**[IMACS FORM 07b: MYOSITIS DISEASE ACTIVITY ASSESSMENT TOOL, Version
2 - 2005]{.underline}**

**Subject's IMACS number:\_\_\_\_\_\_\_\_\_\_\_\_
ASSESSOR:\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ Date
Assessed:\_\_\_\_\_\_\_\_\_\_\_\_\_\_Assessment number:\_\_\_\_\_\_**

**The clinical features recorded are based upon the previous 4 weeks and
the judgment that the feature is DUE TO THE MYOSITIS DISEASE PROCESS and
represents disease activity, which is potentially reversible. Please
rate your overall assessment of the ongoing current disease activity for
each of the systems below by drawing a vertical mark on the 10-cm. line
for each system according to the following scale: left end of line = no
evidence of disease activity, midpoint of line = moderate disease
activity, and right end of line = extreme or maximum disease activity.
Under each organ system is a set of clinical and laboratory findings
that you should assess in order to determine the VAS disease activity
score for each organ system.**

**For each individual item, also rate: A = very active, requiring
disease modifying therapy; B =moderate disease activity / therapy; C =
mild and stable disease activity / therapy; D = resolved (no therapy
required); E = never present; NA= cannot be assessed.**

***[Please review all instructions and the GLOSSARY for definitions
prior to assessment (see pages 4 - 12).]{.underline}***

+---------+--------------------------------+---------------------------+
| **[C    | **(Absent) (Maximum)**         | ###### Ma                 |
| ONSTITU |                                | ximum Value Guidelines (E |
| TIONAL] |                                | xamples of maximal score) |
| {.under |                                |                           |
| line}** |                                | Severe fatigue or malaise |
|         |                                | resulting in being bed    |
| #       |                                | bound and an inability to |
| ####### |                                | perform self care         |
|  **DISE |                                |                           |
| ASE ACT |                                |                           |
| IVITY** |                                |                           |
+---------+--------------------------------+---------------------------+

1\. Pyrexia -- documented fever \> 38 deg. C. A B C D E NA

2\. Weight loss -- unintentional \> 5% A B C D E NA

3\. Fatigue/malaise/lethargy A B C D E NA

4\. Lymphadenopathy (\> 1 cm) A B C D E NA

+--------+---------------------------------+---------------------------+
| **[    | **(Absent) (Maximum)**          | ###### Ma                 |
| CUTANE |                                 | ximum Value Guidelines (E |
| OUS]{. |                                 | xamples of maximal score) |
| underl |                                 |                           |
| ine}** |                                 | \- Ulceration to muscle,  |
|        |                                 | tendon or bone;\          |
| #####  |                                 | - Extensive erythroderma  |
| ### ** |                                 |                           |
| DISEAS |                                 |                           |
| E ACTI |                                 |                           |
| VITY** |                                 |                           |
+--------+---------------------------------+---------------------------+

5\. Cutaneous ulceration A B C D E NA

6\. Erythroderma A B C D E NA

7.  Erythematous rashes accompanied by vesiculobullous change, erosions
    or necrosis A B C D E NA

8.  Panniculitis A B C D E NA

9.  Erythematous rashes without secondary changes B C D E NA

10\. Heliotrope rash B C D E NA

11\. Gottron's papules/sign B C D E NA

12\. Periungual capillary changes B C D E NA

13\. Alopecia: B C D E NA

-   Diffuse hair loss = category B (or category C if mild and stable)

-   Focal, patchy with erythema = category C

~~14. Mechanic's hands B C D E NA~~

+--------+----------------------------------+--------------------------+
| **     | **(Absent) (Maximum)**           | ###### Maxi              |
| [SKELE |                                  | mum Value Guidelines (Ex |
| TAL]{. |                                  | amples of maximal score) |
| underl |                                  |                          |
| ine}** |                                  | \- Severe arthritis with |
|        |                                  | extreme loss of function |
| **[D   |                                  | (bedridden, inability    |
| ISEASE |                                  | for self care)           |
| ACTIV  |                                  |                          |
| ITY]{. |                                  |                          |
| underl |                                  |                          |
| ine}** |                                  |                          |
+--------+----------------------------------+--------------------------+

