescribed 

medications for long-term immune 

suppression, such as after an organ 

transplant, we will look at the frequency 

and severity of any infections you get, 

residuals from the organ transplant 

itself, and whether there has been any 

significant deterioration of other organ 

systems. 


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Proposed 14.00F—How do we evaluate 

human immunodeficiency virus (HIV) 

infection? 

In proposed 14.00F, we incorporate, 

update, and expand information on HIV 

infection contained in current 14.00D3 

through 14.00D7. We also make 

nonsubstantive editorial changes. 

As already noted, we propose to move 

the first sentence of current 14.00D1 to 

proposed 14.00A4. Therefore, we begin 

proposed 14.00F with what is now the 

second sentence of current 14.00D1. It is 

a reminder that an individual with HIV 

infection need not meet the Centers for 

Disease Control definition of acquired 

immune deficiency syndrome (AIDS) to 

meet or medically equal the criteria of 

listing 14.08. We have made minor 

editorial changes to the sentence, but we 

do not intend to change its meaning. 

We propose to move the provisions of 

current 14.00D2 to other sections in the 

proposed rules. In the first four 

paragraphs of current 14.00D2, we 

define the terms ‘‘resistant to 

treatment,’’ ‘‘recurrent,’’ and 

‘‘disseminated,’’ and we would now 

define those terms in proposed 14.00C. 

In the fifth paragraph of current 

14.00D2, we define ‘‘significant 

involuntary weight loss’’ for purposes of 

current listing 14.08I (which has 

become listing 14.08H in these proposed 

rules). In the proposed rules, we include 

this definition in 14.00F5. 

Like current 14.00D3, proposed 

14.00F1 is in two major sections: A 

section explaining how we document 

the diagnosis of HIV infection 

definitively (14.00F1a) and a section 

explaining how we document the 

diagnosis of HIV infection when we do 

not have definitive evidence (14.00F1b). 

In proposed 14.00F1, Documentation of 

HIV infection, we incorporate and 

update the information in current 

14.00D3 to explain the laboratory tests 

or other evidence we accept as 

documentation of HIV infection. 

Proposed 14.00F1a, Documentation of 

HIV infection by definitive diagnosis, 

corresponds to current 14.00D3a. We 

propose to update and expand this 

section to include newer laboratory 

diagnostic techniques that did not exist 

or were not widely used when we 

published the current rules in 1993. 

• Proposed 14.00F1a(i), for HIV 

antibody tests, corresponds to current 

14.00D3a(i). We propose only 

nonsubstantive editorial changes. 

• Proposed 14.00F1a(ii) is new. It 

would add positive ‘‘viral load’’ tests for 

HIV infection, such as quantitative 

plasma HIV RNA, quantitative plasma 

HIV branched DNA, and reverse 

transcriptase-polymerase chain reaction 

(RT–PCR), that were not widely 

available when we published the 

current rules. 

• Proposed 14.00F1a(iii) is for HIV 

DNA detection by polymerase chain 

reaction (PCR). We include it as an 

example of an ‘‘other test’’ in current 

14.00D3a(iii) because it was not widely 

available when we published the 

current rules. 

• Proposed 14.00F1a(iv), for HIV 

antigen, corresponds to current 

14.00D3a(ii). 

• Proposed 14.00F1a(v) is new. It 

would add a positive viral culture for 

HIV from peripheral blood mononuclear 

cells (PBMC) as another test that 

definitively documents HIV infection. 

Even though it is not commonly used, 

we will accept it as definitive evidence 

if it is in your medical records. 

• Proposed 14.00F1a(vi), for other 

tests that are highly specific for 

detection of HIV, corresponds to the 

first paragraph in current 14.00D3a(iii). 

Proposed 14.00F1b, Other acceptable 

documentation of HIV infection, 

corresponds to current 14.00D3b. It 

explains what documentation of HIV 

infection we will accept instead of 

definitive laboratory testing. The 

proposed rule is essentially the same as 

the current rule except for 

nonsubstantive editorial changes. 

In proposed 14.00F2, CD4 tests, we 

combine the provisions in the second 

undesignated paragraph after current 

14.00D3a(iii) and the second paragraph 

in current 14.00D4a. We specify that, 

even though a reduced CD4 count or 

percent alone does not establish a 

definitive diagnosis of HIV infection, a 

CD4 count below 200/mm3 or 14 

percent along with clinical findings 

does offer supportive evidence of 

opportunistic infections without a 

definitive diagnosis. This is because a 

CD4 count below 200 or 14 percent is 

an indicator of an increased 

susceptibility to developing 

opportunistic infections. We also make 

nonsubstantive editorial changes. 

In proposed 14.00F3, Documentation 

of the manifestations of HIV infection, 

we incorporate the information in 

current 14.00D4 with nonsubstantive 

editorial changes. Like proposed 

14.00F1 and current 14.00D4, proposed 

14.00F3 is divided into two main parts. 

The first section explains how we 

document manifestation of HIV 

infection definitively (14.00F3a), and 

the second section explains how we 

document manifestations of HIV 

infection when we do not have 

definitive evidence (14.00F3b). 

Proposed 14.00F3a, Documentation of 

the manifestations of HIV by definitive 

diagnosis, incorporates the first 

paragraph in current 14.00D4a. 

In proposed 14.00F3b, Other 

acceptable documentation of the 

manifestations of HIV infection, we 

incorporate information that is in the 

first paragraph of current 14.00D4b. We 

propose to revise the language of this 

paragraph both editorially and to clarify 

our original intent. In the current rule, 

we indicate that ‘‘if no definitive 

laboratory evidence is available, 

manifestations of HIV infection may be 

documented by medical history, clinical 

and laboratory findings, and 

diagnosis(es) indicated in the medical 

evidence.’’ The sentence may imply that 

we need to have all of the things listed 

(medical history and clinical findings 

and laboratory findings and 

diagnosis(es)) to determine that you 

have a manifestation of HIV infection 

when we do not have definitive 

laboratory findings. That is not our 

intent, so we are clarifying in the 

proposed rule that we may need only 

some of this information to make a 

finding that you have a manifestation of 

HIV infection, depending on the 

prevailing state of medical knowledge 

and clinical practice. We also propose to 

clarify what we mean by ‘‘laboratory 

findings’’ in this context; that is, 

laboratory findings that do not in 

themselves definitively establish the 

existence of a diagnosis of an HIVrelated manifestation. 

In 14.00D4 of the current rules we 

provide specific guidance for 

documenting one particular 

manifestation of HIV infection without 

definitive evidence: cytomegalovirus 

(CMV) disease. In proposed 14.00F3b, 

we expand the section to include two 

additional manifestations. In proposed 

14.00F3b(i), we add guidance to explain 

that Pneumocystis carinii pneumonia 

(PCP) is frequently diagnosed 

presumptively without definitive 

evidence and to provide examples of 

evidence that is supportive of a 

presumptive diagnosis of PCP. We also 

note that Pneumocystis carinii is now 

known as Pneumocystis jiroveci; 

however, ‘‘PCP’’ remains in common 

usage for the pneumonia caused by this 

organism. 

In proposed 14.00F3b(ii), we 

incorporate and expand the information 

now in the second paragraph of current 

14.00D4b, regarding the documentation 

of CMV disease. We propose to clarify 

that a positive serology test for CMV 

identifies a ‘‘history’’ of infection but 

does not confirm an ‘‘active’’ disease 

process. We do not include 

‘‘documentation of CMV disease 

requires confirmation by biopsy’’ as in 

the last sentence of the second 


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Federal Register / Vol. 71, No. 150 / Friday, August 4, 2006 / Proposed Rules 44439 

paragraph of current 14.00D4 because 

we are providing information on 

documentation other than definitive 

laboratory findings. Also, instead of 

stating that we can use generally 

acceptable methods to confirm the 

diagnosis of CMV, we provide examples 

of evidence, such as fever and positive 

CMV serology test, that are supportive 

evidence of a presumptive diagnosis of 

CMV disease. 

In proposed 14.00F3b(iii), we add 

guidance on how toxoplasmosis of the 

brain is presumptively diagnosed since 

the definitive method of diagnosing 

toxoplasmosis of the brain by biopsy is 

not commonly performed. 

In proposed 14.00F4, Manifestations 

specific to women, we incorporate the 

information in current 14.00D5. In 

proposed 14.00F4a, General, we 

incorporate the first paragraph of 

current 14.00D5 and in proposed 

14.00F4b, Additional considerations for 

evaluating HIV infection in women, we 

incorporate the second paragraph of 

current 14.00D5. Except for adding 

paragraph designations and headings 

and minor editorial changes (including 

changes to reflect proposed changes in 

the paragraph designations of the 

listings explained below), the proposed 

provisions are the same as in the current 

rules. 

In proposed 14.00F5, involuntary 

weight loss, we incorporate the last 

paragraph of current 14.00D2 with 

nonsubstantive editorial changes, 

including a change that reflects our 

proposal to redesignate listing 14.08I to 

listing 14.08H. 

Proposed 14.00G—How will we 

consider the effect of treatment in 

evaluating your autoimmune disorder, 

immune deficiency disorder, or HIV 

infection? 

In the current rules, we refer to 

treatment and its effects in four places. 

• In the third paragraph of 14.00B, we 

provide that, for connective tissue 

diseases, we need a longitudinal clinical 

record of at least 3 months 

demonstrating active disease despite 

prescribed treatment, with the 

expectation that the disease will remain 

active for 12 months. 

• In the fifth paragraph of 14.00B, we 

explain that ‘‘the chronic adverse effects 

of treatment (e.g., corticosteroid-related 

ischemic necrosis of bone) may result in 

functional loss’’ in individuals with 

connective tissue disease. 

• In 14.00B6e, we explain that the 

fact that an individual with 

inflammatory arthritis is dependent on 

steroids or any other drug for the control 

of the arthritis is not in itself sufficient 

to establish that the individual is 

disabled. We also explain that we must 

evaluate each case on its own merits, 

taking into consideration any adverse 

effects of treatment. 

• In 14.00D7, Effect of treatment, we 

provide three paragraphs discussing 

how we consider treatment in people 

with HIV infection. This section 

explains that we must consider both the 

positive effects and negative side effects 

of treatment for HIV infection and its 

manifestations, special considerations 

in evaluating treatment in individuals 

with HIV infection and, briefly, the 

kinds of evidence we need. 

We are proposing to remove the 

provisions in the third paragraph of 

14.00B and paragraph 14.00B6e. Neither 

of those sections nor the other current 

rules we will continue to use contain 

provisions that explain in detail how we 

evaluate the positive effects and 

negative side effects of treatment in 

individuals who have autoimmune 

disorders and immune deficiency 

disorders apart from HIV infection. 

Also, most current treatments for HIV 

infection came into use, or came into 

wide use, after we first published listing 

14.08 in 1993. As a consequence, we 

believe that current 14.00D7 needs to be 

updated to reflect the newer and more 

widely used treatments and treatment 

protocols for HIV infection and to reflect 

the considerable medical experience 

that has been gained since 1993 about 

the long-term effects, usefulness, and 

limitations of such treatments. 

Therefore, we propose to add a new 

separate section 14.00G—How will we 

consider the effect of treatment in 

evaluating your autoimmune disorder, 

immune deficiency disorder, or HIV 

infection? The new section would 

address in one place issues of treatment 

that are common to all three types of 

immune system disorders as well as 

issues of treatment that are unique to 

each type of disorder, including 

treatment that is specifically for HIV 

infection. We do not propose to remove 

any guidance about treatment for HIV 

infection that is still relevant, only to 

move it to this new section. In fact, we 

propose to expand and update our rules 

to reflect what has been learned in 

applying different treatments for HIV 

infection since we published the current 

rules more than a decade ago. The 

provisions for addressing both the 

positive effects and negative side effects 

of treatment in individuals who have 

autoimmune disorders and immune 

deficiency disorders other than HIV 

infection would be new in these listings 

and, we believe, would provide useful 

adjudicative guidance that is lacking in 

our current rules. 

Section 14.00G has six subsections. 

The first two (proposed 14.00G1 and 

14.00G2) and the last one (proposed 

14.00G6) are applicable to all immune 

system disorders. Proposed 14.00G3–

14.00G5 provide guidance specific to 

each of the three main types of immune 

system disorders: Autoimmune 

disorders (proposed 14.00G3), immune 

deficiency disorders, excluding HIV 

infection (proposed 14.00G4), and HIV 

infection (proposed 14.00G5). 

In proposed 14.00G1, General, we 

incorporate the first and fifth sentences 

of current 14.00D7. We believe that this 

guidance has general applicability to all 

immune system disorders, not just HIV 

infection. We first explain that we 

consider both the effectiveness of your 

treatment on your signs, symptoms, and 

laboratory findings, and the negative 

side effects of your treatment on your 

functioning. We also explain that we 

will make every reasonable effort to 

obtain a specific description of the 

treatment you receive. Then, we list 

eight factors we consider when we 

evaluate your treatment. They are 

mostly based on factors we mention in 

the current rule, but we propose to 

expand the list and in some cases to 

clarify the existing factors in our current 

rules. For example, instead of referring 

only to the ‘‘dosage [and] frequency of 

administration’’ of your treatment, we 

refer to ‘‘the intrusiveness and 

complexity of your treatment (the 

dosing schedule, need for injections, 

etc).’’ In proposed 14.00G1e, we also 

introduce the term ‘‘variability of your 

response to treatment,’’ a concept we 

address for HIV infection in current 

14.00D7 but that we believe is of 

particular importance in considering the 

effects of treatment in all individuals 

with immune system disorders. We 

explain this concept in more detail in 

proposed 14.00G2. 

Proposed 14.00G1f is new. It 

describes the interactive and cumulative 

effects of treatments for immune system 

disorders and other disorders that 

people with immune system disorders 

may also have. We explain that the 

effects of these treatments taken together 

may be greater than they would be if we 

considered them separately, and we 

provide an example of treatment for HIV 

infection together with treatment for 

hepatitis C. Proposed 14.00G1g is also 

new. It explains that we will also 

consider the duration of your treatment. 

Proposed 14.00G1h is a catchall for 

other relevant factors we have not listed 

in 14.00G1a–14.00G1g. 

In proposed 14.00G2, Variability of 

your response to treatment, we explain 

what we mean by this factor in terms of 

both HIV infection and other immune 


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system disorders. This proposed rule is 

based on the language of the second 

paragraph in current 14.00D7 and the 

second sentence of the third paragraph 

of that section. However, we propose to 

expand that guidance and to apply it to 

all other immune system disorders in 

addition to HIV infection. For example, 

we explain in a general way applicable 

to all immune system disorders that 

some individuals may show an initial 

positive response to drug treatment (or 

a combination of drugs), but the initial 

positive response may be followed by a 

decrease in the effectiveness of the 

medication. 

We provide more specific information 

about treatment of autoimmune 

disorders in proposed 14.00G3, How we 

evaluate the effects of treatment for 

autoimmune disorders on your ability to 

function. This proposed rule repeats the 

rule in the fifth paragraph of current 

14.00B that, when we evaluate the 

effects of your treatment for your 

autoimmune disorder(s), we will 

consider the adverse effects that may 

result in loss of function. We propose to 

expand this guidance to include more 

examples of potential chronic adverse 

effects of steroid treatment and to 

explain that the side effects of some 

medications may be acute or long-term. 

We also propose to add a provision that 

recognizes that the medications used in 

the treatment of autoimmune disorders 

may have effects on mental function, 

including cognition (memory), 

concentration, and mood. 

Proposed 14.00G4, How we evaluate 

the effects of treatment for immune 

deficiency disorders, excluding HIV 

infection, on your ability to function, is 

new. As in proposed 14.00G3, we repeat 

the principle that we will consider the 

side effects of your treatment when we 

evaluate your ability to function. We 

cite intravenous immunoglobulin and 

gamma interferon therapy as examples 

of treatment you may be receiving. We 

also provide examples of side effects of 

treatment for immune deficiency 

disorders, including physical symptoms 

(such as fatigue and headaches), clinical 

signs (such as high blood pressure and 

joint swelling), and limitations in 

mental function, including cognition, 

concentration, and mood. 

Proposed 14.00G5, How we evaluate 

the effects of treatment for HIV infection 

on your ability to function, is in two 

parts. In proposed 14.00G5a, General, as 

in proposed 14.00G3 and 14.00G4, we 

repeat the principle from 14.00D7 that 

we consider the side effects of 

antiretroviral treatment and treatment 

for the manifestation of HIV infection on 

your ability to function. We propose to 

expand our guidance to provide 

examples of the physical and mental 

side effects of antiretroviral drugs. We 

also note that the symptoms of HIV 

infection and the side effects of 

medications may be indistinguishable, 

but that we will consider your 

functional limitations whether they are 

a result of your symptoms from HIV 

infection or the side effects of your 

treatment. 

In proposed 14.00G5b, Structured 

treatment interruptions, we provide new 

guidance specifically about structured 

treatment interruptions (STIs, also 

called drug holidays) in individuals 

with HIV infection. The proposed 

guidance clarifies that STIs are part of 

a prescribed treatment plan and do not 

show that an individual is failing to 

follow treatment, or in themselves 

establish that an individual’s 

impairment is not as severe as alleged. 

In proposed 14.00G6, When there is 

no record of ongoing treatment, we 

explain how we will evaluate the 

medical severity and duration of your 

immune system disorder when you have 

not received ongoing treatment or have 

not had an ongoing relationship with 

any treatment source despite the 

existence of a severe impairment(s). The 

provision is based on a standard 

provision we include in most other 

body systems listings, for example, 

1.00H3 in the musculoskeletal system, 

the third paragraph of 3.00A in the 

respiratory system, and the third 

paragraph of 4.00B3 in the 

cardiovascular system. We also explain 

that if you have just begun treatment 

and we cannot decide whether you are 

disabled based on the evidence we have, 

we may need to wait to determine the 

effect of your treatment. We explain that 

there is no set period because how long 

we may need to wait will depend on the 

facts of your individual case. This is 

consistent with the guidance we provide 

in the last sentence of the third 

paragraph in current 14.00D7, which 

explains we should decide the impact of 

treatment based on a sufficient period of 

treatment. 

Proposed 14.00H—How do we consider 

your symptoms, including your 

constitutional symptoms or pain? 

Proposed 14.00H is new. In it, we 

explain that we will evaluate the impact 

your symptoms have on your ability to 

function when the evidence of your 

immune system disorder(s) shows that 

you have a medically determinable 

impairment that could reasonably be 

expected to produce your symptoms. 

Proposed 14.00I—How do we use the 

functional criteria in these listings? 

Although we indicated in the ANPRM 

that we would not summarize or 

respond to the public comments (68 FR 

24897), there was one theme that was 

common to many of the letters and emails and that was raised repeatedly by 

the medical specialists, advocates for 

people who have immune system 

disorders, and individuals with immune 

system disorders in the presentations at 

the two outreach meetings we held: The 

functional impact of immune system 

disorders, and the inadequacy of the 

immune system rules to address that 

impact, especially for immune system 

disorders other than HIV infection. This 

issue was raised so often, and as a 

matter of such great public interest, that 

we believe that it will be helpful to 

summarize briefly what people said to 

help explain why we are proposing to 

add new rules for evaluating 

functioning in these listings. 

Many people said that we should 

recognize how immune system 

disorders can affect an individual’s 

functioning. Many people described 

physical symptoms, such as pain, 

fatigue, and malaise, as well as mental 

symptoms, including loss of memory, 

loss of concentration, and depression. 

Commenters stressed that these 

symptoms could be very severe. A 

number of people indicated that the 

fatigue associated with these disorders 

was not merely a feeling of tiredness but 

a more profound and debilitating 

experience. Many people also noted that 

the impairments could be both episodic 

and variable in intensity, with some 

people experiencing ‘‘good’’ or 

relatively good days interspersed with 

days in which they were unable to 

function. They pointed out that there 

was a need for the rules to recognize the 

longitudinal effect of these episodic 

limitations on the ability to work. Other 

people pointed out that there is often 

comorbidity of immune system 

disorders; that is, many people have 

features of more than one immune 

system disorder. In those cases, the 

symptoms and limitations are 

multiplied to an effect that is worse than 

simply adding them up. These 

commenters said that under the current 

listings there is no adequate way to 

assess these multiplied effects. Many 

people also pointed out the effect that 

stress can have on the medical 

condition and symptomatology of 

individuals who have immune system 

disorders. Other people described the 

debilitating effects of treatment, not 

only the side effects, but sometimes the 

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consuming schedule of treatment that in 

itself can be limiting. 

A number of the commenters pointed 

with approval to the provisions of 

current listing 14.08N and the text in 

current 14.00D8 that explains that 

listing. These individuals thought that 

the provisions should not be confined to 

people who have HIV infection but 

should be extended to people with other 

kinds of immune system disorders who 

may be continuously limited by their 

symptoms and other manifestations, 

frequently become ill, have periodic 

manifestations, or have the kinds of 

serious limitations described in those 

rules. They urged us to consider 

extending such criteria to all listed 

immune system disorders to ensure that 

we do not overlook individuals who do 

not necessarily have the objective 

evidence needed to meet the other 

criteria in the listings but who may still 

be disabled. 

We carefully considered these 

comments and are proposing a number 

of changes throughout the introductory 

text to the immune system listings to 

address them. We are proposing to 

significantly expand our guidance about 

specific immune system disorders and 

the effects of treatment. We agree with 

those commenters who suggested that 

we include the same kind of criteria for 

evaluating the overall functional impact 

of other immune system disorders as we 

provide in current listing 14.08N for 

people who have HIV infection. 

Therefore, we are proposing to add 

criteria similar to those in current listing 

14.08N for each of the listed 

impairments in this body system. The 

proposed listings for evaluating 

functioning for other immune system 

disorders would be 14.02B, 14.03B, 

14.04D, 14.05E, 14.06B, 14.07C, 14.09D, 

and 14.10B. We are also proposing to 

redesignate current listing 14.08N as 

14.08K for reasons we explain below. 

Proposed 14.00I is the section of the 

introductory text that would explain the 

proposed listings that include 

functional criteria. It corresponds to 

current 14.00D8, but we revised it so 

that it applies to all of the new proposed 

listings that include functional criteria, 

not just the listing for HIV infection 

(current listing 14.08N). 

Like current 14.00D8, proposed 14.00I 

includes eight paragraphs. Except as 

described below, we propose to revise 

each paragraph so that it applies not 

only to HIV infection but to the other 

immune system disorders as well. For 

example, in the first paragraph of 

current 14.00D8 we explain that current 

listing 14.08N (proposed listing 14.08K) 

establishes standards for evaluating 

manifestations of HIV infection that do 

not meet the criteria of any of the 

preceding listings within 14.08; that is, 

current listings 14.08A–14.08M. We also 

explain that we use listing 14.08N both 

for manifestations that are listed in the 

preceding listings within 14.08 and for 

manifestations that are not listed at all. 

We propose to modify this language so 

that it applies to all of the immune 

system disorders within this body 

system. We also propose minor editorial 

changes throughout the paragraphs. 

The following are other changes we 

propose to make in this section. 

In proposed 14.00I2, we propose to 

remove the first sentence in the second 

paragraph of current 14.00D8, which 

explains that for individuals with HIV 

infection, we assess listing-level severity 

under current listing 14.08N based on 

the functional limitations imposed by 

the impairment. We believe that this 

point is already made in proposed 

14.00I1 and that it is unnecessary to 

repeat it in proposed 14.00I2. We 

propose to revise the second sentence, 

which says that we must consider the 

full impact of ‘‘signs, symptoms, and 

laboratory findings’’ on the individual’s 

ability to function. We believe that this 

guidance may not clearly explain what 

we intend. Therefore, we propose to 

revise it to explain that when we use 

one of the listings cited in 14.00I1, we 

will consider all relevant information in 

your case record to determine the full 

impact of your immune system 

disorder(s) on your ability to function 

on a sustained basis. 

In proposed 14.00I3–14.00I8, which 

correspond to the last six paragraphs in 

current 14.00D, we propose to update 

our rules to make their language more 

consistent with our other rules that 

define the term ‘‘marked’’ and the 

domains of functioning. We do not 

intend these changes to be substantively 

different from the current rules. We also 

propose to include references to both 

pain and fatigue throughout proposed 

14.00I6–14.00I8 as symptoms that may 

cause limitations. The current rules are 

not consistent in this regard. 

Proposed 14.00J— How do we evaluate 

your immune system disorder when it 

does not meet one of these listings? 

Proposed 14.00J1 and 14.00J3 would 

replace the guidance we now provide in 

the first and third paragraphs of current 

14.00D6. As in other provisions 

throughout the introductory text, we 

propose to revise the language to make 

it apply generally to all immune system 

disorders, not just HIV infection. Also, 

we propose to remove guidance that is 

already covered in other sections in the 

introductory text of these proposed 

rules, such as the guidance that 

individuals may have signs or 

symptoms of a mental impairment or of 

another physical impairment. 