15\. Arthritis: A B C D E NA

-   Severe active polyarthritis with major loss of function = category A

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-   Moderately active arthritis with moderate loss of function =
    category B

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```
-   Mild arthritis with no to mild loss of function = category C

16\. Polyarthralgias C D E NA

**Subject's IMACS number:\_\_\_\_\_\_\_\_\_\_\_\_
ASSESSOR:\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ Date
Assessed:\_\_\_\_\_\_\_\_\_\_\_\_\_Assessment number:\_\_\_\_\_\_**

+----------+--------------------------------+--------------------------+
| #######  | **(Absent) (Maximum)**         | ###### Maxi              |
|  [GASTRO |                                | mum Value Guidelines (Ex |
| INTESTIN |                                | amples of maximal score) |
| AL DISEA |                                |                          |
| SE ACTIV |                                | Major abdominal crisis   |
| ITY]{.un |                                | requiring surgery or     |
| derline} |                                | intensive care           |
+----------+--------------------------------+--------------------------+

17\. Gastrointestinal ulceration A B C D E NA

18\. Dysphagia: A B C D E NA

-   Moderate/severe dysphagia = category A

-   Mild dysphagia = category B (or category C if mild and stable)

19\. Abdominal pain: A B C D E NA

-   Severe = category A

-   Moderate = category B

-   Mild = category C

+-------+-----------------------------------+--------------------------+
| **P   | **(Absent) (Maximum)**            | ###### Maxi              |
| ULMON |                                   | mum Value Guidelines (Ex |
| ARY** |                                   | amples of maximal score) |
|       |                                   |                          |
| ###   |                                   | Active interstitial lung |
| ####  |                                   | disease or respiratory   |
| [DISE |                                   | muscle weakness          |
| ASE A |                                   | requiring ventilatory    |
| CTIVI |                                   | support                  |
| TY]{. |                                   |                          |
| under |                                   |                          |
| line} |                                   |                          |
+-------+-----------------------------------+--------------------------+

20\. Respiratory muscle weakness without intrinsic lung disease: A B C D
E NA

-   Dyspnea at rest = category A (or category B if moderate)

-   Dyspnea on exertion = category B (or category C if mild and stable)

21\. Active interstitial lung disease (ILD), WITH previously documented
ILD by radiography or pathology: A B C D E NA

*Mark only one of category A - C, using pulmonary function tests for the
primary assessment when available*:

-   Shortness of breath at rest [OR]{.underline} FVC (Forced Vital
    Capacity) or DLCO (Carbon monoxide diffusion capacity) 50% predicted
    [OR]{.underline} requirement for oxygen use or assisted ventilation
    = category A

-   Shortness of breath with normal activity [OR]{.underline} FVC or
    DLCO 50 -- 70% predicted = category B

-   Shortness of breath with exertional activity [OR]{.underline} FVC or
    DLCO \> 70% predicted = category C

22\. Dysphonia: B C D E NA

-   Moderate to severe = category B

-   Mild = category C

+-----+-----------------------------------+---------------------------+
| #   | **(Absent) (Maximum)**            | ###### Ma                 |
| ### |                                   | ximum Value Guidelines (E |
| ### |                                   | xamples of maximal score) |
|  [C |                                   |                           |
| ARD |                                   | Myocarditis or            |
| IAC |                                   | pericarditis requiring    |
|  DI |                                   | intensive unit care       |
| SEA |                                   |                           |
| SE  |                                   |                           |
| ACT |                                   |                           |
| IVI |                                   |                           |
| TY] |                                   |                           |
| {.u |                                   |                           |
| nde |                                   |                           |
| rli |                                   |                           |
| ne} |                                   |                           |
+-----+-----------------------------------+---------------------------+