Proposed 14.00J2 would be a new 

section in this body system. For reasons 

we explain below, we are proposing to 

remove reference listings—that is, 

listings that are met or equaled by 

meeting or equaling the criteria of 

another listing—from this body system. 

However, immune system disorders can 

have effects in virtually every body 

system, and we believe it is important 

to include guidance about those effects 

in the introductory text so that they are 

not overlooked. 

Therefore, we propose to add new 

section 14.00J2 to explain that immune 

system disorders can have effects in 

other body systems; we also provide a 

list of examples of those effects in each 

of the relevant body systems with 

references to other body systems 

listings. The proposed provisions are 

based on language in the second 

paragraph of current 14.00D6, which is 

currently relevant only to the evaluation 

of HIV infection, and on the reference 

listings we are proposing to remove. In 

the latter case, we are also expanding 

the information to provide specific 

examples of impairments that may be 

caused by autoimmune disorders. 

For example, current listings 14.02A6 

and 14.04A4 are met with evidence of 

SLE, systemic sclerosis, or scleroderma 

with ‘‘Digestive involvement, as 

described under the criteria in 5.00ff.’’

Apart from the fact that these listings 

are unnecessary because any individual 

who meets the criteria of a listing in the 

digestive body system (5.00ff) would be 

disabled under that listing, the guidance 

is not very specific. Also, in the current 

rules, we include these criteria only 

under listing 14.02 and 14.04; however, 

other immune system disorders can 

have effects in the digestive system. 

Therefore, we provide in proposed 

14.00J2e that any immune system 

disorder can have effects in the 

digestive system, and we include an 

example of hepatitis C in addition to 

providing a reference to 5.00ff. 

Proposed 14.00J2k provides examples 

of allergic disorders (including skin 

disorders) that individuals with 

immune system disorders may have. It 

would replace current 14.00C. 

How are we proposing to change the 

criteria in the listings for evaluating 

immune system impairments in adults? 

14.01 Category of Impairments, 

Immune System Disorders 

The following is a detailed 

explanation of the significant changes in 

the proposed listings. Some changes are 


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common to several listings so we 

describe them first. 

1. We propose to remove all of the 

reference listings from this body system. 

Every current listing section in this 

body system, except listing 14.07, 

includes reference listings. Reference 

listings are listings that are met by 

satisfying the criteria of another listing. 

For example, current listing 14.02A1, 

Joint involvement, is met when the 

resulting impairment meets the criteria 

of any appropriate listing in the 

musculoskeletal body system, 1.00ff. 

Current listing 14.08G1, for HIV 

infection with anemia, requires 

evaluation under current listing 7.02. 

Therefore, these listings are redundant 

because impairments that meet these 

listings must meet the requirements of 

other listings. We are removing 

reference listings from all of the body 

systems as we revise them. As already 

noted, instead of using reference 

listings, we propose to provide guidance 

in 14.00J of the introductory text stating 

that we may evaluate the resulting 

impairment of an immune system 

disorder under any affected body 

system. 

2. We propose to revise current 

listings 14.02B, 14.03B, 14.04B, and 

14.09D (proposed listings 14.02A, 

14.03A, 14.04A, and 14.09B) as follows: 

• We propose to remove the criterion 

for ‘‘significant, documented’’

constitutional symptoms or signs in 

each of these listings because we define 

the constitutional symptoms and signs 

in proposed 14.00C2. Moreover, it is 

unnecessary to specify ‘‘documented’’

because we always need to document 

the existence of any symptom or sign in 

any disability claim. 

• Each of these current listings, 

except current listing 14.09D, also 

requires you to have all four of the 

constitutional symptoms or signs: 

Severe fatigue, fever, malaise, and 

involuntary weight loss. We propose to 

revise this requirement to ‘‘at least two’’

of the constitutional symptoms or signs 

instead of all four, because we believe 

that the requirement in the current 

listing is too severe. We believe that any 

individual with an autoimmune 

disorder involving two or more organs/ 

body systems with one organ/body 

system involved to at least a moderate 

level of severity and who has at least 

two of the constitutional symptoms and 

signs in these listings will have an 

impairment that precludes any gainful 

activity. We also have added 

‘‘involuntary’’ as a descriptor of weight 

loss in proposed listings 14.02A, 

14.03A, 14.04A, 14.05E, 14.06A, 14.07C, 

14.08K, 14.09B, and 14.10A for the same 

reason we explained earlier in the 

preamble. 

• In proposed listings 14.02A, 

14.03A, and 14.04A, which correspond 

to current listings 14.02B, 14.03B, and 

14.04B, we propose to remove the 

reference to ‘‘lesser involvement’’

because we propose to remove the 

current reference listings to which these 

rules refer. We also believe the phrase 

is unnecessary—the severity of the 

impairment is demonstrated by the 

remaining criteria. 

3. As we have already noted under the 

explanation of proposed 14.00I, we 

propose to add listings based on 

repeated manifestations accompanied 

by functional limitations and modeled 

after current listing 14.08N for each of 

the other immune system disorders. The 

proposed new listings are 

• 14.02B for SLE, 

• 14.03B for systemic vasculitis, 

• 14.04D for systemic sclerosis 

(scleroderma), 

• 14.05E for polymyositis and 

dermatomyositis, 

• 14.06B for undifferentiated and 

mixed connective tissue disease, 

• 14.07C for immune deficiency 

disorders (other than HIV infection), 

• 14.09D for inflammatory arthritides, 

and 

• 14.10B for Sjo ̈gren’s syndrome. 

Each listing requires you to have: 

• The specified immune system 

disorder for that listing, 

• Repeated manifestations that do not 

satisfy the requisite findings of another 

listing for the specified immune system 

disorder, 

• At least two of the constitutional 

symptoms or signs, and 

• ‘‘Marked’’ limitation in one of three 

domains of functioning: Activities of 

daily living, social functioning, or 

completing tasks in a timely manner 

due to deficiencies in concentration, 

persistence, or pace. 

We explain what we mean by 

‘‘repeated’’ in proposed 14.00I3 and by 

‘‘marked’’ in proposed 14.00I4–5. 

The following is an explanation of the 

other significant changes we propose to 

make. We are also proposing minor 

editorial changes in some listings and 

changes to cross-references to the 

introductory text throughout the listings 

to reflect the changes to the introductory 

text in the proposed rules. We do not 

describe all of those changes below. 

Proposed Listing 14.04—Systemic 

sclerosis (scleroderma) 

Proposed listing 14.04B corresponds 

to current listing 14.04C. As we have 

already noted, we propose to expand 

this listing to include provisions for 

individuals who had a childhood form 

of the disorder as children and who still 

have listing-level functional limitations 

as adults. The proposed listing is 

essentially identical to proposed listing 

114.04, which we describe in detail later 

in this preamble, except that it includes 

references to appropriate adult rules 

defining ‘‘inability to ambulate 

effectively’’ and ‘‘inability to perform 

fine and gross movements effectively.’’

We also propose minor clarifications 

in the language of the current listing. 

Current listing 14.04C describes 

‘‘[g]eneralized scleroderma with digital 

contractures.’’ We propose to clarify that 

‘‘digital’’ refers to either the toes or the 

fingers, and to list the effects in the toes 

separately from the effects in the fingers, 

in proposed listings 14.04B1 and 

14.04B2, respectively. We also propose 

to remove the requirement for 

‘‘generalized’’ scleroderma (that is, 

systemic sclerosis) because the very 

serious digital contractures described in 

the proposed listings would in 

themselves be disabling regardless of 

whether the scleroderma is generalized. 

Proposed listing 14.04C corresponds 

to current listing 14.04D. We propose to 

change ‘‘Raynaud’s phenomena’’ in 

current listing 14.04D to ‘‘Raynaud’s 

phenomenon’’ for the same reason 

already described in the explanation of 

proposed 14.00D3. We propose to 

remove the word ‘‘[s]evere’’ as a 

descriptor of Raynaud’s phenomenon in 

this listing because it is unnecessary 

given the severity of the impairment 

demonstrated by the remaining criteria, 

such as ischemia with ulcerations of 

fingers or toes, resulting in the inability 

to ambulate effectively or to perform 

fine and gross movements effectively. 

As in proposed listing 14.04B, we also 

propose to clarify that ‘‘digital’’ refers to 

fingers or toes. 

In proposed listing 14.04C, we also 

propose to revise the criteria in current 

listing 14.04D to provide a better 

description of listing-level Raynaud’s 

phenomenon. The criteria in current 

listing 14.04D require severe Raynaud’s 

phenomenon that is characterized by 

digital ulcerations, ischemia, or 

gangrene. We believe that this does not 

describe an impairment that precludes 

any gainful activity in every case. 

Therefore, in proposed listing 14.04C1 

we would provide criteria for Raynaud’s 

phenomenon characterized by gangrene 

of a toe or finger in at least two 

extremities, or a toe and finger to 

indicate an impairment that would 

preclude any gainful activity. We do not 

propose to require that the gangrene 

result in the inability to ambulate 

effectively or to perform fine and gross 

movements effectively because the 

presence of gangrene of a toe or finger 


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in at least two extremities or in a toe and 

finger by itself is an indication of a very 

serious impairment. In proposed listing 

14.04C2, we provide criteria for 

ischemia with ulcerations of the toes or 

fingers that results in the inability to 

ambulate effectively or to perform fine 

and gross movements effectively; 

Raynaud’s phenomenon characterized 

only by ischemia with ulcerations does 

not by itself describe an impairment that 

would necessarily result in an extreme 

loss of function. Also, ulcerations are an 

outcome of ischemia, so we propose to 

revise the language so that ischemia and 

ulcerations are not listed as though they 

are separate entities, as in the current 

rule. 

Proposed Listing 14.05—Polymyositis 

and Dermatomyositis 

Proposed listing 14.05A corresponds 

to current listing 14.05A. We propose to 

replace the word ‘‘severe’’ as a 

descriptor of proximal limb-girdle 

weakness with the more accurate 

‘‘resulting in inability to ambulate 

effectively or inability to perform fine 

and gross movements effectively, as 

defined in 14.00C6 and 14.00C7.’’ We 

also propose to change ‘‘shoulder and/ 

or pelvic’’ muscle weakness to ‘‘pelvic 

or shoulder’’ muscle weakness because 

pelvic muscle weakness can result in 

the inability to ambulate effectively and 

shoulder muscle weakness can result in 

the inability to perform fine and gross 

movements effectively. Therefore, either 

one of these findings could be sufficient 

in itself to show disability and the 

‘‘and’’ is unnecessary. 

Proposed listing 14.05B corresponds 

to current listing 14.05B1. We propose 

to remove the requirements in the 

opening paragraph for less severe limbgirdle muscle weakness than in 14.05A, 

associated with cervical muscle 

weakness, because impaired swallowing 

or impaired respiration may result in 

listing-level limitations without the 

presence of either of those findings. We 

also propose to remove the phrase ‘‘to 

at least a moderate level of severity’’

because the criterion in proposed 

14.05B is of at least a moderate level of 

severity, making this language 

unnecessary. We propose to revise 

‘‘impaired swallowing with dysphagia’’

to ‘‘impaired swallowing (dysphagia)’’

because dysphagia means impaired 

swallowing. We propose to revise 

‘‘episodes of aspiration’’ to ‘‘aspiration’’

because of the progressive nature of 

muscle weakness that results from 

polymyositis or dermatomyositis. Once 

an episode of aspiration is documented, 

further documentation of multiple 

episodes is unnecessary. In addition, we 

propose to replace ‘‘cricopharyngeal 

weakness’’ with ‘‘muscle weakness’’ in 

proposed 14.05B because impaired 

swallowing with dysphagia and 

aspiration may result from muscles 

other than the cricopharyngeal muscles. 

Proposed listing 14.05C corresponds 

to current listing 14.05B2. We propose 

to remove the requirements in the 

opening paragraph of current 14.05B2 

for the same reasons as in the above 

paragraph for proposed listing 14.05B. 

Proposed listing 14.05D, Diffuse 

calcinosis, is a new adult listing and has 

the same criteria as in proposed listing 

114.05D for children, which we describe 

in detail later in this preamble. We 

propose to add this listing for 

individuals who had a form of the 

disorder as children and who still have 

listing-level functional limitations as 

adults. 

Proposed Listing 14.06—

Undifferentiated and Mixed Connective 

Tissue Disease 

We propose to change the heading of 

current 14.06 to update it and to more 

accurately describe the disorders we 

evaluate under this listing. 

Current listing 14.06 is entirely a 

reference listing, requiring evaluation 

under current listings 14.02A, 14.02B, 

or 14.04. We propose to change it to a 

stand-alone listing containing its own 

criteria. Proposed listing 14.06A uses 

the same criteria as in proposed listings 

14.02A, 14.03A, and 14.04A for 

involvement of two or more body 

systems to at least a moderate level of 

severity and at least two constitutional 

symptoms or signs. Proposed listing 

14.06B incorporates the same functional 

criteria for the evaluation of repeated 

manifestations of undifferentiated and 

mixed connective tissue disease as the 

other listings in this body system. 

Proposed Listing 14.07—Immune 

Deficiency Disorders, Excluding HIV 

Infection 

We propose to change the heading of 

listing 14.07 to update its terminology 

and to more accurately describe the 

disorders we evaluate under this listing. 

The current listing is met with 

documented, recurrent severe infections 

occurring three or more times within a 

5-month period. We propose to replace 

this criterion with a new, more accurate, 

and up-to-date listing. The listing is in 

three parts. 

Proposed listing 14.07A is essentially 

the same as current listing 14.08M 

(proposed listing 14.08J) which 

describes individuals with HIV 

infection whose immune systems are so 

compromised that they frequently 

become ill. However, unlike current 

listing 14.07, current listing 14.08M 

provides that the infections must occur 

three times in a 12-month period, not 

three times in only a 5-month period. 

Current listing 14.08M is also more 

precise. It explains how severe the 

infections need to be by reference to 

resistance to treatment or a requirement 

for hospitalization or intravenous 

treatment. It also specifies six types of 

infections. We believe that the criteria 

in current listing 14.08M for people 

with HIV infection are equally as 

applicable to individuals with other 

kinds of immune deficiency disorders, 

and that they would be more inclusive 

than the criteria in current listing 14.07. 

Proposed listing 14.07B is new. We 

propose to add this listing to recognize 

that some immune system disorders are 

treated by stem cell transplantation. In 

proposed listing 14.07B, we state that 

we will consider you under a disability 

until at least 12 months from the date 

of transplantation and, thereafter, 

evaluate any residual impairment(s) 

under the criteria for the affected body 

system. 

Proposed listing 14.07C would 

incorporate the same functional criteria 

for the evaluation of repeated 

manifestations of immune deficiency 

disorders (excluding HIV infection) as 

in the other proposed listings in this 

body system and for the same reasons as 

described above. 

Proposed Listing 14.08—Human 

Immunodeficiency Virus (HIV) Infection 

We do not propose any substantive 

changes to the criteria in listing 14.08. 

We have carefully considered the 

advances in treatment and consequent 

longevity that have occurred since we 

published the current rules in 1993. 

However, we do not believe that there 

has been sufficient progress in the 

treatment and control of HIV infection 

to warrant any change in these rules. 

Moreover, even as some problems of 

people who have HIV infection appear 

to be improved, new problems have 

arisen to take their place. Advances in 

treatment are a case in point. While 

there have been significant strides in the 

treatment of HIV infection that have 

improved mortality, the treatment itself 

is often disabling both in terms of its 

side effects and its administration. 

Many people must structure their days 

and nights around their treatment, and 

any lapse can have dire consequences. 

Some people respond to treatment 

initially but become unresponsive 

without warning. Others have only 

limited success with their treatments. 

Relatively few people with HIV 

infection are considered ‘‘well.’’

Therefore, from the standpoint of Social 

Security disability policy and efficient 


jlentini on PROD1PC65 with PROPOSALS3
44444 Federal Register / Vol. 71, No. 150 / Friday, August 4, 2006 / Proposed Rules 

administration of the disability 

programs, we have not seen sufficient 

evidence to persuade us to propose any 

significant changes in this listing. 

As already noted, we propose to 

remove current reference listings 

throughout this body system, including 

the reference listings in listing 14.08. 

This would result in the removal of 

several specific listings within 14.08 

and the redesignation of some of the 

current listings; for example, current 

listing 14.08N would become listing 

14.08K. Where we propose to remove a 

reference listing, however, we have 

ensured that we provide guidance in the 

introductory text about where to 

evaluate the impairment. For example, 

current listing 14.08A4, for HIV 

infection with syphilis or neurosyphilis, 

is a reference listing that says only to 

consider the impairment under the 

criteria for the affected body system, 

such as 2.00 (special senses and 

speech), 4.00 (cardiovascular system), or 

11.00 (neurological). Although we 

propose to remove this reference listing, 

we include this same guidance in 

proposed 14.00J2l. 

We also propose to clarify some of the 

rules. We propose to reorganize the 

language in listing 14.08B2 to make it 

clearer that we evaluate under this 

listing candidiasis involving the 

esophagus, trachea, bronchi, or lungs, or 

at another site other than the skin, 

urinary tract, intestinal tract, or oral or 

vulvovaginal mucous membranes. We 

propose to move current listing 14.08C2, 

for PCP, from the listing for protozoan 

and helminthic infections to the listing 

for fungal infections because the 

organism that causes PCP is now known 

to be a fungus. We redesignate it as 

proposed listing 14.08B7. 

We propose to redesignate current 

listing 14.08N as proposed listing 

14.08K. We propose to expand our 

guidance on manifestations we evaluate 

under proposed listing 14.08K by 

adding ‘‘pancreatitis, hepatitis, 

peripheral neuropathy, glucose 

intolerance, muscle weakness, and 

cognitive or other mental impairments’’

as new examples. We also expand our 

list of signs or symptoms by adding 

‘‘nausea, vomiting, headaches, or 

insomnia.’’

We propose minor changes to the 

language of the functional criteria in 

proposed listing 14.08K from the 

current language in listing 14.08N. For 

example, we would replace the words 

‘‘restriction’’ in current listing 14.08N1 

and ‘‘difficulties’’ in current listings 

14.08N2 and 14.08N3 with the word 

‘‘limitation’’ in proposed listings 

14.08K1, 14.08K2, and 14.08K3. We 

propose to make this change because 

‘‘limitation’’ is a clearer term that we 

use throughout our rules. 

Proposed Listing 14.09—Inflammatory 

Arthritis 

We are redesignating current listing 

14.09D as proposed listing 14.09B, 

current listing 14.09B as proposed 

listing 14.09C1, and current listing 

14.09E as proposed listings 14.09C2 to 

put them in a more logical order. In the 

proposed rules, listing 14.09A would 

describe persistent inflammation or 

deformity of major peripheral joints that 

alone is disabling, while listing 14.09B 

would describe disability with lesser 

inflammation or deformity of major 

peripheral joints, organ involvement, 

and constitutional symptoms. Listing 

14.09C would describe listing-level 

inflammatory arthritis of the spine. 

Proposed listing 14.09C1 would 

describe disability based only on 

fixation (ankylosis) of the spine, while 

listing 14.09C2 would describe 

disability based on a lesser degree of 

ankylosis of the spine with organ 

involvement. Proposed listing 14.09D 

would be the same functional listing we 

include in all of the proposed immune 

system listings and would apply to 

inflammatory arthritis affecting any 

joints. 

Proposed listing 14.09A corresponds 

to current listing 14.09A. We propose to 

remove the requirement for a history of 

joint pain, swelling, and tenderness 

from this listing because it is 

unnecessary and to provide only that 

joint inflammation must be ‘‘persistent.’’

(We do refer to joint pain, swelling, and 

tenderness in proposed 14.00D6a.) 

Persistent joint inflammation or 

deformity in two or more major 

peripheral joints resulting in the 

inability to ambulate effectively or 

inability to perform fine and gross 

movements effectively is in itself 

indicative of an impairment that would 

preclude any gainful activity. For the 

same reasons, we also propose to 

remove the requirement for ‘‘signs on 

current physical examination.’’ We 

would not need signs of joint 

inflammation on a current physical 

examination when we have medical 

evidence documenting that you have 

inflammatory arthritis that results in the 

inability to ambulate effectively or 

inability to perform fine and gross 

movements effectively. Also, because of 

the episodic nature of inflammatory 

arthritis a current physical examination 

could show a brief period of 

improvement for a few days even 

though your longitudinal medical 

records may show persistent joint 

inflammation that results in the 

inability to ambulate effectively or 

inability to perform fine and gross 

movements. We propose to change ‘‘two 

or more major joints’’ to ‘‘two or more 

major peripheral joints’’ to distinguish 

these joints from the joints of the spine. 

We define ‘‘major peripheral joints’’ in 

proposed 14.00C8. 

Proposed listing 14.09B corresponds 

to current listing 14.09D. The revisions 

in proposed 14.09B are similar to those 

in proposed listing 14.09A for the same 

reasons and to make it clearer that this 

listing requires joint inflammation in 

one or more major peripheral joints. 

Proposed 14.09B continues to require 

less joint involvement than in A, but we 

would no longer require ‘‘lesser extraarticular features than in C’’ because 

‘‘C’’ refers to current reference listing 

14.09C which we are proposing to 

remove. Instead, we require ‘‘extraarticular features that do not satisfy the 

criteria of a listing.’’ Proposed listing 

14.09B1 corresponds to current listing 

14.09D2 with nonsubstantive editorial 

changes to make it consistent with how 

we present this criterion throughout 

these listings. Proposed listing 14.09B2 

corresponds to current listing 14.09D1 

except that we have removed the phrase 

‘‘significant, documented’’ for reasons 

we have already explained. We also 

propose to correct an error in current 

listing 14.09D1. The explanatory 

abbreviation, ‘‘e.g.’’ (for example) in 

current listing 14.09D1 inaccurately 

indicates that the four constitutional 

symptoms or signs, that is, fatigue, 

fever, malaise, and involuntary weight 

loss, are only examples when they are 

in fact a complete list. Consistent with 

changes in other proposed listings, we 

propose to require at least two of the 

constitutional symptoms or signs 

because we believe that the criteria in 

proposed listing 14.09B are indicative of 

an impairment that precludes any 

gainful activity. 

Proposed listing 14.09C1 corresponds 

to current listing 14.09B. We propose to 

reorganize the criteria and to remove the 

requirements for ‘‘diagnosis established 

by findings of unilateral or bilateral 

sacroiliitis (e.g., erosions or fusions)’’

and ‘‘[h]istory of back pain, tenderness, 

and stiffness’’ because these findings are 

unnecessary. We believe ankylosing 

spondylitis or other 

spondyloarthropathies with ankylosis of 

the dorsolumbar or cervical spines at 

45° or more of flexion documented as 

required in proposed listing 14.09C1 are 

in themselves indicative of an 

impairment that precludes any gainful 

activity. 

Proposed listing 14.09C2 corresponds 

to current listing 14.09E. We propose to 

reorganize this listing to make it more 

consistent with the structure and 


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Federal Register / Vol. 71, No. 150 / Friday, August 4, 2006 / Proposed Rules 44445 

criteria that we use in the proposed 

listings for other autoimmune disorders. 

We propose to remove the phrase ‘‘with 

lesser deformity than in B,’’ which 

describes a deformity that is less than 

the fixation ‘‘of the dorsolumbar or 

cervical spine at 45° or more of flexion’’

under current listing 14.09B, and to 

replace it with fixation ‘‘at 30° or more 

of flexion (but less than 45°).’’ We 

believe that this would be a clearer and 

more specific criterion that would help 

to provide greater uniformity in 

adjudications under this listing. We 

propose to remove the phrase ‘‘lesser 

extra-articular features than in C’’

because it refers to current reference 

listing 14.09C, which we are proposing 

to remove. We also propose to remove 

the phrase ‘‘with signs of unilateral or 

bilateral sacroiliitis’’ because the criteria 

in the proposed listing would be 

sufficient to show listing-level severity 

without this requirement, and the 

phrase ‘‘with the extra-articular features 

described in 14.09D’’ because it is 

unnecessary language. 

Proposed Listing 14.10—Sjo ̈gren’s 

Syndrome 

Proposed listing 14.10 is new. We are 

proposing to add it in response to 

comments we received that Sjo ̈gren’s 

syndrome is distinct from other immune 

system disorders with unique aspects 

that the current immune system listings 

do not address. 

Although individuals with Sjo ̈gren’s 

syndrome can qualify under current 

listings 14.03 and 14.09, and other 

listings, we believe that it is now 

appropriate to list Sjo ̈gren’s syndrome 

separately in these listings. We propose 

to use the same two listing criteria for 

establishing listing-level severity as in 

the other proposed listings for 

autoimmune disorders because 

Sjo ̈gren’s syndrome is an autoimmune 

disorder that can cause the same kinds 

of constitutional symptoms and signs as 

other autoimmune disorders, and 

because it can be as functionally 

limiting as other autoimmune disorders. 

Proposed listing 14.10A is the same as 

proposed listings 14.02A, 14.03A, 

14.04A, and 14.06A, and proposed 

listing 14.10B is the same as proposed 

listings 14.02B, 14.03B, 14.04D, 14.05E, 

14.06B, and 14.09D. We also provide a 

new separate section in the introductory 

text that describes the unique features of 

Sjo ̈gren’s syndrome, proposed 14.00D7. 

What revisions are we proposing to 

make in the immune system disorder 

listings for children—114.00? 

As in proposed 14.00 in the adult 

rules, we propose to change the name of 

this body system to ‘‘Immune System 

Disorders.’’

Except for minor editorial changes, 

we have repeated much of the 

introductory text of proposed 14.00 in 

the introductory text to proposed 

114.00. This is because the same basic 

rules for establishing and evaluating the 

existence and severity of immune 

system disorders in adults also apply to 

children. Because we have already 

described these provisions under the 

explanation of proposed 14.00, the 

following discussions describe only 

those provisions that are unique to the 

childhood rules or that require further 

explanation. We describe only the major 

provisions. For example, we do not 

summarize minor editorial changes that 

refer to ‘‘children’’ instead of adults or 

to the policy of ‘‘functional 

equivalence’’ instead of RFC assessment 

and steps in the adult sequential 

evaluation process. 

Also, where appropriate in the 

introductory text of proposed 114.00, 

we have made an editorial change in the 

terms we use to identify the age 

categories of children in the 

introductory text of current 114.00 to be 

consistent with the terms we use in the 

introductory text of current 112.00, 

Mental Disorders. For example, in 

proposed 114.00F1b(ii), we use 

‘‘newborn and younger infants (birth to 

attainment of age 1)’’ instead of ‘‘an 

infant 12 months of age or less’’ used in 

current 114.00D3b(i). 

Proposed 114.00A—What disorders do 

we evaluate under the immune system 

listings? 

In proposed 114.00A1b, we 

incorporate the first sentence in the last 

paragraph of current 114.00B, which 

explains that immune system disorders 

may affect growth, development, 

attainment of age-appropriate skills, and 

performance of age-appropriate 

activities in children. We propose to 

revise the sentence by adding the phrase 

‘‘or their treatment.’’ We also propose to 

remove the phrase ‘‘attainment of ageappropriate skills’’ because it is 

redundant of ‘‘development.’’

Proposed 114.00A2 is essentially the 

same as proposed 14.00A2 and similar 

to the first and second paragraphs of 

current 114.00B. We propose to expand 

and clarify the guidance in the second 

paragraph to explain that autoimmune 

disorders or their treatment may have a 

considerable impact on the physical, 

psychological, and developmental 

growth of pre-pubertal children that 

often differs from that of post-pubertal 

children or adults. We also remove the 

last sentences from both the first and 

second paragraphs of current 114.00B 

because they cross-refer to 14.00 in the 

part A listings. In part B of these 

proposed rules, we are repeating criteria 

from part A when they are appropriate 

for evaluating children in part B of the 

listings so it should rarely be necessary 

to refer back to 14.00 in part A. 

Proposed 114.00D—What are the listed 

autoimmune disorders in these listings? 

Proposed 114.00D parallels the 

structure and content of proposed 

14.00D in the adult rules, except where 

the features commonly associated with 

the autoimmune disorders in these 

listings differ in children from adults. 

In proposed 114.00D2, Systemic 

vasculitis (114.03), as in current 

114.00C3, we provide guidance (in 

114.00D2a(ii)) on how we evaluate 

Kawasaki disease and add guidance 

about anaphylactoid purpura (HenochSchoenlein purpura). Also, in proposed 

114.00D2a(ii), we do not use the 

example of giant cell arteritis (temporal 

arteritis) that is in proposed 14.00D2a(ii) 

because this disorder occurs almost 

exclusively in individuals over 50 years 

of age. 

In proposed 114.00D3c, Localized 

scleroderma (linear scleroderma or 

morphea), we describe features of focal 

forms of scleroderma in children. These 

disorders occur primarily in children 

and are more common than systemic 

sclerosis in children. In proposed 

114.00D3c(i), we explain that the extent 

of involvement and the location of 

lesions are important factors in 

determining the limitations resulting 

from scleroderma. We also note that it 

may be appropriate to evaluate the 

limitations resulting from these 

impairments under the musculoskeletal 

(101.00) listings. In proposed 

114.00D3c(ii), we describe features of 

isolated morphea of the face and explain 

that it may be more appropriate to 

evaluate the limitations from these 

disorders under the affected body 

system, such as the special senses 

listings (102.00) or mental disorders 

listings (112.00). In 114.00D3c(iii) we 

describe features of chronic variants of 

these syndromes and explain that it is 

appropriate to evaluate the limitations 

from these disorders under the affected 

body system, such as the 

musculoskeletal listings (101.00) or 

respiratory system listings (103.00). 

In proposed 114.00D4, Polymyositis 

and dermatomyositis (114.05), we note 

(in 114.00D4a, General) that 

polymyositis occurs rarely in children 

and describe the features of 

dermatomyositis that occur differently 

in children than in adults. In children, 

polymyositis and dermatomyositis 

usually do not occur in association with 


jlentini on PROD1PC65 with PROPOSALS3
44446 Federal Register / Vol. 71, No. 150 / Friday, August 4, 2006 / Proposed Rules 

malignancies. For this reason, we do not 

include a reference to malignancy or 

provide guidance that we will evaluate 

malignancies under the malignant 

neoplastic diseases listings (113.00ff) in 

proposed 114.00D4, as we do for adults 

in proposed 14.00D4. However, unlike 

in the adult rules, we include a 

reference to calcinosis for children 

because some children develop 

calcinosis late in the disease. Also, 

when dermatomyositis involves other 

organs or body systems, we evaluate the 

involvement under the affected body 

system. In proposed 114.00D4b, 

Documentation of polymyositis or 

dermatomyositis, we note that magnetic 

resonance imaging (MRI) showing 

muscle inflammation or vasculitis 

provides additional evidence of 

childhood dermatomyositis. We did not 

provide this guidance in proposed 

14.00D4b because MRI findings are not 

considered diagnostic of 

dermatomyositis in adults. In proposed 

114.00D4c(i), we explain how to 

evaluate polymyositis and 

dermatomyositis under the listings in 

newborn and younger infants. 

In proposed 114.00D5, 

Undifferentiated and mixed connective 

tissue disease (114.06), we note (in 

proposed 114.00D5a, General) that the 

most common pattern of 

undifferentiated autoimmune disorders 

in children is mixed connective tissue 

disease (MCTD). In proposed 

114.00D5b, Documentation of 

undifferentiated and mixed connective 

disease, we note diagnostic laboratory 

findings specifically for children with 

MCTD and that the clinical findings are 

often suggestive of SLE or childhood 

dermatomyositis. We also note that 

many children later develop features of 

scleroderma. 

In proposed 114.00D6, Inflammatory 

arthritis (114.09), we discuss 

inflammatory arthritides. In proposed 

114.00D6a, General, we incorporate 

guidance in current 114.00C2 and 

114.00E. We explain that we evaluate 

growth impairment resulting from 

inflammatory arthritides under the 

criteria in 100.00ff. In proposed 

114.00D6b, Inflammatory arthritides 

involving the axial spine 

(spondyloarthropathies), we incorporate 

the second sentence in current 114.00E 

and revise some of the examples of 

disorders that may be associated with 

inflammatory spondyloarthropathies 

involving the axial spine with disorders 

that are more common in children. 

Current 114.00E6 provides that the 

fact that a child is dependent on 

steroids, or any other drug, for the 

control of inflammatory arthritis is, in 

and of itself, insufficient to find 

disability. It explains that advances in 

the treatment of inflammatory 

connective tissue disease and in the 

administration of steroids for its 

treatment have corrected some of the 

previously disabling consequences of 

continuous steroid use. Although this 

statement is still true, we are not 

including this provision of current 

114.00E6 in these proposed rules 

because we believe we no longer need 

it in the introductory text of the listings. 

We added current 114.00E6 in 2002 

(66 FR 58010, 58022 and 58045 (2001)). 

It was important when we added it 

because the listings prior to the 

revisions we made in 2002 included a 

listing (prior listing 101.02B) that said 

that all children with rheumatoid 

arthritis who were dependent on 

steroids were disabled. We removed that 

listing in 2002, explaining that, 

although the prior listing was 

appropriate when we first published it, 

advances in treatment and other reasons 

had made it obsolete (66 FR 58022). 

Thus, the paragraph in the introductory 

text served as a reminder that we no 

longer had that listing and that it was no 

longer appropriate to presume disability 

based on steroid use alone. Now that 

several years have passed since we 

removed the prior listing, we do not 

believe that we need this reminder any 

longer. However, in proposed 114.00G3, 

we continue to state that we will 

consider the adverse side effects of 

treatment, including the effects of 

corticosteroids, to ensure that our 

adjudicators remember to consider the 

side effects of steroids and any other 

treatment an individual might have. 

Proposed 114.00F—How do we evaluate 

human immunodeficiency virus (HIV) 

infection? 

Proposed 114.00F parallels the 

structure and content of proposed 

14.00F in the adult rules, except where 

the features commonly associated with 

HIV infection differ in children from 

adults. 

Proposed 114.00F1a, Documentation 

of HIV infection by definitive diagnosis, 

corresponds to 114.00D3a in the current 

rules and 14.00F1a in the proposed 

rules. In this section, we propose to 

lower the age for using HIV antibody 

tests from 24 months of age or older that 

is in current 114.00D3a(i) to 18 months 

or older in proposed 114.00F1a(i) 

because current clinical practice now 

accepts these tests beginning at 18 

months of age. 

In proposed 114.00F1a(iv), we clarify 

the provision in current 114.00D3a(ii) 

by explaining that a specimen that 

contains HIV antigen may be used to 

establish the diagnosis of HIV infection 

in a child age 1 month or older. 

Proposed 114.00F1b, Documentation 

of HIV infection in children from birth 

to the attainment of 18 months is new 

and corresponds to the second 

paragraph in current 114.00D3b, Other 

acceptable documentation of HIV 

infection in children. However, we are 

proposing to move this information 

under proposed 114.00F1b to provide 

documentation of HIV infection by 

definitive diagnosis in children from 

birth to the attainment of 18 months of 

age who have tested positive for HIV 

antibodies. We also propose to lower the 

age for children testing positive for HIV 

antibodies from 24 months of age that is 

in the second paragraph of current 

114.00D3b to 18 months in proposed 

114.00F1b. We are proposing to make 

these changes because current clinical 

practice now accepts these positive test 

results as diagnostic of HIV infection in 

children beginning at 18 months of age 

who have tested positive for HIV 

antibodies. 

In proposed 114.00F1b(i), we propose 

to add ‘‘One or more of the tests listed 

in F1a(ii)–F1a(vii)’’ of proposed 

114.00F1a because these tests are 

accepted as diagnostic of HIV infection. 

In proposed 114.00F1b(iii), we 

propose to change ‘‘12 to 24 months of 

age’’ in current 114.00D3b(ii) to ‘‘12 to 

18 months of age’’ based on how these 

findings are used in current clinical 

practice. 

In proposed 114.00F1b(v), we specify 

that a severely diminished 

immunoglobulin G (IgG) level is ‘‘<4g/ 

l or 400 mg/dl.’’ However, we do not 

provide an IgG level for greater than 

normal range for age due to the 

variability in the higher normal range of 

IgG level in children by age. There is 

consistency in the normal lower average 

range in children, so we are able to 

specify levels for severely diminished 

IgG. 

Proposed 114.00F1c, Other acceptable 

documentation of HIV infection, 

corresponds to current 114.00D3b and 

proposed 14.00F1b. We propose to 

remove the first paragraph in current 

114.00D3b because all infants who have 

HIV antibodies are now tested to 

determine definitively whether they 

have HIV infection. This makes the first 

paragraph in current 114.00D3b 

unnecessary. 

In proposed 114.00F2, CD4 tests, we 

add more detailed guidance to the 

second paragraph of current 114.00D4a 

by specifying that the extent of immune 

depression correlates with the level of 

CD4 counts in children at 6 years of age 

or older, the age at which CD4 levels 

become comparable to adult CD4 levels. 


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In proposed 114.00F3b, Other 

acceptable documentation of the 

manifestations of HIV infection, we 

explain in proposed 114.00F3b(i) for 

PCP and in 114.00F3b(ii) for CMV that 

a CD4 count below 200 in children 6 

years of age or older is supportive 

evidence of a presumptive diagnosis of 

these manifestations. 

Proposed 114.00F4, HIV 

manifestations specific to children, 

corresponds to current 114.00D5, HIV in 

children. In proposed 114.00F4a, 

General, we propose to remove the 

second sentence in current 114.00D5. 

That sentence explains that survival 

times are shorter for children who are 

infected in the first year of life than they 

are for older children and adults. 

However, due to advances in medical 

treatment this is no longer the case. The 

second sentence of proposed 114.00F4a 

is based on the first paragraph in current 

114.00D5. 

In proposed 114.00F4b, Neurologic 

abnormalities, we make some 

nonsubstantive editorial changes to the 

second paragraph in current 114.00D5 

in which we explain that the methods 

of identifying and evaluating 

neurological abnormalities vary 

depending on a child’s age. We also 

replace ‘‘acquisition’’ with ‘‘onset’’ in 

the last sentence of proposed 114.00F4b 

because a sudden ‘‘onset’’ of a new 

learning disability is medically a more 

accurate description of how this 

neurologic abnormality would manifest 

in a child with HIV infection. 

In proposed 114.00F4c, Bacterial 

infections, we incorporate the last two 

paragraphs in current 114.00D5. We 

propose only nonsubstantive editorial 

changes, including removing text that 

only repeats criteria from the listings. 

Proposed 114.00G—How will we 

consider the effect of treatment in 

evaluating your autoimmune disorder, 

immune deficiency disorder, or HIV 

infection? 

In proposed 114.00G2, Variability of 

your response to treatment, we use an 

example of a child who develops otitis 

media instead of pneumonia or 

tuberculosis as we do in proposed 

14.00G2 for an adult because otitis 

media is more common in children. 

In proposed 114.00G3, How we 

evaluate the effects of treatment for 

autoimmune disorders on your ability to 

function, we use examples of impaired 

growth and osteopenia for children 

instead of osteoporosis as we do in 

proposed 14.00G3 for adults because 

impaired growth and osteopenia are 

more common in children. 

Proposed 114.00I—How do we use the 

functional criteria in these listings? 

As in the adult rules, we propose to 

add listings based on functional criteria 

to each of the listings in the immune 

system in addition to listing 114.08. 

Current listing 114.08O is the childhood 

listing that corresponds to current adult 

listing 14.08N, and we are proposing to 

use essentially the same criteria in the 

other listings as we do in this listing. (In 

the proposed rules, current listing 

114.08O would become listing 114.08L.) 

Proposed 114.00I—How do we use the 

functional criteria in these listings?—

corresponds to current 114.00D8 and 

provides guidance for applying the 

listings based on functional criteria. We 

propose to revise the current language to 

reflect the fact that there would now be 

functional listings for each of the listed 

impairments in this body system and for 

consistency with adult rules where 

appropriate. 

Proposed 114.00J—How do we evaluate 

your immune system disorder when it 

does not meet one of these listings? 

In proposed 114.00J2, we repeat the 

guidance in proposed 14.00J but with 

appropriate references to listings in part 

B, and we include growth impairment 

under 100.00ff as an example. 

How are we proposing to change the 

criteria in the listings for evaluating 

immune system impairments in 

children? 

Proposed 114.01 Category of 

Impairments, Immune System Disorders 

As in the adult listings in part A, we 

propose to remove all reference listings 

from part B. We also propose to add 

listings like 114.08O to each of the other 

listings in this body system. The new 

listings would be proposed listings 

114.02B, 114.03B, 114.04D, 114.05E, 

114.06B, 114.07C, 114.09D, and 

114.10B. In addition, current listing 

114.08O would be redesignated as 

listing 114.08L because of the deletion 

of reference listings. The functional 

criteria in the new proposed listings for 

children would be the same as in 

current listing 114.08O (proposed listing 

114.08L). They are different from the 

functional criteria in part A because the 

functional criteria for adults are not 

applicable to the evaluation of 

functioning in children. The childhood 

functional criteria are the same as in 

current listing 114.08O (proposed listing 

114.08L); they use the functional criteria 

in listings 112.02 and 112.12. 

The following is a description of the 

significant proposed changes in part B 

when they are different from the 

changes we propose in part A or require 

additional explanation. 

Proposed Listing 114.04—Systemic 

Sclerosis (Scleroderma) 

Proposed listings 114.04B1 and 

114.04B2 correspond to current listing 

114.04B1. We propose to change the 

requirement in current listing 114.04B1 

for fixed deformity of ‘‘both feet’’ to 

‘‘one or both feet’’ and to add ‘‘inability 

to ambulate effectively’’ to the listing 

criteria. This will allow some children 

with a serious deformity in only one 

foot to qualify based on the functional 

limitation we use to define listing-level 

severity throughout these listings. We 

also propose to add the criterion of ‘‘toe 

contractures’’ to proposed 114.04B1 

even though toe contractures of listinglevel severity would be rare in children 

to make it consistent with the criteria in 

proposed 14.04B1. We are retaining the 

requirement for involvement of both 

hands in proposed listing 114.04B2, 

because inability to use fine and gross 

movements effectively can only occur 

when both upper extremities are 

affected. We propose to add the 

criterion of ‘‘finger contractures’’ to 

proposed 114.004B2 for the same reason 

we are proposing to add ‘‘toe 

contractures’’ to proposed 114.04B1. 

Proposed listings 114.04B3 and 

114.04B4 correspond to current listing 

114.04B2, the listing for ‘‘[m]arked 

destruction or marked atrophy of an 

extremity.’’ We propose to revise the 

rules to 

• Remove the word ‘‘marked,’’

• Change the criterion for 

‘‘destruction’’ to ‘‘irreversible damage,’’

• Require both atrophy and 

irreversible damage in one or both lower 

extremities or both upper extremities, 

and 

• Require either inability to ambulate 

effectively or to use the upper 

extremities to perform fine and gross 

movements effectively. 

We propose to remove the word 

‘‘marked’’ because we use it in various 

other listings and other regulations to 

describe a particular measure of 

functional limitations, and it does not 

describe what we intend in this listing. 

We propose to replace the criterion for 

‘‘marked destruction’’ with a criterion 

for ‘‘irreversible damage’’ because it is a 

more accurate medical description of 

this complication of systemic sclerosis. 

We propose to require both atrophy and 

irreversible damage because we would 

not expect either of these findings alone 

to establish an impairment that results 

in marked and severe functional 

limitations in every case. Finally, we 

propose to require ‘‘inability to 

ambulate effectively’’ or ‘‘inability to 


jlentini on PROD1PC65 with PROPOSALS3
44448 Federal Register / Vol. 71, No. 150 / Friday, August 4, 2006 / Proposed Rules 

perform fine or gross movements 

effectively’’ to establish an impairment 

that is of listing-level severity, 

consistent with other existing and 

proposed listings. 

Proposed listing 114.04C, Raynaud’s 

phenomenon, is a new childhood listing 

and has the same criteria as in proposed 

listing 14.04C for adults. Even though 

listing-level severity would be rare in 

children with Raynaud’s phenomenon, 

it can occur. 

Proposed Listing 114.05—Polymyositis 

and Dermatomyositis 

We propose to remove current listing 

114.05B1 because multiple joint 

contractures are not typically a part of 

the disease process of polymyositis or 

dermatomyositis in children. However, 

if this should occur, we would evaluate 

whether your polymyositis or 

dermatomyositis with multiple joint 

contractures meets or medically equals 

the criteria in proposed listing 114.05E, 

medically equals the criteria in another 

listing, such as proposed listing 

114.05A, or functionally equals the 

listings. 

In proposed listing 114.05D, we 

propose to revise current listing 

114.05B2 by replacing ‘‘cutaneous 

calcification’’ with ‘‘calcinosis.’’ We are 

proposing this change because 

‘‘calcification’’ describes the normal 

process by which calcium salts are 

deposited in bone, and ‘‘calcinosis’’

describes the abnormal deposits of 

calcium salt in body tissues as we 

intend by this criterion. We are also 

proposing to replace ‘‘formation of an 

exoskeleton’’ with ‘‘limitation of joint 

mobility or intestinal motility’’ because 

it is a better description of the known 

complications of dermatomyositis in 

children. 

Proposed Listing 114.07—Immune 

deficiency disorders, excluding HIV 

infection 

We propose to remove current listing 

114.07B because of advances in medical 

knowledge that now allow us to identify 

different subgroups of thymic dysplastic 

syndromes. The subgroups of these 

disorders vary in severity, and therefore, 

they should be evaluated under 

proposed listing 114.07A, B, or C, as 

appropriate to the particular immune 

deficiency disorder and its effects. 

Proposed Listing 114.08—Human 

Immunodeficiency Virus (HIV) Infection 

In proposed listing 114.08A5, we 

incorporate current listing 114.08A6 

except to remove ‘‘Other’’ as a 

descriptor to make it consistent with the 

proposed adult listing. We propose to 

replace ‘‘acquisition’’ as used in current 

listing 114.08H1 with ‘‘onset’’ in 

proposed listing 114.08G1 because a 

sudden ‘‘onset’’ of a new learning 

disability is medically a more accurate 

description of how this neurologic 

abnormality would manifest in a child 

with HIV infection. We are also 

redesignating a number of listings to 

reflect the proposed removal of 

reference listings. 

Proposed Listing 114.10— Sjo ̈gren’s 

Syndrome 

We propose to add a new listing 

114.10 to evaluate Sjo ̈gren’s syndrome 

in children for the same reasons we 

propose to add a Sjo ̈gren’s syndrome 

listing for adults in part A. 

Other Changes 

We propose to make minor 

conforming changes in current 1.00B 

and 101.00B, and 1.00L and 101.00L to 

reflect changes in the proposed immune 

body system listings. 

We also propose to make minor 

conforming changes in current 8.00D3 

and 108.00D3 of the skin disorders 

listings. We would revise these sections 

to indicate that we evaluate Sjo ̈gren’s 

syndrome under the new listing for that 

disorder, listings 14.10 and 114.10. 

Clarity of These Proposed Rules 

Executive Order 12866, as amended 

by Executive Order 13258, requires each 

agency to write all rules in plain 

language. In addition to your 

substantive comments on these 

proposed rules, we invite your 

comments on how to make these 

proposed rules easier to understand. 

For example: 

• Have we organized the material to 

suit your needs? 

• Are the requirements in the rules 

clearly stated? 

• Do the rules contain technical 

language or jargon that is not clear? 

• Would a different format (grouping 

and order of sections, use of headings, 

paragraphing) make the rules easier to 

understand? 

• Would more (but shorter) sections 

be better? 

• Could we improve clarity by adding 

tables, lists, or diagrams? 

• What else could we do to make the 

rules easier to understand? 

Regulatory Procedures 

Executive Order 12866 

We have consulted with the Office of 

Management and Budget (OMB) and 

determined that these proposed rules 

meet the requirements for a significant 

regulatory action under Executive Order 

12866, as amended by Executive Order 

13258. Thus, they were subject to OMB 

review. 

Regulatory Flexibility Act 

We certify that these proposed rules 

would not have a significant economic 

impact on a substantial number of small 

entities because they would affect only 

individuals. Thus, a regulatory 

flexibility analysis as provided in the 

Regulatory Flexibility Act, as amended, 

is not required. 

Paperwork Reduction Act 

These proposed rules contain 

reporting requirements at 14.00B, 

14.00D, 14.00E, 14.00F, 114.00B, 

114.00D, 114.00E, 114.00F, 114.08 and 

114.09. The public reporting burden is 

accounted for in the Information 

Collection Requests for the various 

forms that the public uses to submit the 

information to SSA. Consequently, a 1- 

hour placeholder burden is being 

assigned to the specific reporting 

requirement(s) contained in these rules. 

We are seeking clearance of the burdens 

referenced in these rules because they 

were not considered during the 

clearance of the forms. An Information 

Collection Request has been submitted 

to OMB. We are soliciting comments on 

the burden estimate; the need for the 

information; its practical utility; ways to 

enhance its quality, utility and clarity; 

and on ways to minimize the burden on 

respondents, including the use of 

automated collection techniques or 

other forms of information technology. 

Comments should be submitted and/or 

faxed to the Office of Management and 

Budget and to the Social Security 

Administration at the following 

addresses/numbers: 

Office of Management and Budget, Attn: 

Desk Officer for SSA, New Executive 

Office Building, Room 10230, 725 

17th St., NW., Washington, DC 20530. 

Fax Number: 202–395–6974. 

Social Security Administration, Attn: 

SSA Reports Clearance Officer, Rm. 