23\. Myocarditis or Pericarditis: A B C D E NA

-   Symptomatic or with physical exam changes = category A

-   In the absence of clinical features = category B (or category C if
    mild and stable)

24\. Arrhythmia: A B C D E NA

-   Severe arrhythmia = category A

-   Other arrhythmia, except sinus tachycardia = category B (or category
    C if mild and stable)

25\. Sinus tachycardia C D E NA

**Subject's IMACS number:\_\_\_\_\_\_\_\_\_\_\_\_
ASSESSOR:\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ Date
Assessed:\_\_\_\_\_\_\_\_\_\_\_\_\_Assessment number:\_\_\_\_\_\_**

+-----+-----------------------------------+---------------------------+
| ##  | **(Absent) (Maximum)**            | ###### Ma                 |
| ### |                                   | ximum Value Guidelines (E |
| ##  |                                   | xamples of maximal score) |
| [OT |                                   |                           |
| HER |                                   | Extreme disease activity  |
|  DI |                                   | with major impact on      |
| SEA |                                   | function                  |
| SE  |                                   |                           |
| ACT |                                   |                           |
| IVI |                                   |                           |
| TY] |                                   |                           |
| {.u |                                   |                           |
| nde |                                   |                           |
| rli |                                   |                           |
| ne} |                                   |                           |
+-----+-----------------------------------+---------------------------+

Specify:\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_
A B C D E NA

Specify:\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_
A B C D E NA

+---------+--------------------------------+--------------------------+
| ####    | **(Absent) (Maximum)**         | Overall evaluation for   |
| ### [GL |                                | the totality of disease  |
| OBAL EX |                                | activity in all          |
| TRA-SKE |                                | extra-skeletal muscle    |
| LETAL M |                                | systems                  |
| USCLE D |                                |                          |
| ISEASE  |                                | **(EXCLUDING MUSCLE      |
| ACTIVIT |                                | DISEASE ACTIVITY)**      |
| Y]{.und |                                |                          |
| erline} |                                |                          |
+---------+--------------------------------+--------------------------+

+--------+---------------------------------+---------------------------+
| ###### | **(Absent) (Maximum)**          | ###### Ma                 |
| # [MUS |                                 | ximum Value Guidelines (E |
| CLE DI |                                 | xamples of maximal score) |
| SEASE] |                                 |                           |
| {.unde |                                 | Severe muscle weakness    |
| rline} |                                 | resulting in being bed    |
|        |                                 | bound and an inability to |
| ###### |                                 | perform self care         |
| # [ACT |                                 |                           |
| IVITY] |                                 |                           |
| {.unde |                                 |                           |
| rline} |                                 |                           |
+--------+---------------------------------+---------------------------+

26\. Myositis: A B C D E NA

-   Severe muscle inflammation = category A

-   Moderate muscle inflammation = category B

-   Mild stable muscle inflammation = category C

27\. Myalgia C D E NA

+------+------------------------------------+-------------------------+
| GL   | **(Absent) (Maximum)**             | Overall evaluation for  |
| OBAL |                                    | the totality of disease |
| DIS  |                                    | activity in ALL         |
| EASE |                                    | systems, (**INCLUDING   |
|      |                                    | MUSCLE DISEASE          |
| #### |                                    | ACTIVITY)**             |
| ###  |                                    |                         |
| [GLO |                                    |                         |
| BAL  |                                    |                         |
| DISE |                                    |                         |
| ASE] |                                    |                         |
| {.un |                                    |                         |
| derl |                                    |                         |
| ine} |                                    |                         |
|      |                                    |                         |
| ##   |                                    |                         |
| #### |                                    |                         |
| # [A |                                    |                         |
| CTIV |                                    |                         |
| ITY] |                                    |                         |
| {.un |                                    |                         |
| derl |                                    |                         |
| ine} |                                    |                         |
|      |                                    |                         |
| #### |                                    |                         |
| ###  |                                    |                         |
+------+------------------------------------+-------------------------+