1338 Annex Building, 6401 Security 

Boulevard, Baltimore, MD 21235–

6401. Fax Number: 410–965–6400. 

Comments can be received for up to 

60 days after publication of this notice, 

and your comments will be most useful 

if received by SSA within 30 days of 

publication. To receive a copy of the 

OMB clearance package, you may call 

the SSA Reports Clearance Officer on 

410–965–0454. 

References 

We consulted the following sources 

when developing these proposed rules: 

Bartlett, J.G. and Gallant, J.E., Medical 

Management of HIV Infection (Johns 

Hopkins University 2003). 


jlentini on PROD1PC65 with PROPOSALS3
Federal Register / Vol. 71, No. 150 / Friday, August 4, 2006 / Proposed Rules 44449 

Burchett, S.A. and Pizzo, P.A., HIV Infection 

in Infants, Children, and Adolescents, 

Pediatrics in Review, 24(6), 186–194 

(2001). 

Davidson, A. and Diamond, B., Autoimmune 

Diseases, The New England Journal of 

Medicine, 345(5), 1–21 (2001). 

Furst, D.E., Stem Cell Transplantation for 

Autoimmune Disease: Progress and 

Problems, Current Opinion in 

Rheumatology, 14(3), 220–224 (2002). 

Harris, E.D., et al., Eds., Kelley’s Textbook of 

Rheumatology, (Elsevier, 7th ed. 2005). 

Klippel, J.H., et al., Eds., Primer on the 

Rheumatic Diseases, (Arthritis Foundation, 

12th ed. 2001). 

Sicherer, S.H., et al., Primary 

Immunodeficiency Diseases in Adults, 

Journal of American Medical Association, 

279:58 (1998). 

Tyndall, A. and Koike, T., High-dose 

immunoablative therapy with 

hematopoietic stem cell support in the 

treatment of severe autoimmune disease: 

current status and future direction, Internal 

Medicine, 41(8), 608–12 (2002). 

These references are included in the 

rulemaking record for these proposed 

rules and are available for inspection by 

interested persons by making 

arrangements with the contact person 

shown in this preamble. 

(Catalog of Federal Domestic Assistance 

Program Nos. 96.001, Social SecurityDisability Insurance; 96.002, Social SecurityRetirement Insurance; 96.004, Social 

Security-Survivors Insurance; and 96.006, 

Supplemental Security Income) 

List of Subjects 20 CFR Part 404 

Administrative practice and 

procedure, Blind, Disability benefits, 

Old-Age, Survivors, and Disability 

Insurance, Reporting and recordkeeping 

requirements, Social Security. 

Dated: July 28, 2006. 

Jo Anne B. Barnhart, 

Commissioner of Social Security. 

For the reasons set out in the 

preamble, we propose to amend subpart 

P of part 404 of chapter III of title 20 of 

the Code of Federal Regulations as set 

forth below: 

PART 404—FEDERAL OLD-AGE, 

SURVIVORS AND DISABILITY 

INSURANCE (1950– ) 

1. The authority citation for subpart P 

of part 404 continues to read as follows: 

Authority: Secs. 202, 205(a), (b), and (d)–

(h), 216(i), 221(a) and (i), 222(c), 223, 225, 

and 702(a)(5) of the Social Security Act (42 

U.S.C. 402, 405(a), (b), and (d)–(h), 416(i), 

421(a) and (i), 422(c), 423, 425, and 

902(a)(5)); sec. 211(b), Public Law 104–193, 

110 Stat. 2105, 2189. 

Appendix 1 to Subpart P of Part 404—

[Amended] 

2. Appendix 1 to subpart P of part 404 is 

amended as follows: 

a. Revise the expiration date in item 15 of 

the introductory text before part A of 

appendix 1. 

b. Revise the second sentence of section 

1.00B1 of part A of appendix 1. 

c. Revise the fourth sentence of section 

1.00L of part A of appendix 1. 

d. Revise section 8.00D3 of part A of 

appendix 1. 

e. Revise section 14.00 of part A of 

appendix 1. 

f. Revise the second sentence of section 

101.00B1 of part B of appendix 1. 

g. Revise the fourth sentence of section 

101.00L of part B of appendix 1. 

h. Revise section 108.00D3 of part B of 

appendix 1. 

i. Revise section 114.00 of part B of 

appendix 1. 

Appendix 1 to Subpart P of Part 404—Listing 

of Impairments 

* * * * * 

15. Immune System Disorders (14.00 and 

114.00): (date 8 years from the effective date 

of the final rules.) 

* * * * * 

Part A 

* * * * * 

1.00 Musculoskeletal System 

* * * * * 

B. Loss of function. 

1. General. * * * For inflammatory 

arthritides that may result in loss of function 

because of inflammatory peripheral joint or 

axial arthritis or sequelae, or because of 

extra-articular features, see 14.00D6. * * * 

* * * * * 

L. Abnormal curvatures of the spine. * * * 

When the abnormal curvature of the spine 

results in symptoms related to fixation of the 

dorsolumbar or cervical spine, evaluation of 

equivalence may be made by reference to 

14.09C. * * * 

* * * * * 

8.00 Skin Disorders 

* * * * * 

D. How do we assess impairments that may 

affect the skin and other body systems? 

* * * * * 

3. Autoimmune disorders and other 

immune system disorders (for example, 

systemic lupus erythematosus, scleroderma, 

human immunodeficiency virus (HIV) 

infection, and Sjo ̈gren’s syndrome) often 

involve more than one body system. We first 

evaluate these disorders under the immune 

system listings in 14.00. We evaluate lupus 

erythematosus under 14.02, scleroderma 

under 14.04, symptomatic HIV infection 

under 14.08, and Sjo ̈gren’s syndrome under 

14.10. 

* * * * * 

14.00 Immune System Disorders 

A. What disorders do we evaluate under 

the immune system listings? 

1. We evaluate immune system disorders 

that cause dysfunction in one or more 

components of your immune system. 

a. These listings are examples of immune 

system disorders that are severe enough to 

prevent you from doing any gainful activity. 

The dysfunction may be due to problems in 

antibody production, impaired cell-mediated 

immunity, a combined type of antibody/ 

cellular deficiency, impaired phagocytosis, or 

complement deficiency. 

b. Immune system disorders may result in 

recurrent and unusual infections, or 

inflammation and dysfunction of the body’s 

own tissues. Immune system disorders can 

cause a deficit in a single organ or body 

system that results in extreme (that is, very 

serious) loss of function. They can also cause 

lesser degrees of limitations in two or more 

organs or body systems, and when associated 

with symptoms or signs such as fatigue, 

fever, malaise, diffuse musculoskeletal pain, 

or involuntary weight loss, can also result in 

extreme limitation. 

c. In this preface, we organize the 

discussions of immune system disorders in 

three categories: Autoimmune disorders; 

Immune deficiency disorders, excluding 

human immunodeficiency virus (HIV) 

infection; and HIV infection. 

2. Autoimmune disorders (14.00D). 

Autoimmune disorders are caused by 

dysfunctional immune responses directed 

against the body’s own tissues, resulting in 

chronic, multisystem impairments that differ 

in clinical manifestations, course, and 

outcome. They are sometimes referred to as 

rheumatic diseases, connective tissue 

disorders, or collagen vascular disorders. 

Some of the features of autoimmune 

disorders in adults differ from the features of 

the same disorders in children. 

3. Immune deficiency disorders, excluding 

HIV infection (14.00E). Immune deficiency 

disorders are characterized by recurrent or 

unusual infections that respond poorly to 

treatment, and are often associated with 

complications affecting other parts of the 

body. Immune deficiency disorders are 

classified as either primary (congenital) or 

acquired. Individuals with immune 

deficiency disorders also have an increased 

risk of malignancies and of having 

autoimmune disorders. 

4. Human immunodeficiency virus (HIV) 

infection (14.00F). HIV infection is caused by 

a specific retrovirus and may be 

characterized by increased susceptibility to 

opportunistic infections, cancers, or other 

conditions as described in 14.08. 

B. What information do we need to show 

that you have an immune system disorder? 

Generally, we need your medical history, 

report(s) of physical examination, report(s) of 

laboratory findings, and in some instances, 

appropriate medically acceptable imaging or 

tissue biopsy reports to show that you have 

an immune system disorder. Therefore, we 

will make every reasonable effort to obtain 

your medical history, medical findings, and 

results of laboratory tests. We explain the 

information we need in more detail in the 

sections below. 

C. Definitions. 

1. Appropriate medically acceptable 

imaging includes, but is not limited to, 

angiography, x-ray imaging, computerized 

axial tomography (CAT scan) or magnetic 

resonance imaging (MRI), with or without 

contrast material, myelography, and 

radionuclear bone scans. ‘‘Appropriate’’

means that the technique used is one that is 

generally accepted and consistent with the 


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prevailing state of medical knowledge and 

clinical practice to support the evaluation 

and diagnosis of the impairment. 

2. Constitutional symptoms or signs means 

fatigue, fever, malaise, or involuntary weight 

loss. Severe fatigue means a frequent sense of 

exhaustion that results in significantly 

reduced physical activity or mental function. 

Malaise means frequent feelings of illness, 

bodily discomfort, or lack of well-being that 

result in significantly reduced physical 

activity or mental function. 

3. Disseminated means that a condition is 

spread over a considerable area. The type and 

extent of the spread will depend on your 

specific disease. 

4. Dysfunction means that one or more of 

the body regulatory mechanisms are 

impaired, causing either an excess or 

deficiency of immunocompetent cells or their 

products. 

5. Extra-articular means ‘‘other than the 

joints’’; for example, the effect is in an 

organ(s) such as the heart, lungs, kidneys, or 

skin. 

6. Inability to ambulate effectively has the 

same meaning as in 1.00B2b. 

7. Inability to perform fine and gross 

movements effectively has the same meaning 

as in 1.00B2c. 

8. Major peripheral joints has the same 

meaning as in 1.00F. 

9. Persistent means that a sign(s) or 

symptom(s) has continued over time. The 

precise meaning will depend on the specific 

immune system disorder, the usual course of 

the disorder, and the other circumstances of 

your clinical course. 

10. Recurrent means that a condition that 

previously responded adequately to an 

appropriate course of treatment returns after 

a period of remission or regression. The 

precise meaning, such as the extent of 

response or remission and the time periods 

involved, will depend on the specific disease 

or condition you have, the body system 

affected, the usual course of the disorder and 

its treatment, and the other facts of your 

particular case. 

11. Resistant to treatment means that a 

condition did not respond adequately to an 

appropriate course of treatment. Whether a 

response is adequate or a course of treatment 

is appropriate will depend on the specific 

disease or condition you have, the body 

system affected, the usual course of the 

disorder and its treatment, and the other facts 

of your particular case. 

12. Severe describes medical severity as 

used by the medical community. The term 

does not have the same meaning as it does 

when we use it in connection with a finding 

at the second step of the sequential 

evaluation processes in §§ 404.1520, 416.920, 

and 416.924. 

D. What are the listed autoimmune 

disorders in these listings? 

1. Systemic lupus erythematosus (14.02). 

a. General. Systemic lupus erythematosus 

(SLE) is a chronic inflammatory disease that 

can affect any organ or body system. It is 

frequently, but not always, accompanied by 

constitutional symptoms or signs (fatigue, 

fever, malaise, involuntary weight loss). 

Major organ or body system involvement can 

include: Respiratory (pleuritis, pneumonitis), 

cardiovascular (endocarditis, myocarditis, 

pericarditis, vasculitis), renal 

(glomerulonephritis), hematologic (anemia, 

leukopenia, thrombocytopenia), skin 

(photosensitivity), neurologic (seizures), 

mental (anxiety), fluctuating cognition 

(‘‘lupus fog’’), mood disorders, organic brain 

syndrome, psychosis), or immune system 

(inflammatory arthritis) disorders. 

Immunologically, there is an array of 

circulating serum auto-antibodies and proand anti-coagulant proteins that may occur in 

a highly variable pattern. 

b. Documentation of SLE. Generally, but 

not always, the medical evidence will show 

that your SLE satisfies the criteria in the 

current ‘‘Criteria for the Classification of 

Systemic Lupus Erythematosus’’ by the 

American College of Rheumatology found in 

the most recent edition of the Primer on the 

Rheumatic Diseases published by the 

Arthritis Foundation. 

2. Systemic vasculitis (14.03). 

a. General. (i) Vasculitis is an inflammation 

of blood vessels. It may occur acutely in 

association with adverse drug reactions, 

certain chronic infections, and occasionally, 

malignancies. More often, it is chronic and 

the cause is unknown. Symptoms vary 

depending on which blood vessels are 

involved. Systemic vasculitis may also be 

associated with other autoimmune disorders; 

for example, SLE or dermatomyositis. 

(ii) There are several clinical patterns, 

including but not limited to polyarteritis 

nodosa, Takayasu’s arteritis (aortic arch 

arteritis), giant cell arteritis (temporal 

arteritis), and Wegener’s granulomatosis. 

b. Documentation of systemic vasculitis. 

Angiography or tissue biopsy confirms a 

diagnosis of systemic vasculitis when the 

disease is suspected clinically. Usually the 

results will be in your medical records. 

3. Systemic sclerosis (scleroderma) (14.04). 

a. General. Systemic sclerosis 

(scleroderma) constitutes a spectrum of 

disease in which thickening of the skin is the 

clinical hallmark. Raynaud’s phenomenon, 

often medically severe and progressive, is 

present frequently and may be the peripheral 

manifestation of a vasospastic abnormality in 

the heart, lungs, and kidneys. The CREST 

syndrome (calcinosis, Raynaud’s 

phenomenon, esophageal dysmotility, 

sclerodactyly, and telangiectasia) is a variant 

that may slowly progress over years to the 

generalized process, systemic sclerosis. 

b. Diffuse cutaneous systemic sclerosis. In 

diffuse cutaneous systemic sclerosis (also 

known as diffuse scleroderma), major organ 

or systemic involvement can include the 

gastrointestinal tract, lungs, heart, kidneys, 

and muscle in addition to skin or blood 

vessels. Although arthritis can occur, joint 

dysfunction results primarily from soft 

tissue/cutaneous thickening, fibrosis, and 

contractures. 

c. Localized scleroderma (linear 

scleroderma and morphea). 

(i) Localized scleroderma (linear 

scleroderma and morphea) is more common 

in children than in adults; however, this type 

of scleroderma can persist into adulthood. 

The extent of involvement of linear 

scleroderma and a description of the lesions 

are important in assessing the severity of the 

impairment. For example, linear scleroderma 

involving the arm but not crossing any joints 

is not as functionally limiting as 

sclerodactyly (scleroderma localized to the 

fingers). Linear scleroderma of a lower 

extremity involving skin thickening and 

atrophy of underlying muscle or bone can 

result in contracture(s) and leg length 

discrepancies. In such cases, evaluation 

under the musculoskeletal (1.00ff) listing 

may be appropriate. 

(ii) When there is isolated morphea of the 

face causing facial disfigurement from 

unilateral hypoplasia of the mandible, 

maxilla, zygoma, or orbit, adjudication may 

be more appropriate under the criteria in the 

special senses listings (2.00ff) or mental 

disorders listings (12.00ff). 

(iii) Chronic variants of these syndromes 

include disseminated morphea, Shulman’s 

disease (diffuse fasciitis with eosinophilia), 

and eosinophilia-myalgia syndrome (often 

associated with toxins such as toxic oil or 

contaminated tryptophan), all of which can 

impose medically severe musculoskeletal 

dysfunction and may also lead to restrictive 

pulmonary disease. We evaluate these 

variants of the disease under the criteria in 

the musculoskeletal listings (1.00ff) or 

respiratory system listings (3.00ff). 

d. Documentation of systemic sclerosis 

(scleroderma). Documentation involves 

differentiating the clinical features of 

systemic sclerosis (scleroderma) from other 

autoimmune disorders; however, there may 

be an overlap. 

4. Polymyositis and dermatomyositis 

(14.05). 

a. General. Polymyositis and 

dermatomyositis are related disorders that 

are characterized by an inflammatory process 

in striated muscle, occurring alone or in 

association with other autoimmune disorders 

or malignancy. Symmetric weakness, and 

less frequently pain and tenderness of the 

proximal limb-girdle (shoulder or pelvic) 

musculature, are the most common 

manifestations. There may also be 

involvement of the cervical, cricopharyngeal, 

esophageal, intercostal, and diaphragmatic 

muscles. 

b. Documentation of polymyositis and 

dermatomyositis. Generally, but not always, 

polymyositis is associated with elevated 

serum muscle enzymes (creatine 

phosphokinase (CPK), aminotransferases, 

aldolase), and characteristic abnormalities on 

electromyography and muscle biopsy. In 

dermatomyositis there are characteristic skin 

findings in addition to the findings of 

polymyositis. 

c. Additional information about how we 

evaluate polymyositis and dermatomyositis 

under the listings. 

(i) Weakness of your pelvic girdle muscles 

that results in your inability to rise 

independently from a squatting or sitting 

position or to climb stairs may be an 

indication that you are unable to ambulate 

effectively. Weakness of your shoulder girdle 

muscles may result in your inability to 

perform lifting, carrying, and reaching 

overhead, and also may seriously affect your 

ability to perform activities requiring fine 

movements. We evaluate these limitations 

under 14.05A. 


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(ii) We use the malignant neoplastic 

diseases listings (13.00ff) to evaluate 

malignancies associated with polymyositis or 

dermatomyositis. We evaluate the 

involvement of other organs/body systems 

under the criteria for the listings in the 

affected body system. 

5. Undifferentiated and mixed connective 

tissue disease (14.06). 

a. General. This listing includes syndromes 

with clinical and immunologic features of 

several autoimmune disorders, but which do 

not satisfy the criteria for any of the specific 

disorders described. For example, you may 

have clinical features of systemic lupus 

erythematosus and systemic vasculitis, and 

the serologic (blood test) findings of 

rheumatoid arthritis. 

b. Documentation of undifferentiated and 

mixed connective tissue disease. 

Undifferentiated connective tissue disease is 

diagnosed when clinical features and 

serologic (blood test) findings, such as 

rheumatoid factor or antinuclear antibody 

(consistent with an autoimmune disorder) are 

present but do not satisfy the criteria for a 

specific disease. Mixed connective tissue 

disease (MCTD) is diagnosed when clinical 

features and serologic findings of two or 

more autoimmune diseases overlap. 

6. Inflammatory arthritis (14.09). 

a. General. The inflammatory arthritides 

include a vast array of disorders that differ 

in cause, course, and outcome. Clinically, 

inflammation of major peripheral joints may 

be the dominant manifestation causing 

difficulties with ambulation or fine and gross 

movements; there may be joint pain, 

swelling, and tenderness. The arthritis may 

affect other joints, or cause less functional 

limitations in ambulation or performance of 

fine and gross movements. However, in 

combination with extra-articular features, 

including constitutional symptoms or signs 

(fatigue, fever, malaise, involuntary weight 

loss), inflammatory arthritis may result in an 

extreme limitation. 

b. Inflammatory arthritides involving the 

axial spine (spondyloarthropathies). In 

adults, inflammatory arthritides involving 

the axial spine may be associated with 

heterogeneous disorders such as: 

(i) Reiter’s syndrome; 

(ii) Ankylosing spondylitis; 

(iii) Psoriatic arthritis; 

(iv) Whipple’s disease; 

(v) Behc ̧et’s disease; and 

(vi) Inflammatory bowel disease. 

c. Inflammatory arthritides involving the 

peripheral joints. The inflammatory 

arthropathies involving peripheral joints may 

be associated with disorders such as: 

(i) Rheumatoid arthritis; 

(ii) Sjo ̈gren’s syndrome; 

(iii) Psoriatic arthritis; 

(iv) Crystal deposition disorders (gout and 

pseudogout); 

(v) Lyme disease; and 

(vi) Inflammatory bowel disease. 

d. Documentation of inflammatory 

arthritides. Generally, but not always, the 

diagnosis of inflammatory arthritis is made 

by the clinical features and serologic findings 

described in the most recent edition of the 

Primer on Rheumatic Diseases published by 

the Arthritis Foundation. 

e. How we evaluate the inflammatory 

arthritides under the listings. 

(i) Listing-level severity in 14.09A and 

14.09C1 is shown by an impairment that 

results in an ‘‘extreme’’ (very serious) 

limitation. In 14.09A, the criterion is satisfied 

with persistent inflammation or deformity in 

two or more major peripheral joints resulting 

in the inability to ambulate effectively or 

inability to perform fine and gross 

movements effectively, as defined in 14.00C6 

and 14.00C7. In 14.09C1, if you have the 

required ankylosis (fixation) of your cervical 

or dorsolumbar spine, we will find that you 

have an extreme limitation in your ability to 

see in front of you, above you, and to the 

side. Therefore, inability to ambulate 

effectively is implicit in 14.09C1, even 

though you might not require bilateral upper 

limb assistance. 

(ii) Listing-level severity is shown in 

14.09B, 14.09C2, and 14.09D when the 

arthritis does not result in the extreme 

limitation in 14.09A or 14.09C1, involves one 

or more major peripheral joints, or involves 

other joints, but is complicated by extraarticular features that cumulatively result in 

an ‘‘extreme’’ (very serious) limitation or 

‘‘marked’’ (serious) limitations in at least two 

areas of functioning. Extra-articular 

impairments may also meet listings in other 

body systems. 

(iii) Extra-articular features of 

inflammatory arthritis may involve any body 

system. Commonly occurring extra-articular 

impairments include: Musculoskeletal (heel 

enthesopathy), ophthalmologic (iridocyclitis, 

keratoconjunctivitis sicca, uveitis), 

pulmonary (pleuritis, pulmonary fibrosis or 

nodules, restrictive lung disease), 

cardiovascular (aortic valve insufficiency, 

arrhythmias, coronary arteritis, myocarditis, 

pericarditis, Raynaud’s phenomenon, 

systemic vasculitis), renal (amyloidosis of the 

kidney), hematologic (chronic anemia, 

thrombocytopenia), neurologic (peripheral 

neuropathy, radiculopathy, spinal cord or 

cauda equina compression with sensory and 

motor loss), and immune system (Felty’s 

syndrome (hypersplenism with compromised 

immune competence)) disorders. 

(iv) If permanent deformity of a major 

peripheral joint is the dominant feature of 

your impairment, we evaluate your 

impairment under 1.02. 

(v) If there has been surgical reconstruction 

of a major weight-bearing joint, we evaluate 

your impairment under 1.03. 

(vi) If both inflammation and chronic 

deformities are present, we evaluate your 

impairment under the criteria of any 

appropriate listing. 

7. Sjo ̈ gren’s syndrome (14.10). 

a. General. (i) Sjo ̈gren’s syndrome is an 

immune-mediated disorder of the exocrine 

glands. Involvement of the lacrimal and 

salivary glands is the hallmark feature, 

resulting in symptoms of dry eyes and dry 

mouth, and possible complications such as 

corneal damage, blepharitis (eyelid 

inflammation), dysphagia (difficulty in 

swallowing), dental caries, and the inability 

to speak for extended periods of time. 

Involvement of the exocrine glands of the 

upper airways may result in persistent dry 

cough. 

(ii) Many other organ systems may be 

involved, including musculoskeletal 

(arthritis, myositis), respiratory (interstitial 

fibrosis), gastrointestinal (dysmotility, 

dysphagia, involuntary weight loss), 

genitourinary (interstitial cystitis, renal 

tubular acidosis), skin (purpura, vasculitis), 

neurologic (central nervous system disorders, 

cranial and peripheral neuropathies), mental 

(cognitive dysfunction, poor memory), and 

neoplastic (lymphoma). Fatigue and malaise 

are frequently reported. Sjo ̈gren’s syndrome 

may be associated with other autoimmune 

disorders (for example, rheumatoid arthritis 

or SLE); usually the clinical features of the 

associated disorder predominate. 

b. Documentation of Sjo ̈ gren’s syndrome. If 

you have Sjo ̈gren’s syndrome, the medical 

evidence will generally, but not always, show 

that your disease satisfies the criteria in the 

current ‘‘Criteria for the Classification of 

Sjo ̈gren’s Syndrome’’ by the American 

College of Rheumatology found in the most 

recent edition of the Primer on the 

Rheumatic Diseases published by the 

Arthritis Foundation. 

E. How do we evaluate immune deficiency 

disorders, excluding HIV infection (14.07)? 

1. General. 

a. Immune deficiency disorders can be 

classified as: 

(i) Primary (congenital); for example, Xlinked agammaglobulinemia, thymic 

hypoplasia (DiGeorge syndrome), severe 

combined immunodeficiency (SCID), chronic 

granulomatous disease (CGD), C1 esterase 

inhibitor deficiency. 

(ii) Acquired; for example, medicationrelated. 

b. Primary immune deficiency disorders 

are seen mainly in children. However, recent 

advances in the treatment of these disorders 

have allowed many affected children to 

survive well into adulthood. Occasionally, 

these disorders are first diagnosed in 

adolescence or adulthood. 