[MYOSITIS DISEASE ACTIVITY ASSESSMENT TOOL, Version 2 --
2005]{.underline}

This is a combined tool that captures the physician's assessment of
disease activity of various organ systems via the

MYOSITIS DISEASE ACTIVITY ASSESSMENT VISUAL ANALOGUE SCALES (MYOACT)

# 

# *[and]{.underline}* the presence or absence of clinical features via the 

# 

# MYOSITIS INTENTION TO TREAT ACTIVITY INDEX (MITAX)

MYOSITIS DISEASE ACTIVITY ASSESSMENT VISUAL ANALOGUE SCALES (MYOACT)

(Modified from the Vasculitis Activity Index, QE Whiting-O\'Keefe et al.
1999, Arthritis and Rheumatism [42]{.underline}: 2365-71)

Please assess the following systems using your expert clinical judgement
of how active the patient's myositis has been WITHIN THE PAST FOUR
WEEKS. Disease activity is defined as potentially reversible clinically
evident pathology or physiology resulting from the myositis disease
process. **Clinical findings known or suspected to be due to another
disease process or due to therapy should not be considered in this
evaluation.** Please rate your overall assessment of the ongoing current
disease activity for each of the systems listed by drawing a vertical
mark on the 10-cm. line for each system according to the following
scale: left end of line = no evidence of disease activity, midpoint of
line = moderate disease activity, and right end of line = extreme or
maximum disease activity. Please write in NA if the system cannot be
assessed. Under each organ system is listed a set of clinical and
laboratory findings that you should assess in order to determine the VAS
disease activity score for each organ system.

***[MYOACT scoring System:]{.underline}*** The proposed scoring system
for the MYOACT is the sum of the 10 cm visual analogue scale scores for
each of the six individual organ systems (CONSTITUTIONAL, CUTANEOUS,
SKELETAL, GASTROINTESTINAL, PULMONARY, CARDIAC) and divided by the total
possible score (range = 0 - 60). If one or more organ systems were not
assessed, the resulting score would be divided by the maximum possible
score from those assessed items. The categories of OTHER DISEASE
ACTIVITY, global extraskeletal muscle, muscle and GLOBAL disEASE
ACTIVITY are not included in the MYOACT score but are scored separately.

# 

**MYOSITIS INTENTION TO TREAT ACTIVITY INDEX (MITAX)**

(Modified BILAG Approach, EM Hay et al., 1993, Q J Med [86]{.underline}:
447-58)

The clinical features recorded are based upon:

1.  the presence of clinical features at this time or symptoms **within
    the previous four weeks that are due to active disease, and**

2.  the presumption that the feature is due to the myositis syndrome
    disease process

**For each item of the MITAX, please choose (circle one choice) among
the following options:**

A - If the feature is thought to be sufficiently active to require
disease-modifying treatment

B - If the feature is less active than A; moderate activity requiring
moderate therapies

C - If the feature stable or mild, requiring only symptomatic therapy

D - If the feature has resolved (no medication is required)

E - If the feature has never been present

NA - if the feature cannot be assessed

[***MITAX scoring system):***]{.underline} The scoring system is based
primarily on the physician's intention to treat (Categories A -- E). In
some cases, a patient may meet the clinical manifestations of a
particularly category, but the treatment does not match the intention to
treat of that particular category; in those cases, the category matching
the clinical symptoms should be marked. If more than one clinical
symptom is present within a system, and different categories would be
scored for each, mark the category that is most severe.