2. Documentation of immune deficiency 

disorders. The medical evidence must 

include documentation of the specific type of 

immune deficiency. Documentation may be 

by laboratory evidence or by other generally 

acceptable methods consistent with the 

prevailing state of medical knowledge and 

clinical practice. 

3. Immune deficiency disorders treated by 

stem cell transplantation. 

a. Evaluation in the first 12 months. If you 

undergo stem cell transplantation for your 

immune deficiency disorder, we will 

consider you disabled until at least 12 

months from the date of the transplant. 

b. Evaluation after the 12-month period 

has elapsed. After the 12-month period has 

elapsed, we will consider any residuals of 

your immune deficiency disorder as well as 

any residual impairment(s) resulting from the 

treatment, such as complications arising 

from: 

(i) Graft-versus-host (GVH) disease. 

(ii) Immunosuppressant therapy, such as 

frequent infections. 

(iii) Significant deterioration of other organ 

systems. 

4. Medication-induced immune 

suppression. Medication effects can result in 

varying degrees of immune suppression, but 


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most resolve when the medication is ceased. 

However, if you are prescribed medication 

for long-term immune suppression, such as 

after an organ transplant, we will evaluate: 

a. The frequency and severity of infections. 

b. Residuals from the organ transplant 

itself, after the 12-month period has elapsed. 

c. Significant deterioration of other organ 

systems. 

F. How do we evaluate human 

immunodeficiency virus (HIV) infection? Any 

individual with HIV infection, including one 

with a diagnosis of acquired immune 

deficiency syndrome (AIDS), may be found 

disabled under 14.08 if his or her impairment 

meets the criteria in that listing or is 

medically equivalent to the criteria in that 

listing. 

1. Documentation of HIV infection. The 

medical evidence must include 

documentation of HIV infection. 

Documentation may be by laboratory 

evidence or by other generally acceptable 

methods consistent with the prevailing state 

of medical knowledge and clinical practice. 

When you have had laboratory testing for 

HIV infection, we will make every reasonable 

effort to obtain reports of the results of that 

testing. 

a. Documentation of HIV infection by 

definitive diagnosis. A definitive diagnosis of 

HIV infection is documented by one or more 

of the following laboratory tests: 

(i) HIV antibody tests. HIV antibodies are 

usually first detected by an ELISA screening 

test performed on serum. Because the ELISA 

can yield false positive results, confirmation 

is required using a more definitive test, such 

as a Western blot or an immunofluorescence 

assay. 

(ii) Positive ‘‘viral load’’ (VL) tests. These 

tests are normally used to quantitate the 

amount of the virus present but also 

document HIV infection. Such tests include 

the quantitative plasma HIV RNA, 

quantitative plasma HIV branched DNA, and 

reverse transcriptase-polymerase chain 

reaction (RT–PCR). 

(iii) HIV DNA detection by polymerase 

chain reaction (PCR). 

(iv) A specimen that contains HIV antigen 

(for example, serum specimen, lymphocyte 

culture, or cerebrospinal fluid). 

(v) A positive viral culture for HIV from 

peripheral blood mononuclear cells (PBMC). 

(vi) Other tests that are highly specific for 

detection of HIV and that are consistent with 

the prevailing state of medical knowledge. 

b. Other acceptable documentation of HIV 

infection. We may also document HIV 

infection without the definitive laboratory 

evidence described in 14.00F1a, provided 

that such documentation is consistent with 

the prevailing state of medical knowledge 

and clinical practice, and is consistent with 

the other evidence in your case record. If no 

definitive laboratory evidence is available, 

we may document HIV infection by the 

medical history, clinical and laboratory 

findings, and diagnosis(es) indicated in the 

medical evidence. For example, we will 

accept a diagnosis of HIV infection without 

definitive laboratory evidence if you have an 

opportunistic disease that is predictive of a 

defect in cell-mediated immunity (for 

example, toxoplasmosis of the brain, 

Pneumocystis carinii pneumonia (PCP)), and 

there is no other known cause of diminished 

resistance to that disease (for example, longterm steroid treatment, lymphoma). In such 

cases, we will make every reasonable effort 

to obtain full details of the history, medical 

findings, and results of testing. 

2. CD4 tests. Individuals who have HIV 

infection or other disorders of the immune 

system may have tests showing a reduction 

of either the absolute count or the percentage 

of their T-helper lymphocytes (CD4 cells). 

The extent of immune suppression correlates 

with the level or rate of decline of the CD4 

count. Generally, when the CD4 count is 200/ 

mme or less (14 percent or less) the 

susceptibility to opportunistic infection is 

greatly increased. Although a reduced CD4 

count alone does not establish a definitive 

diagnosis of HIV infection, a CD4 count 

below 200 does offer supportive evidence 

when there are clinical findings, but not a 

definitive diagnosis of an opportunistic 

infection(s). However, a reduced CD4 count 

alone does not document the severity or 

functional consequences of HIV infection. 

3. Documentation of the manifestations of 

HIV infection. The medical evidence must 

also include documentation of the 

manifestations of HIV infection. 

Documentation may be by laboratory 

evidence or by other generally acceptable 

methods consistent with the prevailing state 

of medical knowledge and clinical practice. 

When you have had laboratory testing for a 

manifestation of HIV infection, we will make 

every reasonable effort to obtain reports of 

the results of that testing. 

a. Documentation of the manifestations of 

HIV infection by definitive diagnosis. The 

definitive method of diagnosing 

opportunistic diseases or conditions that are 

manifestations of HIV infection is by culture, 

serologic test, or microscopic examination of 

biopsied tissue or other material (for 

example, bronchial washings). We will make 

every reasonable effort to obtain specific 

laboratory evidence of an opportunistic 

disease or other condition whenever this 

information is available. If a histologic or 

other test has been performed, the evidence 

should include a copy of the appropriate 

report. If we cannot obtain the report, the 

summary of hospitalization or a report from 

the treating source should include details of 

the findings and results of the diagnostic 

studies (including appropriate medically 

acceptable imaging studies) or microscopic 

examination of the appropriate tissues or 

body fluids. 

b. Other acceptable documentation of the 

manifestations of HIV infection. We may also 

document manifestations of HIV infection 

without the definitive laboratory evidence 

described in 14.00F3a, provided that such 

documentation is consistent with the 

prevailing state of medical knowledge and 

clinical practice, and is consistent with the 

other evidence in your case record. If no 

definitive evidence is available, we may 

document the manifestations of HIV infection 

with other appropriate evidence. For 

example, many conditions are now 

commonly diagnosed based on some or all of 

the following: Medical history, clinical 

manifestations, laboratory findings 

(including appropriate medically acceptable 

imaging), and treatment responses. In such 

cases, we will make every reasonable effort 

to obtain full details of the history, medical 

findings, and results of testing. 

(i) Although a definitive diagnosis of PCP 

requires identifying the organism in 

bronchial washings, induced sputum, or lung 

biopsy, these tests are frequently bypassed if 

PCP can be diagnosed presumptively. (Note: 

Pneumocystis carinii is now known as 

Pneumocystis jiroveci; however, ‘‘PCP’’

remains in common usage for the pneumonia 

caused by this organism.) Supportive 

evidence includes: Fever, dyspnea, hypoxia, 

and CD4 count below 200. Also supportive 

are bilateral lung interstitial infiltrates on xray, or a typical pattern on CT scan, or a 

gallium scan positive for pulmonary uptake. 

Response to anti-PCP therapy usually 

requires 5–7 days. 

(ii) Documentation of cytomegalovirus 

(CMV) disease (14.08D) may present special 

problems because definitive diagnosis 

(except for chorioretinitis, which may be 

diagnosed by an ophthalmologist on 

funduscopic exam) requires identification of 

viral inclusion bodies or a positive culture 

from the affected organ and the absence of 

any other infectious agent likely to be 

causing the disease. A positive serology test 

identifies a history of infection with CMV, 

but it does not confirm an active disease 

process. Therefore, a presumptive diagnosis 

of CMV disease requires corroborating 

evidence that CMV is causing the disease. 

Supportive evidence includes: Fever, 

positive CMV serology test, urinary culture 

positive for CMV, and CD4 count below 200. 

A clear response to anti-CMV therapy also 

supports a diagnosis. 

(iii) A definitive diagnosis of 

toxoplasmosis of the brain is made by brain 

biopsy, but this procedure carries significant 

risk and is not commonly performed. This 

condition is usually diagnosed 

presumptively based on symptoms or signs of 

fever, headache, focal neurologic deficits, 

seizures, typical lesions on brain imaging, 

and a positive serology test. 

4. Manifestations specific to women. 

a. General. Most women with severe 

immunosuppression secondary to HIV 

infection exhibit the typical opportunistic 

infections and other conditions, such as PCP, 

candida esophagitis, wasting syndrome, 

cryptococcosis, and toxoplasmosis. However, 

HIV infection may have different 

manifestations in women than in men. 

Adjudicators must carefully scrutinize the 

medical evidence and be alert to the variety 

of medical conditions specific to, or common 

in, women with HIV infection that may affect 

their ability to function in the workplace. 

b. Additional considerations for evaluating 

HIV infection in women. Many of these 

manifestations (for example, vulvovaginal 

candidiasis, pelvic inflammatory disease) 

occur in women with or without HIV 

infection, but can be more severe or resistant 

to treatment, or occur more frequently in a 

woman whose immune system is suppressed. 

Therefore, when evaluating the claim of a 

woman with HIV infection, it is important to 

consider gynecologic and other problems 

specific to women, including any associated 


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symptoms (for example, pelvic pain), in 

assessing the severity of the impairment and 

resulting functional limitations. We may 

evaluate manifestations of HIV infection in 

women under the specific criteria (for 

example, cervical cancer under 14.08E), 

under an applicable general category (for 

example, pelvic inflammatory disease under 

14.08A4) or, in appropriate cases, under 

14.08K. 

5. Involuntary weight loss. As used in 

14.08H, ‘‘significant involuntary weight loss’’

does not correspond to a specific minimum 

amount or percentage of weight loss. For 

purposes of this listing, an involuntary 

weight loss of at least 10 percent of baseline 

is always considered significant. Loss of less 

than 10 percent may or may not be 

significant, depending on the individual’s 

baseline weight and body habitus. (For 

example, a 7-pound weight loss in a 100- 

pound woman who is 63 inches tall might be 

considered significant; but a 14-pound 

weight loss in a 200-pound woman who is 

the same height might not be significant.) 

G. How will we consider the effect of 

treatment in evaluating your autoimmune 

disorder, immune deficiency disorder, or HIV 

infection? 

1. General. If your impairment does not 

otherwise meet the requirements of a listing 

we will consider your medical treatment both 

in terms of its effectiveness in improving the 

signs, symptoms, and laboratory 

abnormalities of your specific immune 

system disorder or its manifestations, and in 

terms of any side effects that limit your 

functioning. We will make every reasonable 

effort to obtain a specific description of the 

treatment you receive (including surgery) for 

your immune system disorder. We consider: 

a. The effects of medications you take. 

b. Adverse side effects (acute and chronic). 

c. The intrusiveness and complexity of 

your treatment (for example, the dosing 

schedule, need for injections). 

d. The effect of treatment on your mental 

functioning (for example, cognitive changes, 

mood disturbance). 

e. Variability of your response to treatment 

(see 14.00G2). 

f. The interactive and cumulative effects of 

your treatments. For example, many 

individuals with immune system disorders 

receive treatment both for their immune 

system disorders and for the manifestations 

of the disorders or co-occurring impairments, 

such as treatment for HIV infection and 

hepatitis C. The interactive and cumulative 

effects of these treatments may be greater 

than the effects of each treatment considered 

separately. 

g. The duration of your treatment. 

h. Any other aspects of treatment that may 

interfere with your ability to function. 

2. Variability of your response to treatment. 

Your response to treatment and the adverse 

or beneficial consequences of your treatment 

may vary widely. The effects of your 

treatment may be temporary or long term. For 

example, some individuals may show an 

initial positive response to a drug or 

combination of drugs followed by a decrease 

in effectiveness. When we evaluate your 

response to treatment and how your 

treatment may affect you, we consider such 

factors as disease activity before treatment, 

requirements for changes in therapeutic 

regimes, the time required for therapeutic 

effectiveness of a particular drug or drugs, 

the limited number of drug combinations that 

may be available for your impairment(s), and 

the time-limited efficacy of some drugs. For 

example, an individual with HIV infection or 

another immune deficiency disorder who 

develops pneumonia or tuberculosis may not 

respond to the same antibiotic regimen used 

in treating individuals without these 

disorders or may not respond to an antibiotic 

that he or she responded to before. Therefore, 

we must consider the effects of your 

treatment on an individual basis, including 

the effects of your treatment on your ability 

to function. 

3. How we evaluate the effects of treatment 

for autoimmune disorders on your ability to 

function. Some medications may have acute 

or long-term side effects. When we consider 

the effects of corticosteroids or other 

treatments for autoimmune disorders on your 

ability to function, we consider the factors in 

14.00G1 and 14.00G2. Long-term 

corticosteroid treatment can cause ischemic 

necrosis of bone, posterior subcapsular 

cataract, weight gain, glucose intolerance, 

increased susceptibility to infection, and 

osteoporosis that may result in a loss of 

function. In addition, medications used in 

the treatment of autoimmune disorders may 

also have effects on mental function 

including cognition (for example, memory), 

concentration, and mood. 

4. How we evaluate the effects of treatment 

for immune deficiency disorders, excluding 

HIV infection, on your ability to function. 

When we consider the effects of your 

treatment for your immune deficiency 

disorder on your ability to function, we 

consider the factors in 14.00G1 and 14.00G2. 

A frequent need for treatment such as 

intravenous immunoglobulin and gamma 

interferon therapy can be intrusive and 

interfere with your ability to work on a 

sustained basis. We will also consider 

whether you have chronic side effects from 

these or other medications, including fatigue, 

fever, headaches, high blood pressure, joint 

swelling, muscle aches, nausea, shortness of 

breath, or limitations in mental function 

including cognition (for example, memory), 

concentration, and mood. 

5. How we evaluate the effects of treatment 

for HIV infection on your ability to function. 

a. General. When we consider the effects of 

antiretroviral drugs (including the effects of 

highly active antiretroviral therapy (HAART)) 

and the effects of treatments for the 

manifestations of HIV infection on your 

ability to function, we consider the factors in 

14.00G1 and 14.00G2. Side effects of 

antiretroviral drugs include, but are not 

limited to: Bone marrow suppression, 

pancreatitis, gastrointestinal intolerance 

(nausea, vomiting, diarrhea), neuropathy, 

rash, hepatotoxicity, lipodystrophy, glucose 

intolerance, and lactic acidosis. In addition, 

medications used in the treatment of HIV 

infection may also have effects on mental 

function, including cognition (for example, 

memory), concentration, and mood, and may 

result in malaise, fatigue, joint and muscle 

pain, and insomnia. The symptoms of HIV 

infection and the side effects of medication 

may be indistinguishable from each other. 

We will consider all of your functional 

limitations, whether they result from your 

symptoms of HIV infection or the side effects 

of your treatment. 

b. Structured treatment interruptions. A 

structured treatment interruption (STI, also 

called a ‘‘drug holiday’’) is a treatment 

practice during which your treating source 

advises you to stop taking your medications 

temporarily. An STI in itself does not imply 

that your medical condition has improved or 

that you are noncompliant with your 

treatment because you are following your 

treating source’s advice. Therefore, if you 

have stopped taking medication because your 

treating source prescribed or recommended 

an STI, we will not find that you are failing 

to follow treatment or draw inferences about 

the severity of your impairment on this fact 

alone. We will consider why your treating 

source has prescribed or recommended an 

STI and all the other information in your case 

record when we determine the severity of 

your impairment. 

6. When there is no record of ongoing 

treatment. If you have not received ongoing 

treatment or have not had an ongoing 

relationship with the medical community 

despite the existence of a severe 

impairment(s), we will evaluate the medical 

severity and duration of your immune system 

impairment on the basis of the current 

objective medical evidence and other 

evidence in your case record, taking into 

consideration your medical history, 

symptoms, clinical and laboratory findings, 

and medical source opinions. If you have just 

begun treatment and we cannot determine 

whether you are disabled based on the 

evidence we have, we may need to wait to 

determine the effect of the treatment on your 

ability to function. The amount of time we 

need to wait will depend on the facts of your 

case. If you have not received treatment, you 

may not be able to show an impairment that 

meets the criteria of one of the immune 

system listings, but your immune system 

impairment may medically equal a listing or 

be disabling based on a consideration of your 

residual functional capacity, age, education, 

and work experience. 

H. How do we consider your symptoms, 

including your constitutional symptoms or 

pain? 

Your symptoms, including pain, fatigue, 

and malaise, may be important factors in our 

determination whether your immune system 

disorder(s) meets or medically equals a 

listing or in our determination whether you 

are otherwise able to work. In order for us to 

consider your symptoms, you must have 

medical signs or laboratory findings showing 

the existence of a medically determinable 

impairment(s) that could reasonably be 

expected to produce the symptoms. If you 

have such an impairment(s), we will evaluate 

the intensity, persistence, and functional 

effects of your symptoms using the rules 

throughout 14.00 and in our other 

regulations. See §§ 404.1528, 404.1529, 

416.928, and 416.929. 

I. How do we use the functional criteria in 

these listings? 

1. The following listings in this body 

system include standards for evaluating the 


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limitations resulting from repeated 

manifestations of immune system disorders 

that do not meet the criteria of the other 

sections of their respective listings: 14.02B, 

for systemic lupus erythematosus; 14.03B, for 

systemic vasculitis; 14.04D, for systemic 

sclerosis (scleroderma); 14.05E, for 

polymyositis and dermatomyositis; 14.06B, 

for undifferentiated and mixed connective 

tissue disease; 14.07C, for immune deficiency 

disorders, excluding HIV infection; 14.08K, 

for HIV infection; 14.09D, for inflammatory 

arthritides; and 14.10B, for Sjo ̈ogren’s 

syndrome. 

2. When we use one of the listings cited 

in 14.00I1, we will consider all relevant 

information in your case record to determine 

the full impact of your immune system 

disorder(s) on your ability to function on a 

sustained basis. Important factors we will 

consider when we evaluate your functioning 

under these listings include, but are not 

limited to: Your symptoms, the frequency 

and duration of manifestations of your 

immune system disorder, periods of 

exacerbation and remission, and the 

functional impact of your treatment, 

including the side effects of your medication. 

3. As used in these listings, ‘‘repeated’’

means that the manifestations occur on an 

average of three times a year, or once every 

4 months, each lasting 2 weeks or more; or 

the manifestations do not last for 2 weeks but 

occur substantially more frequently than 

three times in a year or once every 4 months; 

or they occur less frequently than an average 

of three times a year or once every 4 months 

but last substantially longer than 2 weeks. 

4. To satisfy the functional criterion in a 

listing, your immune system disorder must 

result in a marked level of limitation in one 

of three general areas of functioning: 

Activities of daily living, social functioning, 

or difficulties in completing tasks due to 

deficiencies in concentration, persistence, or 

pace. Functional limitation may result from 

the impact of the disease process itself on 

your mental functioning, physical 

functioning, or both your mental and 

physical functioning. This could result from 

persistent or intermittent symptoms, such as 

depression, fatigue, or pain, resulting in a 

limitation of your ability to do a task, to 

concentrate, to persevere at a task, or to 

perform the task at an acceptable rate of 

speed. You may also have limitations 

because of your treatment and its side effects 

(see 14.00G). 

5. When ‘‘marked’’ is used as a standard for 

measuring the degree of functional 

limitation, it means more than moderate but 

less than extreme. We do not define 

‘‘marked’’ by a specific number of different 

activities of daily living in which your 

functioning is impaired, different behaviors 

in which your social functioning is impaired, 

or tasks that you are able to complete, but by 

the nature and overall degree of interference 

with your functioning. You may have a 

marked limitation when several activities or 

functions are impaired, or even when only 

one is impaired. Also, you need not be totally 

precluded from performing an activity to 

have a marked limitation, as long as the 

degree of limitation seriously interferes with 

your ability to function independently, 

appropriately, and effectively. The term 

‘‘marked’’ does not imply that you must be 

confined to bed, hospitalized, or in a nursing 

home. 

6. Activities of daily living include, but are 

not limited to, such activities as doing 

household chores, grooming and hygiene, 

using a post office, taking public 

transportation, or paying bills. We will find 

that you have ‘‘marked’’ limitation of 

activities of daily living if you have a serious 

limitation in your ability to maintain a 

household or take public transportation 

because of symptoms, such as pain, fatigue, 

anxiety, or difficulty concentrating, imposed 

by your immune system disorder (including 

manifestations of the disorder) or its 

treatment, even if you are able to perform 

some self-care activities. 

7. Social functioning includes the capacity 

to interact independently, appropriately, 

effectively, and on a sustained basis with 

others. It includes the ability to communicate 

effectively with others. We will find that you 

have ‘‘marked’’ difficulty maintaining social 

functioning if you have serious limitation in 

social interaction on a sustained basis 

because of symptoms, such as pain, fatigue, 

anxiety, or difficulty concentrating, or a 

pattern of exacerbation and remission, 

caused by your immune system disorder 

(including manifestations of the disorder) or 

its treatment even if you are able to 

communicate with close friends or relatives. 

8. Completing tasks in a timely manner 

involves the ability to sustain concentration, 

persistence, or pace to permit timely 

completion of tasks commonly found in work 

settings. We will find that you have 

‘‘marked’’ difficulty completing tasks if you 

have serious limitation in your ability to 

sustain concentration or pace adequate to 

complete work-related tasks because of 

symptoms, such as pain, fatigue, anxiety, or 

difficulty concentrating, caused by your 

immune system disorder (including 

manifestations of the disorder) or its 

treatment even if you are able to do some 

routine activities of daily living. 

J. How do we evaluate your immune system 

disorder when it does not meet one of these 

listings? 

1. These listings are only examples of 

immune system disorders that we consider 

severe enough to prevent you from doing any 

gainful activity. If your impairment(s) does 

not meet the criteria of any of these listings, 

we must also consider whether you have an 

impairment(s) that satisfies the criteria of a 

listing in another body system. 

2. Individuals with immune system 

disorders, including HIV infection, may 

manifest signs or symptoms of a mental 

impairment or of another physical 

impairment. We may evaluate these 

impairments under any affected body system. 

For example, we will evaluate: 

a. Musculoskeletal involvement, such as 

surgical reconstruction of a joint, under 

1.00ff. 

b. Ocular involvement, such as dry eye, 

under 2.00ff. 

c. Respiratory impairments, such as 

pleuritis, under 3.00ff. 

d. Cardiovascular impairments, such as 

cardiomyopathy, under 4.00ff. 

e. Digestive impairments, such as hepatitis 

(including hepatitis C), under 5.00ff. 

f. Genitourinary impairments, such as 

nephropathy, under 6.00ff. 

g. Hematologic abnormalities, such as 

anemia, granulocytopenia, and 

thrombocytopenia, under 7.00ff. 

h. Skin impairments, such as persistent 

fungal and other infectious skin eruptions, 

and photosensitivity, under 8.00ff. 

i. Neurologic impairments, such as 

neuropathy or seizures, under 11.00ff. 

j. Mental disorders, such as depression, 

anxiety, or cognitive deficits, under 12.00ff. 

k. Allergic disorders, such as asthma or 

atopic dermatitis, under 3.00ff or 8.00ff or 

under the criteria in another affected body 

system. 

l. Syphilis or neurosyphilis under the 

criteria for the affected body system; for 

example, 2.00 Special senses and speech, 

4.00 Cardiovascular system, or 11.00 

Neurological. 

3. If you have a severe medically 

determinable impairment(s) that does not 

meet a listing, we will determine whether 

your impairment(s) medically equals a 

listing. (See §§ 404.1526 and 416.926.) If it 

does not, you may or may not have the 

residual functional capacity to engage in 

substantial gainful activity. Therefore, we 

proceed to the fourth, and if necessary, the 

fifth steps of the sequential evaluation 

process in §§ 404.1520 and 416.920. We use 

the rules in §§ 404.1594, 416.994, and 

416.994a as appropriate, when we decide 

whether you continue to be disabled. 

14.01 Category of Impairments, Immune 

System Disorders 

14.02 Systemic lupus erythematosus. As 

described in 14.00D1. With: 

A. Involvement of two or more organs/ 

body systems, with: 

1. One of the organs/body systems 

involved to at least a moderate level of 

severity, and 

2. At least two of the following 

constitutional symptoms or signs: Severe 

fatigue, fever, malaise, or involuntary weight 

loss. 

OR 

B. Repeated manifestations of SLE but 

without the requisite findings in A, resulting 

in at least two of the constitutional symptoms 

or signs in A2, and one of the following at 

the marked level: 

1. Limitation of activities of daily living. 

2. Limitation in maintaining social 

functioning. 

3. Limitation in completing tasks in a 

timely manner due to deficiencies in 

concentration, persistence, or pace. 

14.03 Systemic vasculitis. As described in 

14.00D2. With: 

A. Involvement of two or more organs/ 

body systems, with: 

1. One of the organs/body systems 

involved to at least a moderate level of 

severity, and 

2. At least two of the following 

constitutional symptoms or signs: Severe 

fatigue, fever, malaise, or involuntary weight 

loss. 

OR 

B. Repeated manifestations of systemic 

vasculitis but without the requisite findings 


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in A, resulting in at least two of the 

constitutional symptoms or signs in A2, and 

one of the following at the marked level: 

1. Limitation of activities of daily living. 

2. Limitation in maintaining social 

functioning. 

3. Limitation in completing tasks in a 

timely manner due to deficiencies in 

concentration, persistence, or pace. 

14.04 Systemic sclerosis (scleroderma). 

As described in 14.00D3. With: 

A. Involvement of two or more organs/ 

body systems, with: 

1. One of the organs/body systems 

involved to at least a moderate level of 

severity, and 

2. At least two of the following 

constitutional symptoms or signs: Severe 

fatigue, fever, malaise, or involuntary weight 

loss. 

OR 

B. With one of the following: 

1. Toe contractures or fixed deformity of 

one or both feet, resulting in the inability to 

ambulate effectively as defined in 14.00C6; or 

2. Finger contractures or fixed deformity in 

both hands, resulting in the inability to 

perform fine and gross movements effectively 

as defined in 14.00C7; or 

3. Atrophy with irreversible damage in one 

or both lower extremities, resulting in the 

inability to ambulate effectively as defined in 

14.00C6; or 

4. Atrophy with irreversible damage in 

both upper extremities, resulting in the 

inability to perform fine and gross 

movements effectively as defined in 14.00C7. 

OR 

C. Raynaud’s phenomenon, characterized 

by: 

1. Gangrene of a toe or finger in at least two 

extremities, or of a toe and finger; or 

2. Ischemia with ulcerations of toes or 

fingers, resulting in the inability to ambulate 

effectively or to perform fine and gross 

movements effectively as defined in 14.00C6 

and 14.00C7; or 

D. Repeated manifestations of systemic 

sclerosis (scleroderma) but without the 

requisite findings in A, B, or C, resulting in 

at least two of the constitutional symptoms 

or signs in A2, and one of the following at 

the marked level: 

1. Limitation of activities of daily living. 

2. Limitation in maintaining social 

functioning. 

3. Limitation in completing tasks in a 

timely manner due to deficiencies in 

concentration, persistence, or pace. 

14.05 Polymyositis and dermatomyositis. 

As described in 14.00D4. With: 

A. Proximal limb-girdle (pelvic or 

shoulder) muscle weakness, resulting in 

inability to ambulate effectively or inability 

to perform fine and gross movements 

effectively as defined in 14.00C6 and 

14.00C7. 

OR 

B. Impaired swallowing (dysphagia) with 

aspiration due to muscle weakness. 

OR 

C. Impaired respiration due to intercostal 

and diaphragmatic muscle weakness. 

OR 

D. Diffuse calcinosis with limitation of 

joint mobility or intestinal motility. 

OR 

E. Repeated manifestations of polymyositis 

or dermatomyositis but without the requisite 

findings in A, B, or C, resulting in at least 

two of the following constitutional symptoms 

or signs: Severe fatigue, fever, malaise, or 

involuntary weight loss, and one of the 

following at the marked level: 

1. Limitation of activities of daily living. 

2. Limitation in maintaining social 

functioning. 

3. Limitation in completing tasks in a 

timely manner due to deficiencies in 

concentration, persistence, or pace. 

14.06 Undifferentiated and mixed 

connective tissue disease. As described in 

14.00D5. With: 

A. Involvement of two or more organs/ 

body systems, with: 

1. One of the organs/body systems 

involved to at least a moderate level of 

severity, and 

2. At least two of the following 

constitutional symptoms or signs: Severe 

fatigue, fever, malaise, or involuntary weight 

loss. 

OR 

B. Repeated manifestations of 

undifferentiated or mixed connective tissue 

disease but without the requisite findings in 

A, resulting in at least two of the 

constitutional symptoms or signs in A2, and 

one of the following at the marked level: 

1. Limitation of activities of daily living. 

2. Limitation in maintaining social 

functioning. 

3. Limitation in completing tasks in a 

timely manner due to deficiencies in 

concentration, persistence, or pace. 

14.07 Immune deficiency disorders, 

excluding HIV infection. As described in 

14.00E. With: 

A. One or more of the following infections. 

The infection(s) must either be resistant to 

treatment, or require hospitalization or 

intravenous treatment three or more times in 

a 12-month period. 

1. Sepsis; or 

2. Meningitis; or 

3. Pneumonia; or 

4. Septic arthritis; or 

5. Endocarditis; or 

6. Sinusitis documented by appropriate 

medically acceptable imaging. 

OR 

B. Stem cell transplantation as described 

under 14.00E3. Consider under a disability 

until at least 12 months from the date of 

transplantation. Thereafter, evaluate any 

residual impairment(s) under the criteria for 

the affected body system. 

OR 

C. Repeated manifestations of an immune 

deficiency disorder but without the requisite 

findings in A or B, resulting in at least two 

of the following constitutional symptoms or 

signs: Severe fatigue, fever, malaise, or 

involuntary weight loss, and one of the 

following at the marked level: 

1. Limitation of activities of daily living. 

2. Limitation in maintaining social 

function. 

3. Limitation in completing tasks in a 

timely manner due to deficiencies in 

concentration, persistence, or pace. 

14.08 Human immunodeficiency virus 

(HIV) infection. With documentation as 

described in 14.00F and one of the following: 

A. Bacterial infections: 

1. Mycobacterial infection (for example, 

caused by M. avium-intracellulare, M. 

kansasii, or M. tuberculosis) at a site other 

than the lungs, skin, or cervical or hilar 

lymph nodes, or pulmonary tuberculosis 

resistant to treatment; or 

2. Nocardiosis; or 

3. Salmonella bacteremia, recurrent nontyphoid; or 

4. Multiple or recurrent bacterial 

infection(s), including pelvic inflammatory 

disease, requiring hospitalization or 

intravenous antibiotic treatment three or 

more times in a 12-month period. 

OR 

B. Fungal Infections: 

1. Aspergillosis; or 

2. Candidiasis involving the esophagus, 

trachea, bronchi, or lungs, or at another site 

other than the skin, urinary tract, intestinal 

tract, or oral or vulvovaginal mucous 

membranes; or 

3. Coccidioidomycosis, at a site other than 

the lungs or lymph nodes; or 

4. Cryptococcosis, at a site other than the 

lungs (for example, cryptococcal meningitis); 

or 

5. Histoplasmosis, at a site other than the 

lungs or lymph nodes; or 

6. Mucormycosis; or 

7. Pneumocystis carinii (jiroveci) 

pneumonia or extrapulmonary pneumocystis 

carinii (jiroveci) infection. 

OR 

C. Protozoan or helminthic infections: 

1. Cryptosporidiosis, isosporiasis, or 

microsporidiosis, with diarrhea lasting for 1 

month or longer; or 

2. Strongyloidiasis, extra-intestinal; or 

3. Toxoplasmosis of an organ other than 

the liver, spleen, or lymph nodes. 

OR 

D. Viral infections: 

1. Cytomegalovirus disease (documented as 

described in 14.00F3b(ii)) at a site other than 

the liver, spleen or lymph nodes; or 

2. Herpes simplex virus causing: 

a. Mucocutaneous infection (for example, 

oral, genital, perianal) lasting for 1 month or 

longer; or 

b. Infection at a site other than the skin or 

mucous membranes (for example, bronchitis, 

pneumonitis, esophagitis, or encephalitis); or 

c. Disseminated infection; or 

3. Herpes zoster: 

a. Disseminated; or 

b. With multidermatomal eruptions that 

are resistant to treatment; or 

4. Progressive multifocal 

leukoencephalopathy. 

OR 

E. Malignant neoplasms: 

1. Carcinoma of the cervix, invasive, FIGO 

stage II and beyond; or 

2. Kaposi’s sarcoma with: 

a. Extensive oral lesions; or 

b. Involvement of the gastrointestinal tract, 

lungs, or other visceral organs; or 


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3. Lymphoma (for example, primary 

lymphoma of the brain, Burkitt’s lymphoma, 

immunoblastic sarcoma, other non-Hodgkin’s 

lymphoma, Hodgkin’s disease); or 

4. Squamous cell carcinoma of the anus. 

OR 

F. Conditions of the skin or mucous 

membranes (other than described in B2, D2, 

or D3, above), with extensive fungating or 

ulcerating lesions not responding to 

treatment (for example, dermatological 

conditions such as eczema or psoriasis, 

vulvovaginal or other mucosal candida, 

condyloma caused by human papillomavirus, 

genital ulcerative disease). 

OR 

G. HIV encephalopathy, characterized by 

cognitive or motor dysfunction that limits 

function and progresses. 

OR 

H. HIV wasting syndrome, characterized by 

involuntary weight loss of 10 percent or more 

of baseline (or other significant involuntary 

weight loss, as described in 14.00F5) and, in 

the absence of a concurrent illness that could 

explain the findings, either: 

1. Chronic diarrhea with two or more loose 

stools daily lasting for 1 month or longer; or 

2. Chronic weakness and documented fever 

greater than 38 °C (100.4 °F) for the majority 

of 1 month or longer. 

OR 

I. Diarrhea, lasting for 1 month or longer, 

resistant to treatment, and requiring 

intravenous hydration, intravenous 

alimentation, or tube feeding. 

OR 

J. One or more of the following infections 

(other than described in A–I, above). The 

infection(s) must either be resistant to 

treatment, or require hospitalization or 

intravenous treatment three or more times in 

a 12-month period. 

1. Sepsis; or 

2. Meningitis; or 

3. Pneumonia; or 

4. Septic arthritis; or 

5. Endocarditis; or 

6. Sinusitis documented by appropriate 

medically acceptable imaging. 

OR 

K. Repeated (as defined in 14.00I3) 

manifestations of HIV infection, including 

those listed in 14.08A–J, but without the 

requisite findings for those listings (for 

example, carcinoma of the cervix not meeting 

the criteria in 14.08E, diarrhea not meeting 

the criteria in 14.08I), or other manifestations 

(for example, oral hairy leukoplakia, 

myositis, pancreatitis, hepatitis, peripheral 

neuropathy, glucose intolerance, muscle 

weakness, cognitive or other mental 

impairment) resulting in significant, 

documented symptoms or signs (for example, 

fatigue, fever, malaise, involuntary weight 

loss, pain, night sweats, nausea, vomiting, 

headaches, or insomnia) and one of the 

following at the marked level (as defined in 

14.00I5): 

1. Limitation of activities of daily living. 

2. Limitation in maintaining social 

functioning. 

3. Limitation in completing tasks in a 

timely manner due to deficiencies in 

concentration, persistence, or pace. 

14.09 Inflammatory arthritis. As 

described in 14.00D6. With: 

A. Persistent inflammation or deformity in 

two or more major peripheral joints resulting 

in the inability to ambulate effectively or 

inability to perform fine and gross 

movements effectively as defined in 14.00C6 

and 14.00C7. 

OR 

B. Inflammation or deformity in one or 

more major peripheral joints, but with less 

joint involvement than in A and extraarticular features that do not satisfy the 

criteria of a listing, with: 

1. Involvement of two or more organs/body 

systems with one of the organs/body systems 

involved to at least a moderate level of 

severity, and 

2. At least two of the following 

constitutional symptoms or signs: Severe 

fatigue, fever, malaise, or involuntary weight 

loss. 

OR 

C. Ankylosing spondylitis or other 

spondyloarthropathies, with: 

1. Ankylosis (fixation) of the dorsolumbar 

or cervical spines as shown by appropriate 

medically acceptable imaging and measured 

on physical examination at 45° or more of 

flexion from the vertical position (zero 

degrees); or 

2. Ankylosis (fixation) of the dorsolumbar 

or cervical spine as shown by appropriate 

medically acceptable imaging and measured 

on physical examination at 30° or more of 

flexion (but less than 45°) measured from the 

vertical position (zero degrees), and 

involvement of two or more organs/body 

systems with one of the organs/body systems 

involved to at least a moderate level of 

severity. 

OR 

D. Repeated manifestations of 

inflammatory arthritis but without the 

requisite findings in A, B, or C, resulting in 

at least two of the constitutional symptoms 

or signs in B2, and one of the following at 

the marked level: 

1. Limitation of activities of daily living. 

2. Limitation in maintaining social 

functioning. 

3. Limitation in completing tasks in a 

timely manner due to deficiencies in 

concentration, persistence, or pace. 

14.10 Sjo ̈ gren’s syndrome. As described in 

14.00D7. With: 

A. Involvement of two or more organs/ 

body systems, with: 

1. One of the organs/body systems 

involved to at least a moderate level of 

severity, and 

2. At least two of the following 

constitutional symptoms or signs: Severe 

fatigue, fever, malaise, or involuntary weight 

loss. 

OR 

B. Repeated manifestations of Sjo ̈gren’s 

syndrome but without the requisite findings 

in A, resulting in at least two of the 

constitutional symptoms or signs in A2, and 

one of the following at the marked level: 

1. Limitation of activities of daily living. 

2. Limitation in maintaining social 

functioning. 

3. Limitation in completing tasks in a 

timely manner due to deficiencies in 

concentration, persistence, or pace. 

* * * * * 

Part B 

* * * * * 

101.00 Musculoskeletal System 

* * * * * 

B. Loss of function. 

1. General. * * * For inflammatory 

arthritides that result in loss of function 

because of inflammatory peripheral joint or 

axial arthritis or sequelae, or because of 

extra-articular features, see 114.00D6. * * * 

* * * * * 

L. Abnormal curvatures of the spine. * * * 

When the abnormal curvature of the spine 

results in symptoms related to fixation of the 

dorsolumbar or cervical spine, evaluation of 

equivalence may be made by reference to 

114.09C. * * * 

* * * * * 

108.00 Skin Disorders 

* * * * * 

D. How do we assess impairments that may 

affect the skin and other body systems? 

* * * * * 

3. Autoimmune disorders and other 

immune system disorders (for example, 

systemic lupus erythematosus, scleroderma, 

human immunodeficiency virus (HIV) 

infection, and Sjo ̈gren’s syndrome) often 

involve more than one body system. We first 

evaluate these disorders under the immune 

system listings in 114.00. We evaluate lupus 

erythematosus under 114.02, scleroderma 

under 114.04, symptomatic HIV infection 

under 114.08, and Sjo ̈gren’s syndrome under 

114.10. 

* * * * * 

114.00 Immune System Disorders 

A. What disorders do we evaluate under 

the immune system listings? 

1. We evaluate immune system disorders 

that cause dysfunction in one or more 

components of your immune system. 

a. These listings are examples of immune 

system disorders that are severe enough to 

result in marked and severe functional 

limitations. The dysfunction may be due to 

problems in antibody production, impaired 

cell-mediated immunity, a combined type of 

antibody/cellular deficiency, impaired 

phagocytosis, or complement deficiency. 

b. Immune system disorders may result in 

recurrent and unusual infections, or 

inflammation and dysfunction of the body’s 

own tissues. Immune system disorders can 

cause a deficit in a single organ or body 

system that results in extreme (that is, very 

serious) loss of function. They can also cause 

lesser degrees of limitations in two or more 

organs or body systems, and when associated 

with symptoms or signs such as fatigue, 

fever, malaise, diffuse musculoskeletal pain, 

or involuntary weight loss, can also result in 

extreme limitation. In children, immune 

system disorders or their treatment may also 

affect growth, development, and performance 

of age-appropriate activities. 


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c. In this preface, we organize the 

discussions of immune system disorders in 

three categories: Autoimmune disorders; 

Immune deficiency disorders, excluding 

human immunodeficiency virus (HIV) 

infection; and HIV infection. 

2. Autoimmune disorders (114.00D). 

Autoimmune disorders are caused by 

dysfunctional immune responses directed 

against the body’s own tissues, resulting in 

chronic, multisystem impairments that differ 

in clinical manifestations, course, and 

outcome. They are sometimes referred to as 

rheumatic diseases, connective tissue 

disorders, or collagen vascular disorders. 

Some of the features of autoimmune 

disorders in children differ from the features 

of the same disorders in adults. The impact 

of the disorders or their treatment on 

physical, psychological, and developmental 

growth of pre-pubertal children may be 

considerable, and often differs from that of 

post-pubertal adolescents or adults. 

3. Immune deficiency disorders, excluding 

HIV infection (114.00E). Immune deficiency 

disorders are characterized by recurrent or 

unusual infections that respond poorly to 

treatment, and are often associated with 

complications affecting other parts of the 

body. Immune deficiency disorders are 

classified as either primary (congenital) or 

acquired. Children with immune deficiency 

disorders also have an increased risk of 

malignancies and of having autoimmune 

disorders. 

4. Human immunodeficiency virus (HIV) 

infection (114.00F). HIV infection is caused 

by a specific retrovirus and may be 

characterized by increased susceptibility to 

opportunistic infections, cancers, or other 

conditions as described in 114.08. 

B. What information do we need to show 

that you have an immune system disorder? 

Generally, we need your medical history, 

report(s) of physical examination, report(s) of 

laboratory findings, and in some instances, 

appropriate medically acceptable imaging or 

tissue biopsy reports to show that you have 

an immune system disorder. Therefore, we 

will make every reasonable effort to obtain 

your medical history, medical findings, and 

results of laboratory tests. We explain the 

information we need in more detail in the 

sections below. 

C. Definitions 

1. Appropriate medically acceptable 

imaging includes, but is not limited to, 

angiography, x-ray imaging, computerized 

axial tomography (CAT scan) or magnetic 

resonance imaging (MRI), with or without 

contrast material, myelography, and 

radionuclear bone scans. ‘‘Appropriate’’

means that the technique used is one that is 

generally accepted and consistent with the 

prevailing state of medical knowledge and 

clinical practice to support the evaluation 

and diagnosis of the impairment. 

2. Constitutional symptoms or signs means 

fatigue, fever, malaise, or involuntary weight 

loss. Severe fatigue means a frequent sense of 

exhaustion that results in significantly 

reduced physical activity or mental function. 

Malaise means frequent feelings of illness, 

bodily discomfort, or lack of well-being that 

result in significantly reduced physical 

activity or mental function. 

3. Disseminated means that a condition is 

spread over a considerable area. The type and 

extent of the spread will depend on your 

specific disease. 

4. Dysfunction means that one or more of 

the body regulatory mechanisms are 

impaired, causing either an excess or 

deficiency of immunocompetent cells or their 

products. 

5. Extra-articular means ‘‘other than the 

joints’’; for example, the effect is in an 

organ(s) such as the heart, lungs, kidneys, or 

skin. 

6. Inability to ambulate effectively has the 

same meaning as in 101.00B2b. 

7. Inability to perform fine and gross 

movements effectively has the same meaning 

as in 101.00B2c. 

8. Major peripheral joints has the same 

meaning as in 101.00F. 

9. Persistent means that a sign(s) or 

symptom(s) has continued over time. The 

precise meaning will depend on the specific 

immune system disorder, the usual course of 

the disorder, and the other circumstances of 

your clinical course. 

10. Recurrent means that a condition that 

previously responded adequately to an 

appropriate course of treatment returns after 

a period of remission or regression. The 

precise meaning, such as the extent of 

response or remission and the time periods 

involved, will depend on the specific disease 

or condition you have, the body system 

affected, the usual course of the disorder and 

its treatment, and the other facts of your 

particular case. 

11. Resistant to treatment means that a 

condition did not respond adequately to an 

appropriate course of treatment. Whether a 

response is adequate or a course of treatment 

is appropriate will depend on the specific 

disease or condition you have, the body 

system affected, the usual course of the 

disorder and its treatment, and the other facts 

of your particular case. 

12. Severe describes medical severity as 

used by the medical community. The term 

does not have the same meaning as it does 

when we use it in connection with a finding 

at the second step of the sequential 

evaluation process in § 416.924. 

D. What are the listed autoimmune 

disorders in these listings? 

1. Systemic lupus erythematosus (114.02). 

a. General. Systemic lupus erythematosus 

(SLE) is a chronic inflammatory disease that 

can affect any organ or body system. It is 

frequently, but not always, accompanied by 

constitutional symptoms or signs (fatigue, 

fever, malaise, involuntary weight loss). 

Major organ or body system involvement can 

include: Respiratory (pleuritis, pneumonitis), 

cardiovascular (endocarditis, myocarditis, 

pericarditis, vasculitis), renal 

(glomerulonephritis), hematologic (anemia, 

leukopenia, thrombocytopenia), skin 

(photosensitivity), neurologic (seizures), 

mental (anxiety, fluctuating cognition 

(‘‘lupus fog’’), mood disorders, organic brain 

syndrome, psychosis), or immune system 

(inflammatory arthritis) disorders. 

Immunologically, there is an array of 

circulating serum auto-antibodies and proand anti-coagulant proteins that may occur in 

a highly variable pattern. 

b. Documentation of SLE. Generally, but 

not always, the medical evidence will show 

that your SLE satisfies the criteria in the 

current ‘‘Criteria for the Classification of 

Systemic Lupus Erythematosus’’ by the 

American College of Rheumatology found in 

the most recent edition of the Primer on the 

Rheumatic Diseases published by the 

Arthritis Foundation. 

2. Systemic vasculitis (114.03). 

a. General. (i) Vasculitis is an inflammation 

of blood vessels. It may occur acutely in 

association with adverse drug reactions, 

certain chronic infections, and occasionally, 

malignancies. More often, it is chronic and 

the cause is unknown. Symptoms vary 

depending on which blood vessels are 

involved. Systemic vasculitis may also be 

associated with other autoimmune disorders; 

for example, SLE or dermatomyositis. 

(ii) Children can develop the vasculitis of 

Kawasaki disease, of which the most serious 

manifestation is formation of coronary artery 

aneurysms and related complications. We 

evaluate heart problems related to Kawasaki 

disease under the criteria in the 

cardiovascular listings (104.00ff). Children 

can also develop the vasculitis of 

anaphylactoid purpura (Henoch-Schoenlein 

purpura), which may cause intestinal and 

renal disorders. We evaluate intestinal and 

renal disorders related to vasculitis of 

anaphylactoid purpura under the criteria in 

the digestive (105.00ff) or genitourinary 

(106.00ff) listings. Other clinical patterns 

include, but are not limited to, polyarteritis 

nodosa, Takayasu’s arteritis (aortic arch 

arteritis), and Wegener’s granulomatosis. 

b. Documentation of systemic vasculitis. 

Angiography or tissue biopsy confirms a 

diagnosis of systemic vasculitis when the 

disease is suspected clinically. Usually the 

results will be in your medical records. 

3. Systemic sclerosis (scleroderma) 

(114.04). 

a. General. Systemic sclerosis 

(scleroderma) constitutes a spectrum of 

disease in which thickening of the skin is the 

clinical hallmark. Raynaud’s phenomenon, 

often medically severe and progressive, is 

present frequently and may be the peripheral 

manifestation of a vasospastic abnormality in 

the heart, lungs, and kidneys. The CREST 

syndrome (calcinosis, Raynaud’s 

phenomenon, esophageal dysmotility, 

sclerodactyly, and telangiectasia) is a variant 

that may slowly progress over years to the 

generalized process, systemic sclerosis. 

b. Diffuse cutaneous systemic sclerosis. In 

diffuse cutaneous systemic sclerosis (also 

known as diffuse scleroderma), major organ 

or systemic involvement can include the 

gastrointestinal tract, lungs, heart, kidneys, 

and muscle in addition to skin or blood 

vessels. Although arthritis can occur, joint 

dysfunction results primarily from soft 

tissue/cutaneous thickening, fibrosis, and 

contractures. 

c. Localized scleroderma (linear 

scleroderma and morphea). 

(i) Localized scleroderma (linear 

scleroderma and morphea) is more common 

in children than systemic scleroderma. The 

extent of involvement of linear scleroderma 

and a description of the lesions are important 

in assessing the severity of the impairment. 


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For example, linear scleroderma involving 

the arm but not crossing any joints is not as 

functionally limiting as sclerodactyly 

(scleroderma localized to the fingers). Linear 

scleroderma of a lower extremity involving 

skin thickening and atrophy of underlying 

muscle or bone can result in contracture(s) 

and leg length discrepancies. In such cases, 

evaluation under the musculoskeletal 

(101.00ff) listings may be appropriate. 

(ii) When there is isolated morphea of the 

face causing facial disfigurement from 

unilateral hypoplasia of the mandible, 

maxilla, zygoma, or orbit, adjudication may 

be more appropriate under the criteria in the 

special senses listings (102.00ff) or mental 

disorders listings (112.00ff). 