**Scoring system for the MITAX:**

+------------+---------------------------------------------------------+
| **CATEGORY | Denotes disease thought to be sufficiently active to    |
| A**        | require disease-modifying treatment:                    |
|            |                                                         |
| **A =      | \[High dose daily corticosteroids alone or in           |
| [A         | combination with high doses of other                    |
| ]{.underli |                                                         |
| ne}ctive** | immunosuppressives or intravenous gammaglobulin         |
|            | (IVIG)\]                                                |
| **(9       |                                                         |
| points)**  |                                                         |
+------------+---------------------------------------------------------+
| **CATEGORY | > Denotes disease that is less active than in "A";      |
| B**        | > reversible problems requiring moderate                |
|            | >                                                       |
| **B =      | > doses of corticosteroids and other immunosuppressive  |
| [B         | > agents, or use of antimalarials,                      |
| ]{.underli | >                                                       |
| ne}eware** | > non-steroidal anti-inflammatory drugs (NSAIDs),       |
|            | > topical steroids or tacrolimus. \[If                  |
| **(3       | > immunosuppressives                                    |
| points)**  | >                                                       |
|            | > or IVIG were used to treat signs and symptoms of      |
|            | > Category A, the doses of at least one                 |
|            | >                                                       |
|            | > agent would be reduced from levels required in        |
|            | > category A.\]                                         |
+------------+---------------------------------------------------------+
| **CATEGORY | Indicates stable mild disease, requiring only           |
| C**        | symptomatic therapy, such that                          |
|            |                                                         |
| **C =      | immunosuppressive therapy can be reduced below the      |
| [C]{.un    | level of category B.                                    |
| derline}on |                                                         |
| tentment** | \[Doses of corticosteroids, other immunosuppressive     |
|            | agents, antimalarials, NSAIDs or                        |
| **(1       |                                                         |
| point)**   | symptomatic therapies are likely to be low.\]           |
+------------+---------------------------------------------------------+
| >          | System previously affected but currently inactive and   |
| **CATEGORY | no medication is required                               |
| > D**      |                                                         |
| >          | (i.e., in remission)                                    |
| > **D =    |                                                         |
| > [D]{     |                                                         |
| .underline |                                                         |
| }iscount** |                                                         |
|            |                                                         |
| **(0       |                                                         |
| points)**  |                                                         |
+------------+---------------------------------------------------------+
| **CATEGORY | Indicates system never involved                         |
| E**        |                                                         |
|            |                                                         |
| **E = No   |                                                         |
| evidence   |                                                         |
| of this    |                                                         |
| system     |                                                         |
| being      |                                                         |
| active now |                                                         |
| or         |                                                         |
| pr         |                                                         |
| eviously** |                                                         |
|            |                                                         |
| **(0       |                                                         |
| points)**  |                                                         |
+------------+---------------------------------------------------------+

Each organ system of the MITAX is scored by selecting the highest
category selected for any of the individual items under that organ
system (i.e., the worse item carries the score for the organ system).
The proposed scoring system for the MITAX is the sum of the worst
category scores for each of the seven individual organ systems
(CONSTITUTIONAL, CUTANEOUS, SKELETAL, GASTROINTESTINAL, PULMONARY,
CARDIAC, MUSCLE) divided by the maximum possible score (range = 1 - 63).
If one or more organ systems were not assessed, the score would be
calculated by dividing the sum by the maximum possible score of the
assessed organ systems. The categories of OTHER DISEASE ACTIVITY, global
extraskeletal muscle and the GLOBAL disEASE ACTIVITY are not included in
the MITAX score but are scored separately.

# APPENDIX 1: GLOSSARY for the MYOSITIS DISEASE ACTIVITY ASSESSMENT TOOL, Version 2- 2005

**[Please Review Glossary for definitions prior to
assessment.]{.underline}**

> **[CONSTITUTIONAL]{.underline}**

1\. *Pyrexia:* refers to a documented fever in excess of 38°C.

2\. *Weight loss*: unintentional weight loss of greater than 5% of body
weight.

3\. *Fatigue*: sufficiently severe to interfere with normal daily
activities and not attributable to another cause.

### 

### **CUTANEOUS DISEASE ACTIVITY**

5\. *Cutaneous ulceration:* primary injury to dermis or deeper, due to
dermatomyositis.

*6. Erythroderma:* generalized widespread confluent erythema, involving
both sun-exposed and non-sun-exposed skin.

*7. Erythematous rashes:* including assessment of malar rash, facial
erythema, linear extensor erythema, V and shawl-sign rash, erythema in a
periungual distribution, and non-sun-exposed erythema.

> *Secondary changes:*
>
> *Necrosis:* epidermal necrosis manifesting as a superficial bullous
> lesion whose roof is gray in color from epidermal cell death,
> erosions, or scale/crusts, sitting on an erythematous base. These
> lesions are painful when acute.

*8. Panniculitis:* tender erythematous or violaceous nodules or
depressions due to inflammation in the subcutaneous fat.

*10. Heliotrope rash:* purple, lilac-colored or erythematous patches
over eyelids or in a periorbital distribution, often associated with
periorbital edema.

*11. Gottron\'s papules or sign:* erythematous to violaceous papules,
plaques or macules (sign) over extensor surfaces of joints, which are
sometimes scaly.

*12. Periungual capillary changes:* dilatation of periungual capillaries
which may be accompanied by vessel dropout and which is visible by naked
eye examination or by using additional magnification with otoscopy.

*13. Alopecia:* hair loss.

*Diffuse* = Nonscarring, non-erythematous widespread alopecia (telogen
effluvium)

*Focal* = Focal patchy alopecia with erythema (localized to areas of
inflammation)

*14. Mechanic\'s hands:* hyperkeratosis and scaling with frequent
fissuring and cracking along the lateral and palmar aspects of the
fingers.

### 

### **SKELETAL DISEASE ACTIVITY**

15\. *Arthritis*: active joint inflammation marked by tenderness, warmth
or swelling.

> Category A = *Severe active poly*a*rthritis:* arthritis of 2 or more
> joints with clinically significant loss of the functional range of
> movement of the involved joints and requiring assistance with
> activities of daily living.
>
> Category B = *Moderately active arthritis:* arthritis of one or more
> joints with some loss of functional range of movement of the involved
> joints, but not requiring assistance with activities of daily living
>
> Category C = *Mildly active arthritis:* arthritis of one or more
> joints with neither loss of range of motion nor impaired activities of
> daily living.

16\. *Polyarthralgias*: joint pain with stiffness due to active disease
in 2 or more joints.

### **GASTROINTESTINAL DISEASE ACTIVITY**

17\. *Gastrointestinal ulceration:* Clinical ulceration (severe
abdominal pain with GI blood loss) [or]{.underline} documented by
radiographic study (includes pneumatosis intestinalis) [o]{.underline}r
by biopsy.

18\. *Dysphagia*: difficulty swallowing, chewing or eating documented by
clinical symptoms [or]{.underline} by modified barium swallow
examination, manometry, or other objective approaches.

**If dysphagia has been stable and unchanged and is present for \> 6
months, this most likely represents damage and not activity, unless
there is good evidence of reversibility.**

> Category A *= Severe dysphagia:* accompanied by aspiration pneumonia,
> nasal regurgitation, or difficulty in protecting the airway;
>
> Category B *= Moderate dysphagia:* frequent or moderate symptomatic
> difficulty swallowing, chewing or eating.
>
> Category C *= Mild dysphagia:* occasional dysphagia [or]{.underline}
> subclinical dysphagia noted on objective testing; can eat regular
> diet.

19\. *Abdominal pain:* Pain in the abdominal area due to the myositis
disease process

> Category A *= Severe abdominal pain*, requiring hospitalization,
> treatment, [or]{.underline} bowel rest with nothing per oral route
> (NPO)

Category B *= Moderate abdominal pain*, requiring treatment, but not
hospitalization or NPO

Category C *= Mild abdominal pain* - no intervention required and does
not interfere with function.

**[PULMONARY DISEASE ACTIVITY]{.underline}**

20\. *Respiratory muscle weakness without intrinsic lung disease:*
Shortness of breath, dyspnea worsened with exertion [or]{.underline}
hypoventilation on pulmonary function testing **without** intrinsic lung
disease.