(iii) Chronic variants of these syndromes 

include disseminated morphea, Shulman’s 

disease (diffuse fasciitis with eosinophilia), 

and eosinophilia-myalgia syndrome (often 

associated with toxins such as toxic oil or 

contaminated tryptophan), all of which can 

impose medically severe musculoskeletal 

impairment and may also lead to restrictive 

pulmonary disease. We evaluate these 

variants of the disease under the criteria in 

the musculoskeletal listings (101.00ff) or 

respiratory system listings (103.00ff). 

d. Documentation of systemic sclerosis 

(scleroderma). Documentation involves 

differentiating the clinical features of 

systemic sclerosis (scleroderma) from other 

autoimmune disorders; however, there may 

be an overlap. 

4. Polymyositis and dermatomyositis 

(114.05). 

a. General. 

(i) Polymyositis and dermatomyositis are 

related disorders that are characterized by an 

inflammatory process in striated muscle, 

occurring alone or in association with other 

autoimmune disorders. Symmetric weakness, 

and less frequently pain and tenderness of 

the proximal limb-girdle (shoulder or pelvic) 

musculature, are the most common 

manifestations. There may also be 

involvement of the cervical, cricopharyngeal, 

esophageal, intercostal, and diaphragmatic 

muscles. 

(ii) Polymyositis occurs rarely in children; 

the more common presentation in children is 

dermatomyositis with symmetric proximal 

muscle weakness and characteristic skin 

rash. The clinical course of dermatomyositis 

can be more severe when it is accompanied 

by systemic vasculitis rather than just 

localized to striated muscle. Late in the 

disease, some children with dermatomyositis 

develop calcinosis of the skin and 

subcutaneous tissues, muscles and joints. We 

evaluate the involvement of other organs/ 

body systems under the criteria for the 

listings in the affected body system. 

b. Documentation of polymyositis and 

dermatomyositis. Generally, but not always, 

polymyositis is associated with elevated 

serum muscle enzymes (creatine 

phosphokinase (CPK), aminotransferases, 

aldolase), and characteristic abnormalities on 

electromyography and muscle biopsy. In 

children, the diagnosis of dermatomyositis is 

supported largely by medical history, 

findings on physical examination that 

include the characteristic skin rash, and 

elevated serum muscle enzymes. Additional 

evidence of the diagnosis of childhood 

dermatomyositis is depiction on MRI of 

muscle inflammation or vasculitis. 

c. Additional information about how we 

evaluate polymyositis and dermatomyositis 

under the listings. 

(i) In newborn and younger infants (birth 

to attainment of age 1), we consider muscle 

weakness that affects motor skills, such as 

head control, reaching, grasping, taking 

solids, or self-feeding under 114.05A. In 

older infants and toddlers (age 1 to 

attainment of age 3), we also consider muscle 

weakness affecting the child’s ability to roll 

over, sit, crawl, or walk under 114.05A. 

(ii) If you are of preschool age through 

adolescence (age 3 to attainment of age 18), 

weakness of your pelvic girdle muscles that 

results in your inability to rise independently 

from a squatting or sitting position or to 

climb stairs may be an indication that you are 

unable to ambulate effectively. Weakness of 

your shoulder girdle muscles may result in 

your inability to perform lifting, carrying, 

and reaching overhead, and also may 

seriously affect your ability to perform 

activities requiring fine movements. We 

evaluate these limitations under 114.05A. 

5. Undifferentiated and mixed connective 

tissue disease (114.06). 

a. General. This listing includes syndromes 

with clinical and immunologic features of 

several autoimmune disorders, but which do 

not satisfy the criteria for any of the specific 

disorders described. For example, you may 

have clinical features of systemic lupus 

erythematosus and systemic vasculitis, and 

the serologic (blood test) findings of 

rheumatoid arthritis. The most common 

pattern of undifferentiated autoimmune 

disorders in children is mixed connective 

tissue disease (MCTD). 

b. Documentation of undifferentiated and 

mixed connective tissue disease. 

Undifferentiated connective tissue disease is 

diagnosed when clinical features and 

serologic (blood test) findings, such as 

rheumatoid factor or antinuclear antibody 

(consistent with an autoimmune disorder) are 

present but do not satisfy the criteria for a 

specific disease. Children with MCTD have 

laboratory findings of extremely high 

antibody titers to extractable nuclear antigen 

(ENA) or ribonucleoprotein (RNP) without 

high titers of anti-dsDNA or anti-SM 

antibodies. There are often clinical findings 

suggestive of SLE or childhood 

dermatomyositis. Many children later 

develop features of scleroderma. 

6. Inflammatory arthritis (114.09). 

a. General. The inflammatory arthritides 

include a vast array of disorders that differ 

in cause, course, and outcome. Clinically, 

inflammation of major peripheral joints may 

be the dominant manifestation causing 

difficulties with ambulation or fine and gross 

movements; there may be joint pain, 

swelling, and tenderness. The arthritis may 

affect other joints, or cause less functional 

limitations in ambulation or performance of 

fine and gross movements. However, in 

combination with extra-articular features, 

including constitutional symptoms or signs 

(fatigue, fever, malaise, involuntary weight 

loss), inflammatory arthritis may result in an 

extreme limitation. You may also have 

impaired growth as a result of the 

inflammatory arthritides because of its effects 

on the immature skeleton, open epiphyses, 

and young cartilage and bone. We evaluate 

any associated growth impairment under the 

criteria in 100.00ff. 

b. Inflammatory arthritides involving the 

axial spine (spondyloarthropathies). In 

children, inflammatory arthritides involving 

the axial spine may be associated with 

heterogeneous disorders such as: 

(i) Reactive arthropathies; 

(ii) Juvenile ankylosing spondylitis; 

(iii) Psoriatic arthritis; 

(iv) SEA syndrome (seronegative 

enthesopathy arthropathy syndrome); 

(v) Behc ̧et’s disease; and 

(vi) Inflammatory bowel disease. 

c. Inflammatory arthritides involving the 

peripheral joints. In children, the 

inflammatory arthropathies involving 

peripheral joints may be associated with 

disorders such as: 

(i) Juvenile rheumatoid arthritis; 

(ii) Sjo ̈gren’s syndrome; 

(iii) Psoriatic arthritis; 

(iv) Crystal deposition disorders (gout and 

pseudogout); 

(v) Lyme disease; and 

(vi) Inflammatory bowel disease. 

d. Documentation of inflammatory 

arthritides. Generally, but not always, the 

diagnosis of inflammatory arthritis is made 

by the clinical features and serologic findings 

described in the most recent edition of the 

Primer on Rheumatic Diseases published by 

the Arthritis Foundation. 

e. How we evaluate the inflammatory 

arthritides under the listings. 

(i) Listing-level severity in 114.09A and 

114.09C1 is shown by an impairment that 

results in an ‘‘extreme’’ (very serious) 

limitation. In 114.09A, the criterion is 

satisfied with persistent inflammation or 

deformity in two or more major peripheral 

joints resulting in the inability to ambulate 

effectively or inability to perform fine and 

gross movements effectively, as defined in 

114.00C6 and 114.00C7. In 114.09C1, if you 

have the required ankylosis (fixation) of your 

cervical or dorsolumbar spine, we will find 

that you have an extreme limitation in your 

ability to see in front of you, above you, and 

to the side. Therefore, inability to ambulate 

effectively is implicit in 114.09C1, even 

though you might not require bilateral upper 

limb assistance. 

(ii) Listing-level severity is shown in 

114.09B, 114.09C2, and 114.09D when the 

arthritis does not result in the extreme 

limitation in 114.09A or 114.09C1, involves 

one or more major peripheral joints, or 

involves other joints, but is complicated by 

extra-articular features that cumulatively 

result in an ‘‘extreme’’ (very serious) 

limitation or ‘‘marked’’ (serious) limitations 

in at least two areas of functioning. Extraarticular impairments may also meet listings 

in other body systems. 

(iii) Extra-articular features of 

inflammatory arthritis may involve any body 

system. Commonly occurring extra-articular 

impairments include: Musculoskeletal (heel 

enthesopathy), ophthalmologic (iridocyclitis, 

keratoconjunctivitis sicca, uveitis), 

pulmonary (pleuritis, pulmonary fibrosis or 


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nodules, restrictive lung disease), 

cardiovascular (aortic valve insufficiency, 

arrhythmias, coronary arteritis, myocarditis, 

pericarditis, Raynaud’s phenomenon, 

systemic vasculitis), renal (amyloidosis of the 

kidney), hematologic (chronic anemia, 

thrombocytopenia), neurologic (peripheral 

neuropathy, radiculopathy, spinal cord or 

cauda equina compression with sensory and 

motor loss), and immune system (Felty’s 

syndrome (hypersplenism with compromised 

immune competence)) disorders. 

(iv) If permanent deformity of a major 

peripheral joint is the dominant feature of 

your impairment, we evaluate your 

impairment under 101.02. 

(v) If there has been surgical reconstruction 

of a major weight-bearing joint, we evaluate 

your impairment under 101.03. 

(vi) If both inflammation and chronic 

deformities are present, we evaluate your 

impairment under the criteria of any 

appropriate listing. 

7. Sjo ̈ gren’s syndrome (114.10). 

a. General. (i) Sjo ̈gren’s syndrome is an 

immune-mediated disorder of the exocrine 

glands. Involvement of the lacrimal and 

salivary glands is the hallmark feature, 

resulting in symptoms of dry eyes and dry 

mouth, and possible complications such as 

corneal damage, blepharitis (eyelid 

inflammation), dysphagia (difficulty in 

swallowing), dental caries, and the inability 

to speak for extended periods of time. 

Involvement of the exocrine glands of the 

upper airways may result in persistent dry 

cough. 

(ii) Many other organ systems may be 

involved, including musculoskeletal 

(arthritis, myositis), respiratory (interstitial 

fibrosis), gastrointestinal (dysmotility, 

dysphagia, involuntary weight loss), 

genitourinary (interstitial cystitis, renal 

tubular acidosis), skin (purpura, vasculitis,), 

neurologic (central nervous system disorders, 

cranial and peripheral neuropathies), mental 

(cognitive dysfunction, poor memory), and 

neoplastic (lymphoma). Fatigue and malaise 

are frequently reported. Sjo ̈gren’s syndrome 

may be associated with other autoimmune 

disorders (for example, rheumatoid arthritis 

or SLE); usually the clinical features of the 

associated disorder predominate. 

b. Documentation of Sjo ̈ gren’s syndrome. If 

you have Sjo ̈gren’s syndrome, the medical 

evidence will generally, but not always, show 

that your disease satisfies the criteria in the 

current ‘‘Criteria for the Classification of 

Sjo ̈gren’s Syndrome’’ by the American 

College of Rheumatology found in the most 

recent edition of the Primer on the 

Rheumatic Diseases published by the 

Arthritis Foundation. 

E. How do we evaluate immune deficiency 

disorders, excluding HIV infection (114.07)? 

1. General. 

a. Immune deficiency disorders can be 

classified as: 

(i) Primary (congenital); for example, Xlinked agammaglobulinemia, thymic 

hypoplasia (DiGeorge syndrome), severe 

combined immunodeficiency (SCID), chronic 

granulomatous disease (CGD), C1 esterase 

inhibitor deficiency. 

(ii) Acquired; for example, medicationrelated. 

b. Primary immune deficiency disorders 

are seen mainly in children. However, recent 

advances in the treatment of these disorders 

have allowed many affected children to 

survive well into adulthood. Occasionally, 

these disorders are first diagnosed in 

adolescence or adulthood. 

2. Documentation of immune deficiency 

disorders. The medical evidence must 

include documentation of the specific type of 

immune deficiency. Documentation may be 

by laboratory evidence or by other generally 

acceptable methods consistent with the 

prevailing state of medical knowledge and 

clinical practice. 

3. Immune deficiency disorders treated by 

stem cell transplantation. 

a. Evaluation in the first 12 months. If you 

undergo stem cell transplantation for your 

immune deficiency disorder, we will 

consider you disabled until at least 12 

months from the date of the transplant. 

b. Evaluation after the 12-month period 

has elapsed. After the 12-month period has 

elapsed, we will consider any residuals of 

your immune deficiency disorder as well as 

any residual impairment(s) resulting from 

treatment, such as complications arising 

from: 

(i) Graft-versus-host (GVH) disease. 

(ii) Immunosuppressant therapy, such as 

frequent infections. 

(iii) Significant deterioration of other organ 

systems. 

4. Medication-induced immune 

suppression. Medication effects can result in 

varying degrees of immune suppression, but 

most resolve when the medication is ceased. 

However, if you are prescribed medication 

for long-term immune suppression, such as 

after an organ transplant, we will evaluate: 

a. The frequency and severity of infections. 

b. Residuals from the organ transplant 

itself, after the 12-month period has elapsed. 

c. Significant deterioration of other organ 

systems. 

F. How do we evaluate human 

immunodeficiency virus (HIV) infection? Any 

child with HIV infection, including one with 

a diagnosis of acquired immune deficiency 

syndrome (AIDS), may be found disabled 

under 114.08 if his or her impairment meets 

the criteria in that listing or is medically 

equivalent to the criteria in that listing. 

1. Documentation of HIV infection. The 

medical evidence must include 

documentation of HIV infection. 

Documentation may be by laboratory 

evidence or by other generally acceptable 

methods consistent with the prevailing state 

of medical knowledge and clinical practice. 

When you have had laboratory testing for 

HIV infection, we will make every reasonable 

effort to obtain reports of the results of that 

testing. 

a. Documentation of HIV infection by 

definitive diagnosis. A definitive diagnosis of 

HIV infection is documented by one or more 

of the following laboratory tests: 

(i) HIV antibody tests. HIV antibodies are 

usually first detected by an ELISA screening 

test performed on serum. Because the ELISA 

can yield false positive results, confirmation 

is required using a more definitive test, such 

as a Western blot or an immunofluorescence 

assay. Positive results on these tests are 

considered to be diagnostic of HIV infection 

in a child age 18 months or older. (See b. 

below, for information about HIV antibody 

testing in children younger than 18 months 

of age.) 

(ii) Positive ‘‘viral load’’ (VL) tests. These 

tests are normally used to quantitate the 

amount of the virus present but also 

document HIV infection. Such tests include 

the quantitative plasma HIV RNA, 

quantitative plasma HIV branched DNA, and 

reverse transcriptase-polymerase chain 

reaction (RT–PCR). 

(iii) HIV DNA detection by polymerase 

chain reaction (PCR). 

(iv) A specimen that contains HIV antigen 

(for example, serum specimen, lymphocyte 

culture, or cerebrospinal fluid), in a child age 

1 month or older. 

(v) A positive viral culture for HIV from 

peripheral blood mononuclear cells (PBMC). 

(vi) An immunoglobulin A (IgA) 

serological assay that is specific for HIV. 

(vii) Other tests that are highly specific for 

detection of HIV and that are consistent with 

the prevailing state of medical knowledge. 

b. Documentation of HIV infection in 

children from birth to the attainment of 18 

months. For children from birth to the 

attainment of 18 months of age, and who 

have tested positive for HIV antibodies, HIV 

infection is documented by: 

(i) One or more of the tests listed in 

F1a(ii)–F1a(vii). 

(ii) For newborn and younger infants (birth 

to attainment of age 1), a CD4 (T4) count of 

1500/mm3 or less, or a CD4 count less than 

or equal to 20 percent of total lymphocytes. 

(iii) For older infants and toddlers from 12 

to 18 months of age, a CD4 (T4) count of 750/ 

mm3 or less, or a CD4 count less than or 

equal to 20 percent of total lymphocytes. 

(iv) An abnormal CD4/CD8 ratio. 

(v) A severely diminished immunoglobulin 

G (IgG) level (<4 g/l or 400 mg/dl), or 

significantly greater than normal range for 

age. 

c. Other acceptable documentation of HIV 

infection. We may also document HIV 

infection without the definitive laboratory 

evidence described in 114.00F1a, provided 

that such documentation is consistent with 

the prevailing state of medical knowledge 

and clinical practice, and is consistent with 

the other evidence in your case record. If no 

definitive laboratory evidence is available, 

we may document HIV infection by the 

medical history, clinical and laboratory 

findings, and diagnosis(es) indicated in the 

medical evidence. For example, we will 

accept a diagnosis of HIV infection without 

definitive laboratory evidence if you have an 

opportunistic disease that is predictive of a 

defect in cell-mediated immunity (for 

example, Pneumocystis carinii pneumonia 

(PCP)), and there is no other known cause of 

diminished resistance to that disease (for 

example, long-term steroid treatment, 

lymphoma). In such cases, we will make 

every reasonable effort to obtain full details 

of the history, medical findings, and results 

of testing. 

2. CD4 tests. Children who have HIV 

infection or other disorders of the immune 

system may have tests showing a reduction 

of either the absolute count or the percentage 


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of their T-helper lymphocytes (CD4 cells). 

The extent of immune suppression correlates 

with the level or rate of decline of the CD4 

count. At age 6, children begin to have CD4 

counts comparable to the levels found in 

adults. Generally, in these children when the 

CD4 count is 200/mm3 or less (14 percent or 

less) the susceptibility to opportunistic 

infection is greatly increased. Although a 

reduced CD4 count alone does not establish 

a definitive diagnosis of HIV infection, a CD4 

count below 200 does offer supportive 

evidence when there are clinical findings, 

but not a definitive diagnosis of an 

opportunistic infection(s). However, a 

reduced CD4 count alone does not document 

the severity or functional consequences of 

HIV infection. 

3. Documentation of the manifestations of 

HIV infection. The medical evidence must 

also include documentation of the 

manifestations of HIV infection. 

Documentation may be by laboratory 

evidence or by other generally acceptable 

methods consistent with the prevailing state 

of medical knowledge and clinical practice. 

When you have had laboratory testing for a 

manifestation of HIV infection, we will make 

every reasonable effort to obtain reports of 

the results of that testing. 

a. Documentation of the manifestations of 

HIV infection by definitive diagnosis. The 

definitive method of diagnosing 

opportunistic diseases or conditions that are 

manifestations of HIV infection is by culture, 

serologic test, or microscopic examination of 

biopsied tissue or other material (for 

example, bronchial washings). We will make 

every reasonable effort to obtain specific 

laboratory evidence of an opportunistic 

disease or other condition whenever this 

information is available. If a histologic or 

other test has been performed, the evidence 

should include a copy of the appropriate 

report. If we cannot obtain the report, the 

summary of hospitalization or a report from 

the treating source should include details of 

the findings and results of the diagnostic 

studies (including appropriate medically 

acceptable imaging studies) or microscopic 

examination of the appropriate tissues or 

body fluids. 

b. Other acceptable documentation of the 

manifestations of HIV infection. We may also 

document manifestations of HIV infection 

without the definitive laboratory evidence 

described in 114.00F3a, provided that such 

documentation is consistent with the 

prevailing state of medical knowledge and 

clinical practice, and is consistent with the 

other evidence in your case record. If no 

definitive evidence is available, we may 

document the manifestations of HIV infection 

with other appropriate evidence. For 

example, many conditions are now 

commonly diagnosed based on some or all of 

the following: Medical history, clinical 

manifestations, laboratory findings 

(including appropriate medically acceptable 

imaging), and treatment responses. In such 

cases, we will make every reasonable effort 

to obtain full details of the history, medical 

findings, and results of testing. 

(i) Although a definitive diagnosis of PCP 

requires identifying the organism in 

bronchial washings, induced sputum, or lung 

biopsy, these tests are frequently bypassed if 

PCP can be diagnosed presumptively. (Note: 

Pneumocystis carinii is now known as 

Pneumocystis jiroveci; however, ‘‘PCP’’

remains in common usage for the pneumonia 

caused by this organism.) Supportive 

evidence includes: Fever, dyspnea, hypoxia, 

and CD4 count below 200 in children 6 years 

of age or older. Also supportive are bilateral 

lung interstitial infiltrates on x-ray, or a 

typical pattern on CT scan, or a gallium scan 

positive for pulmonary uptake. Response to 

anti-PCP therapy usually requires 5–7 days. 

(ii) Documentation of cytomegalovirus 

(CMV) disease (114.08D) may present special 

problems because definitive diagnosis 

(except for chorioretinitis, which may be 

diagnosed by an ophthalmologist on 

funduscopic exam) requires identification of 

viral inclusion bodies or a positive culture 

from the affected organ and the absence of 

any other infectious agent likely to be 

causing the disease. A positive serology test 

identifies a history of infection with CMV, 

but it does not confirm an active disease 

process. Therefore, a presumptive diagnosis 

of CMV disease requires corroborating 

evidence that CMV is causing the disease. 

Supportive evidence includes: Fever, 

positive CMV serology test, urinary culture 

positive for CMV, and CD4 count below 200 

in children 6 years of age or older. A clear 

response to anti-CMV therapy also supports 

a diagnosis. 

(iii) A definitive diagnosis of 

toxoplasmosis of the brain is made by brain 

biopsy, but this procedure carries significant 

risk and is not commonly performed. This 

condition is usually diagnosed 

presumptively based on symptoms or signs of 

fever, headache, focal neurologic deficits, 

seizures, typical lesions on brain imaging, 

and a positive serology test. 

4. HIV infection manifestations specific to 

children. 

a. General. The clinical manifestation and 

course of disease in children who become 

infected with HIV perinatally or in the first 

6 years of life may differ from that in 

adolescents (age 12 to attainment of age 18) 

and adults. Newborn and younger infants 

(birth to attainment of age 1) and older 

infants and toddlers (age 1 to attainment of 

age 3) may present with failure to thrive or 

PCP; preschool children (age 3 to attainment 

of age 6) and primary school children (age 6 

to attainment of age 12) may present with 

recurrent infections, neurological problems, 

or developmental abnormalities. Adolescents 

may also exhibit neurological abnormalities 

such as HIV encephalopathy, or have growth 

problems. 

b. Neurologic abnormalities. The methods 

of identifying and evaluating neurologic 

abnormalities may vary depending on a 

child’s age. For example, in an infant 

impaired brain growth can be documented by 

a decrease in the growth rate of the head. In 

an older child, impaired brain growth may be 

documented by brain atrophy on a CT scan 

or MRI. Neurologic abnormalities in infants 

and young children may present as serious 

developmental delays or in the loss of 

previously acquired developmental 

milestones. In school-age children and 

adolescents, this type of neurologic 

abnormality would generally present as the 

loss of previously acquired intellectual 

abilities. This may be evidenced by a 

decrease in intelligence quotient (IQ) scores, 

by a child forgetting information he or she 

previously learned, by being unable to learn 

new information, or by a sudden onset of a 

new learning disability. 

c. Bacterial infections. Children with HIV 

infection may contract any of a broad range 

of bacterial infections. Certain major 

infections caused by pyogenic bacteria (for 

example, some pneumonias) can be severely 

limiting, especially in pre-adolescent 

children. We evaluate these major bacterial 

infections under 114.08A4. Although 

114.08A4 applies only to children under 13 

years of age, children age 13 and older may 

have an impairment that medically equals 

this listing if the circumstances of the case 

warrant; for example, if there is delayed 

puberty. We will evaluate pelvic 

inflammatory disease in older girls under 

114.08A5. 

G. How will we consider the effect of 

treatment in evaluating your autoimmune 

disorder, immune deficiency disorder, or HIV 

infection? 

1. General. If your impairment does not 

otherwise meet the requirements of a listing 

we will consider your medical treatment both 

in terms of its effectiveness in improving the 

signs, symptoms, and laboratory 

abnormalities of your specific immune 

system disorder or its manifestations, and in 

terms of any side effects that limit your 

functioning. We will make every reasonable 

effort to obtain a specific description of the 

treatment you receive (including surgery) for 

your immune system disorder. We consider: 

a. The effects of medications you take. 

b. Adverse side effects (acute and chronic). 

c. The intrusiveness and complexity of 

your treatment (for example, the dosing 

schedule, need for injections). 

d. The effect of treatment on your mental 

functioning (for example, cognitive changes, 

mood disturbance). 

e. Variability of your response to treatment 

(see 114.00G2). 

f. The interactive and cumulative effects of 

your treatments. For example, many 

individuals with immune system disorders 

receive treatment both for their immune 

system disorders and for the manifestations 

of the disorders or co-occuring impairments, 

such as treatment for HIV infection and 

hepatitis C. The interactive and cumulative 

effects of these treatments may be greater 

than the effects of each treatment considered 

separately. 

g. The duration of your treatment. 

h. Any other aspects of treatment that may 

interfere with your ability to function. 

2. Variability of your response to treatment. 

Your response to treatment and the adverse 

or beneficial consequences of your treatment 

may vary widely. The effects of your 

treatment may be temporary or long term. For 

example, some individuals may show an 

initial positive response to a drug or 

combination of drugs followed by a decrease 

in effectiveness. When we evaluate your 

response to treatment and how your 

treatment may affect you, we consider such 

factors as disease activity before treatment, 


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requirements for changes in therapeutic 

regimes, the time required for therapeutic 

effectiveness of a particular drug or drugs, 

the limited number of drug combinations that 

may be available for your impairment(s), and 

the time-limited efficacy of some drugs. For 

example, a child with HIV infection or 

another immune deficiency disorder who 

develops otitis media may not respond to the 

same antibiotic regimen used in treating 

children without these disorders or may not 

respond to an antibiotic that he or she 

responded to before. Therefore, we must 

consider the effects of your treatment on an 

individual basis, including the effects of your 

treatment on your ability to function. 

3. How we evaluate the effects of treatment 

for autoimmune disorders on your ability to 

function. Some medications may have acute 

or long-term side effects. When we consider 

the effects of corticosteroids or other 

treatments for autoimmune disorders on your 

ability to function, we consider the factors in 

114.00G1 and 114.00G2. Long-term 

corticosteroid treatment can cause ischemic 

necrosis of bone, posterior subcapsular 

cataract, impaired growth, weight gain, 

glucose intolerance, increased susceptibility 

to infection, and osteopenia that may result 

in a loss of function. In addition, medications 

used in the treatment of autoimmune 

disorders may also have effects on mental 

function including cognition (for example, 

memory), concentration, and mood. 

4. How we evaluate the effects of treatment 

for immune deficiency disorders, excluding 

HIV infection, on your ability to function. 

When we consider the effects of your 

treatment for your immune deficiency 

disorder on your ability to function, we 

consider the factors in 114.00G1 and 

114.00G2. A frequent need for treatment such 

as intravenous immunoglobulin and gamma 

interferon therapy can be intrusive and 

interfere with your ability to function. We 

will also consider whether you have chronic 

side effects from these or other medications, 

including fatigue, fever, headaches, high 

blood pressure, joint swelling, muscle aches, 

nausea, shortness of breath, or limitations in 

mental function including cognition (for 

example, memory) concentration, and mood. 

5. How we evaluate the effects of treatment 

for HIV infection on your ability to function. 

a. General. When we consider the effects of 

antiretroviral drugs (including the effects of 

highly active antiretroviral therapy (HAART)) 

and the effects of treatments for the 

manifestations of HIV infection on your 

ability to function, we consider the factors in 

114.00G1 and 114.00G2. Side effects of 

antiretroviral drugs include, but are not 

limited to: Bone marrow suppression, 

pancreatitis, gastrointestinal intolerance 

(nausea, vomiting, diarrhea), neuropathy, 

rash, hepatotoxicity, lipodystrophy, glucose 

intolerance, and lactic acidosis. In addition, 

medications used in the treatment of HIV 

infection may also have effects on mental 

function, including cognition (for example, 

memory), concentration, and mood, and may 

result in malaise, fatigue, joint and muscle 

pain, and insomnia. The symptoms of HIV 

infection and the side effects of medication 

may be indistinguishable from each other. 

We will consider all of your functional 

limitations, whether they result from your 

symptoms of HIV infection or the side effects 

of your treatment. 

b. Structured treatment interruptions. A 

structured treatment interruption (STI, also 

called a ‘‘drug holiday’’) is a treatment 

practice during which your treating source 

advises you to stop taking your medications 

temporarily. An STI in itself does not imply 

that your medical condition has improved or 

that you are noncompliant with your 

treatment because you are following your 

treating source’s advice. Therefore, if you 

have stopped taking medication because your 

treating source prescribed or recommended 

an STI, we will not find that you are failing 

to follow treatment or draw inferences about 

the severity of your impairment on this fact 

alone. We will consider why your treating 

source has prescribed or recommended an 

STI and all the other information in your case 

record when we determine the severity of 

your impairment. 

6. When there is no record of ongoing 

treatment. If you have not received ongoing 

treatment or have not had an ongoing 

relationship with the medical community 

despite the existence of a severe 

impairment(s), we will evaluate the medical 

severity and duration of your immune system 

impairment on the basis of the current 

objective medical evidence and other 

evidence in your case record, taking into 

consideration your medical history, 

symptoms, clinical and laboratory findings, 

and medical source opinions. If you have just 

begun treatment and we cannot determine 

whether you are disabled based on the 

evidence we have, we may need to wait to 

determine the effect of the treatment on your 

ability to function. The amount of time we 

need to wait will depend on the facts of your 

case. If you have not received treatment, you 

may not be able to show an impairment that 

meets the criteria of one of the immune 

system listings, but your immune system 

impairment may medically equal a listing or 

functionally equal the listings. 

H. How do we consider your symptoms, 

including your constitutional symptoms or 

pain? 

Your symptoms, including pain, fatigue, 

and malaise, may be important factors in our 

determination whether your immune system 

disorder(s) meets or medically equals a 

listing or in our determination whether you 

otherwise have marked and severe functional 

limitations. In order for us to consider your 

symptoms, you must have medical signs or 

laboratory findings showing the existence of 

a medically determinable impairment(s) that 

could reasonably be expected to produce the 

symptoms. If you have such an 

impairment(s), we will evaluate the intensity, 

persistence, and functional effects of your 

symptoms using the rules throughout 114.00 

and in our other regulations. See §§ 416.928, 

and 416.929. 

I. How do we use the functional criteria in 

these listings? 

1. The following listings in this body 

system include standards for evaluating the 

limitations resulting from manifestations of 

immune system disorders that do not meet 

the criteria of the other sections of their 

respective listings: 114.02B, for systemic 

lupus erythematosus; 114.03B, for systemic 

vasculitis; 114.04D, for systemic sclerosis 

(scleroderma); 114.05E, for polymyositis and 

dermatomyositis; 114.06B, for 

undifferentiated and mixed connective tissue 

disease; 114.07C, for immune deficiency 

disorders, excluding HIV infection; 114.08L, 

for HIV infection; 114.09D, for inflammatory 

arthritides; and 114.10B, for Sjo ̈gren’s 

syndrome. 

2. When we use one of the listings cited 

in 114.00I1, we will consider all relevant 

information in your case record to determine 

the full impact of your immune system 

disorder(s) on your ability to function on a 

sustained basis. Important factors we will 

consider when we evaluate your functioning 

under these listings include, but are not 

limited to: Your symptoms, the frequency 

and duration of manifestations of your 

immune system disorder, periods of 

exacerbation and remission, and the 

functional impact of your treatment, 

including the side effects of your medication. 

3. To satisfy the functional criterion in a 

listing, your immune system disorder must 

result in an ‘‘extreme’’ limitation in one 

domain of functioning or ‘‘marked’’

limitations in two domains of functioning 

depending on your age. (See 112.00C for 

additional discussion of these areas of 

functioning and §§ 416.924a and 416.926a for 

additional guidance on the evaluation of 

functioning in children.) Functional 

limitation may result from the impact of the 

disease process itself on your mental 

functioning, physical functioning, or both 

your mental and physical functioning. This 

could result from persistent or intermittent 

symptoms, such as depression, fatigue, or 

pain, resulting in a limitation of your ability 

to do a task, to concentrate, to persevere at 

a task, or to perform the task at an acceptable 

rate of speed. You may also have limitations 

because of your treatment and its side effects 

(see 114.00G). 

J. How do we evaluate your immune system 

disorder when it does not meet one of these 

listings? 

1. These listings are only examples of 

immune system disorders that we consider 

severe enough to result in marked and severe 

functional imitations. If your impairment(s) 

does not meet the criteria of any of these 

listings, we must also consider whether you 

have an impairment(s) that satisfies the 

criteria of a listing in another body system. 

2. Individuals with immune system 

disorders, including HIV infection, may 

manifest signs or symptoms of a mental 

impairment or of another physical 

impairment. We may evaluate these 

impairments under any affected body system. 

For example, we will evaluate: 

a. Growth impairment under 100.00ff. 

b. Musculoskeletal involvement, such as 

surgical reconstruction of a joint, under 

101.00ff. 

c. Ocular involvement, such as dry eye, 

under 102.00ff 

d. Respiratory impairments, such as 

pleuritis, under 103.00ff. 

e. Cardiovascular impairments, such as 

cardiomyopathy, under 104.00ff. 

f. Digestive impairments, such as hepatitis 

(including hepatitis C), under 105.00ff. 


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g. Genitourinary impairments, such as 

nephropathy, under 106.00ff. 

h. Hematologic abnormalities, such as 

anemia, granulocytopenia, and 

thrombocytopenia, under 107.00ff. 

i. Skin impairments, such as persistent 

fungal and other infectious skin eruptions, 

and photosensitivity, under 108.00ff. 

j. Neurologic impairments, such as 

neuropathy or seizures, under 111.00ff. 

k. Mental disorders, such as depression, 

anxiety, or cognitive deficits, under 112.00ff. 

l. Allergic disorders, such as asthma or 

atopic dermatitis, under 103.00ff or 108.00ff 

or under the criteria in another affected body 

system. 

m. Syphilis or neurosyphilis under the 

criteria for the affected body system; for 

example, 102.00 Special senses and speech, 

104.00 Cardiovascular system, or 111.00 

Neurological. 

3. If you have a severe medically 

determinable impairment(s) that does not 

meet a listing, we will determine whether 

your impairment(s) medically equals a 

listing. (See § 416.926.) If it does not, we will 

also consider whether you have an 

impairment(s) that functionally equals the 

listings. (See § 416.926a.) We use the rules in 

§ 416.994a when we decide whether you 

continue to be disabled. 

114.01 Category of Impairments, Immune 

System Disorders 

114.02 Systemic lupus erythematosus. As 

described in 114.00D1. With: 

A. Involvement of two or more organs/ 

body systems, with: 

1. One of the organs/body systems 

involved to at least a moderate level of 

severity, and 

2. At least two of the following 

constitutional symptoms or signs: Severe 

fatigue, fever, malaise, or involuntary weight 

loss. 

OR 

B. Any other manifestation(s) of SLE 

resulting in one of the following: 

1. For children from birth to attainment of 

age 1, at least one of the criteria in 

paragraphs A–E of 112.12; or 

2. For children age 1 to attainment of age 

3, at least one of the appropriate age-group 

criteria in paragraph B1 of 112.02; or 

3. For children age 3 to attainment of age 

18, at least two of the appropriate age-group 

criteria in paragraph B2 of 112.02. 

114.03 Systemic vasculitis. As described 

in 114.00D2. With: 

A. Involvement of two or more organs/ 

body systems, with: 

1. One of the organs/body systems 

involved to at least a moderate level of 

severity, and 

2. At least two of the following 

constitutional symptoms or signs: Severe 

fatigue, fever, malaise, or involuntary weight 

loss. 

OR 

B. Any other manifestation(s) of systemic 

vasculitis resulting in one of the following: 

1. For children from birth to attainment of 

age 1, at least one of the criteria in 

paragraphs A–E of 112.12; or 

2. For children age 1 to attainment of age 

3, at least one of the appropriate age-group 

criteria in paragraph B1 of 112.02; or 

3. For children age 3 to attainment of age 

18, at least two of the appropriate age-group 

criteria in paragraph B2 of 112.02. 

114.04 Systemic sclerosis (scleroderma). 

As described in 114.00D3. With: 

A. Involvement of two or more organs/ 

body systems, with: 

1. One of the organs/body systems 

involved to at least a moderate level of 

severity, and 

2. At least two of the following 

constitutional symptoms or signs: Severe 

fatigue, fever, malaise, or involuntary weight 

loss. 

OR 

B. With one of the following: 

1. Toe contractures or fixed deformity of 

one or both feet, resulting in the inability to 

ambulate effectively as defined in 114.00C6; 

or 

2. Finger contractures or fixed deformity in 

both hands, resulting in the inability to 

perform fine and gross movements effectively 

as defined in 114.00C7; or 

3. Atrophy with irreversible damage in one 

or both lower extremities, resulting in the 

inability to ambulate effectively as defined in 

114.00C6; or 

4. Atrophy with irreversible damage in 

both upper extremities, resulting in the 

inability to perform fine and gross 

movements effectively as defined in 

114.00C7. 

OR 

C. Raynaud’s phenomenon, characterized 

by: 

1. Gangrene of a toe or finger in at least two 

extremities, or of a toe and finger; or 

2. Ischemia with ulcerations of toes or 

fingers, resulting in the inability to ambulate 

effectively or to perform fine and gross 

movements effectively as defined in 

114.00C6 and 114.00C7; or 

D. Any other manifestation(s) of systemic 

sclerosis (scleroderma) resulting in one of the 

following: 

1. For children from birth to attainment of 

age 1, at least one of the criteria in 

paragraphs A–E of 112.12; or 

2. For children age 1 to attainment of age 

3, at least one of the appropriate age-group 

criteria in paragraph B1 of 112.02; or 

3. For children age 3 to attainment of age 

18, at least two of the appropriate age-group 

criteria in paragraph B2 of 112.02. 

114.05 Polymyositis and 

dermatomyositis. As described in 114.00D4. 

With: 

A. Proximal limb-girdle (pelvic or 

shoulder) muscle weakness, resulting in 

inability to ambulate effectively or inability 

to perform fine and gross movements 

effectively as defined in 114.00C6 and 

114.00C7. 

OR 

B. Impaired swallowing (dysphagia) and 

aspiration due to muscle weakness. 

OR 

C. Impaired respiration due to intercostal 

and diaphragmatic muscle weakness. 

OR 

D. Diffuse calcinosis with limitation of 

joint mobility or intestinal motility. 

OR 

E. Any other manifestation(s) of 

polymyositis or dermatomyositis resulting in 

one of the following: 

1. For children from birth to attainment of 

age 1, at least one of the criteria in 

paragraphs A–E of 112.12; or 

2. For children age 1 to attainment of age 

3, at least one of the appropriate age-group 

criteria in paragraph B1 of 112.02; or 

3. For children age 3 to attainment of age 

18, at least two of the appropriate age-group 

criteria in paragraph B2 of 112.02. 

114.06 Undifferentiated and mixed 

connective tissue disease. As described in 

114.00D5. With: 

A. Involvement of two or more organs/ 

body systems, with: 

1. One of the organs/body systems 

involved to at least a moderate level of 

severity, and 

2. At least two of the following 

constitutional symptoms or signs: Severe 

fatigue, fever, malaise, or involuntary weight 

loss. 

OR 

B. Any other manifestation(s) of 

undifferentiated or mixed connective tissue 

disease resulting in one of the following: 

1. For children from birth to attainment of 

age 1, at least one of the criteria in 

paragraphs A–E of 112.12; or 

2. For children age 1 to attainment of age 

3, at least one of the appropriate age-group 

criteria in paragraph B1 of 112.02; or 

3. For children age 3 to attainment of age 

18, at least two of the appropriate age-group 

criteria in paragraph B2 of 112.02. 

114.07 Immune deficiency disorders, 

excluding HIV infection. As described in 

114.00E. With: 

A. One or more of the following infections. 

The infection(s) must either be resistant to 

treatment, or require hospitalization or 

intravenous treatment three or more times in 

a 12-month period. 

1. Sepsis; or 

2. Meningitis; or 

3. Pneumonia; or 

4. Septic arthritis; or 

5. Endocarditis; or 

6. Sinusitis documented by appropriate 

medically acceptable imaging. 

OR 

B. Stem cell transplantation as described 

under 114.00E3. Consider under a disability 

until at least 12 months from the date of 

transplantation. Thereafter, evaluate any 

residual impairment(s) under the criteria for 

the affected body system. 

OR 

C. Any other manifestations(s) of an 

immune deficiency disorder resulting in one 

of the following: 

1. For children from birth to attainment of 

age 1, at least one of the criteria in 

paragraphs A–E of 112.12; or 

2. For children age 1 to attainment of age 

3, at least one of the appropriate age-group 

criteria in paragraph B1 of 112.02; or 

3. For children age 3 to attainment of age 

18, at least two of the appropriate age-group 

criteria in paragraph B2 of 112.02. 

114.08 Human immunodeficiency virus 

(HIV) infection. With documentation as 


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described in 114.00F and one of the 

following: 

A. Bacterial infections: 

1. Mycobacterial infection (for example, 

caused by M. avium-intracellulare, M. 

kansasii, or M. tuberculosis) at a site other 

than the lungs, skin, or cervical or hilar 

lymph nodes, or pulmonary tuberculosis 

resistant to treatment; or 

2. Nocardiosis; or 

3. Salmonella bacteremia, recurrent nontyphoid; or 

4. In a child less than 13 years of age, 

multiple or recurrent pyogenic bacterial 

infection(s) (sepsis, pneumonia, meningitis, 

bone or joint infection, or abscess of an 

internal organ or body cavity, but not otitis 

media or superficial skin or mucosal 

abscesses) occurring two or more times in 2 

years; or 

5. Multiple or recurrent bacterial 

infection(s), including pelvic inflammatory 

disease, requiring hospitalization or 

intravenous antibiotic treatment three or 

more times in a 12-month period. 

OR 

B. Fungal infections: 

1. Aspergillosis; or 

2. Candidiasis involving the esophagus, 

trachea, bronchi, or lungs, or at another site 

other than the skin, urinary tract, intestinal 

tract, or oral or vulvovaginal mucous 

membranes; or 

3. Coccidioidomycosis, at a site other than 

the lungs or lymph nodes; or 

4. Cryptococcosis, at a site other than the 

lungs (for example, cryptococcal meningitis); 

or 

5. Histoplasmosis, at a site other than the 

lungs or lymph nodes; or 

6. Mucormycosis; or 

7. Pneumocystis carinii (jiroveci) 

pneumonia or extrapulmonary pneumocystis 

carinii (jiroveci) infection. 

OR 

C. Protozoan or helminthic infections: 

1. Cryptosporidiosis, isosporiasis, or 

microsporidiosis, with diarrhea lasting for 1 

month or longer; or 

2. Strongyloidiasis, extra-intestinal; or 

3. Toxoplasmosis of an organ other than 

the liver, spleen, or lymph nodes. 

OR 

D. Viral infections: 

1. Cytomegalovirus disease (documented as 

described in 114.00F3b(ii)) at a site other 

than the liver, spleen, or lymph nodes; or 

2. Herpes simplex virus causing: 

a. Mucocutaneous infection (for example, 

oral, genital, perianal) lasting for 1 month or 

longer; or 

b. Infection at a site other than the skin or 

mucous membranes (for example, bronchitis, 

pneumonitis, esophagitis, or encephalitis); or 

c. Disseminated infection; or 

3. Herpes zoster: 

a. Disseminated; or 

b. With multidermatomal eruptions that 

are resistant to treatment; or 

4. Progressive multifocal 

leukoencephalopathy. 

OR 

E. Malignant neoplasms: 

1. Carcinoma of the cervix, invasive, FIGO 

stage II and beyond; or 

2. Kaposi’s sarcoma with: 

a. Extensive oral lesions; or 

b. Involvement of the gastrointestinal tract, 

lungs, or other visceral organs; or 

3. Lymphoma (for example, primary 

lymphoma of the brain, Burkitt’s lymphoma, 

immunoblastic sarcoma, other non-Hodgkin’s 

lymphoma, Hodgkin’s disease); or 

4. Squamous cell carcinoma of the anus. 

OR 

F. Conditions of the skin or mucous 

membranes (other than described in B2, D2, 

or D3, above), with extensive fungating or 

ulcerating lesions not responding to 

treatment (for example, dermatological 

conditions such as eczema or psoriasis, 

vulvovaginal or other mucosal candida, 

condyloma caused by human papillomavirus, 

genital ulcerative disease). 

OR 

G. Neurological manifestations of HIV 

infection (for example, HIV encephalopathy, 

peripheral neuropathy) resulting in one of 

the following: 

1. Loss of previously acquired, or marked 

delay in achieving, developmental 

milestones or intellectual ability (including 

the sudden onset of a new learning 

disability); or 

2. Impaired brain growth (acquired 

microcephaly or brain atrophy—see 

114.00F4b); or 

3. Progressive motor dysfunction affecting 

gait and station or fine and gross motor skills. 

OR 

H. Growth disturbance, with: 

1. An involuntary weight loss (or failure to 

gain weight at an appropriate rate for age) 

resulting in a fall of 15 percentiles from an 

established growth curve (on standard 

growth charts) that persists for 2 months or 

longer, or 

2. An involuntary weight loss (or failure to 

gain weight at an appropriate rate for age) 

resulting in a fall to below the third 

percentile from an established growth curve 

(on standard growth charts) that persists for 

2 months or longer; or 

3. Involuntary weight loss of 10 percent or 

more of baseline that persists for 2 months 

or longer. 

OR 

I. Diarrhea, lasting for 1 month or longer, 

resistant to treatment, and requiring 

intravenous hydration, intravenous 

alimentation, or tube feeding. 

OR 

J. Lymphoid interstitial pneumonia/ 

pulmonary lymphoid hyperplasia (LIP/PLH 

complex), with respiratory symptoms that 

significantly interfere with age-appropriate 

activities, and that cannot be controlled by 

prescribed treatment. 

OR 

K. One or more of the following infections 

(other than described in A-J, above). The 

infection(s) must either be resistant to 

treatment, or require hospitalization or 

intravenous treatment three or more times in 

a 12-month period. 

1. Sepsis; or 

2. Meningitis; or 

3. Pneumonia; or 

4. Septic arthritis; or 

5. Endocarditis; or 

6. Sinusitis documented by appropriate 

medically acceptable imaging. 

OR 

L. Any other manifestation(s) of HIV 

infection, including those listed in 114.08A–

K, but without the requisite findings for those 

listings (for example, oral candidiasis not 

meeting the criteria in 114.08F, diarrhea not 

meeting the criteria in 114.08I), or other 

manifestation(s) (for example, oral hairy 

leukoplakia, hepatomegaly), resulting in one 

of the following: 

1. For children from birth to attainment of 

age 1, at least one of the criteria in 

paragraphs A-E of 112.12; or 

2. For children age 1 to attainment of age 

3, at least one of the appropriate age-group 

criteria in paragraph B1 of 112.02; or 

3. For children age 3 to attainment of age 

18, at least two of the appropriate age-group 

criteria in paragraph B2 of 112.02. 

114.09 Inflammatory arthritis. As 

described in 114.00D6. With: 

A. Persistent inflammation or deformity in 

two or more major peripheral joints resulting 

in the inability to ambulate effectively or the 

inability to perform fine and gross 

movements effectively as defined in 

114.00C6 and 114.00C7. 

OR 

B. Inflammation or deformity in one or 

more major peripheral joints, but with less 

joint involvement than in A and extraarticular features that do not satisfy the 

criteria of a listing, with: 

1. Involvement of two or more organs/body 

systems with one of the organs/body systems 

involved to at least a moderate level of 

severity, and 

2. At least two of the following 

constitutional symptoms or signs: Severe 

fatigue, fever, malaise, or involuntary weight 

loss. 

OR 

C. Ankylosing spondylitis or other 

spondyloarthropathies, with: 

1. Ankylosis (fixation) of the dorsolumbar 

or cervical spines as shown by appropriate 

medically acceptable imaging and measured 

on physical examination at 45° or more of 

flexion from the vertical position (zero 

degrees); or 

2. Ankylosis (fixation) of the dorsolumbar 

or cervical spine as shown by appropriate 

medically acceptable imaging and measured 

on physical examination at 30° or more of 

flexion (but less than 45°) measured from the 

vertical position (zero degrees), and 

involvement of two or more organs/body 

systems with one of the organs/body systems 

involved to at least a moderate level of 

severity. 

OR 

D. Any other manifestation(s) of 

inflammatory arthritis resulting in one of the 

following: 

1. For children from birth to attainment of 

age 1, at least one of the criteria in 

paragraphs A-E of 112.12; or 


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2. For children age 1 to attainment of age 

3, at least one of the appropriate age-group 

criteria in paragraph B1 of 112.02; or 

3. For children age 3 to attainment of age 

18, at least two of the appropriate age-group 

criteria in paragraph B2 of 112.02. 

114.10 Sjo ̈ gren’s syndrome. As described n 

114.00D7. With: 

A. Involvement of two or more organs/ 

body systems, with: 

1. One of the organs/body systems 

involved to at least a moderate level of 

severity, and 

2. At least two of the following 

constitutional symptoms or signs: Severe 

fatigue, fever, malaise, or involuntary weight 

loss. 

OR 

B. Any other manifestation(s) of Sjo ̈gren’s 

syndrome resulting in one of the following: 

1. For children from birth to attainment of 

age 1, at least one of the criteria in 

paragraphs A-E of 112.12; or 

2. For children age 1 to attainment of age 

3, at least one of the appropriate age-group 

criteria in paragraph B1 of 112.02; or 

3. For children age 3 to attainment of age 

18, at least two of the appropriate age-group 

criteria in paragraph B2 of 112.02. 

[FR Doc. 06–6655 Filed 8–3–06; 8:45 am] 

BILLING CODE 4191–02–P 


jlentini on PROD1PC65 with PROPOSALS3