21\. *Interstitial lung disease:* Previously documented by radiography
or pathology. Primary assessment of *lung function* should be by
pulmonary function tests when available, but can be assessed by physical
exam when pulmonary function testing not available. Only active features
are graded, not those based on pulmonary fibrosis or irreversible
features.

22\. *Dysphonia*: alteration in voice quality, resonance, articulation
or speech rate from normal

*Moderate to severe dysphonia=* persistent voice symptoms or those that
interfere with communication

*Mild dysphonia =* intermittent voice symptoms not interfering with
communication

**[CARDIOVASCULAR DISEASE ACTIVITY]{.underline}**

23\. *Myocarditis or pericarditis*: Inflammation of the myocardium or
pericardium defined clinically or with echocardiographic or pathologic
evidence.

*24. Arrhythmia:* clinical or electrocardiographic evidence of irregular
heart beats

> *Severe arrhythmia =* symptomatic and requiring therapy or other
> intervention, excluding sinus tachycardia.

*Other cardiac arrhythmias =* symptomatic, but not requiring
intervention, excluding sinus tachycardia

**[OTHER DISEASE ACTIVITY]{.underline}**

Please specify features not listed above felt to be due to the myositis
disease process. Then on the VAS scale rate the severity of the
feature(s).

**[MUSCLE DISEASE ACTIVITY]{.underline}**

26\. *Myositis:* Muscle inflammation based upon manual muscle strength
testing, functional assessments, laboratory or other testing. In
patients with stable muscle atrophy and muscle damage, only weakness
attributable to active myositis is scored.

*Category A = Severe muscle inflammation,* requiring assistance with
activities of daily living and severe loss of function

*Category B = Moderate muscle inflammation:* not requiring assistance
with activities of daily living with moderate loss of function

*Category C = Stable mild muscle inflammation*: little or no loss of
function

27\. Myalgia: muscle pain or tenderness.

######### 

######### Appendix 2: MITAX SCORING A -- E system, Version 2- 2005

**[1) CONSTITUTIONAL DISEASE ACTIVITY]{.underline}**

CATEGORY A:

###  Pyrexia (\>38°C) **plus** 

Two other constitutional features

Unintentional weight loss

Fatigue/malaise/lethargy

Lymphadenopathy (\>1cm)

### Category B 

> Any ONE constitutional feature of moderate activity requiring category
> B therapy (i.e., moderate doses of corticosteroids other
> immunosuppressive agents or use of antimalarials, NSAIDs or topical
> steroids or tacrolimus).

### Category C 

> Presence of any one constitutional feature that is mild and stable,
> requiring only symptomatic therapy or therapy at doses below the level
> required in category B

CATEGORY D

Previously involved and no medication is currently required (in
remission).

CATEGORY E

No previous involvement

> **[2) CUTANEOUS DISEASE ACTIVITY]{.underline}**

CATEGORY A

Any one of the following features with severe activity requiring
category A therapy (disease modifying treatment in high dose):

> 1\. Cutaneous ulceration

2.  Erythroderma

3.  Erythema accompanied by vesiculobullous or erosive changes or
    necrosis

4.  Panniculitis

CATEGORY B

> Any one of the following features with moderate activity requiring
> category B therapy (i.e., moderate doses of corticosteroids other
> immunosuppressive agents or use of antimalarials, NSAIDs or topical
> steroids or tacrolimus):

1.  Any Category A cutaneous sign with moderate activity with therapy at
    doses below the level required in category A

2.  Erythema without secondary changes

3.  Heliotrope rash

> 4\. Gottron\'s papules or sign
>
> 5\. Periungual capillary changes
>
> 6\. Alopecia, diffuse
>
> 7\. Mechanic\'s hands

CATEGORY C

Any one of the following features with mild and stable activity
requiring category C therapy:

> 1\. Category A or B cutaneous signs with mild and stable activity,
> requiring only symptomatic therapy or therapy at doses below the level
> required in category B
>
> 2\. Alopecia, focal patchy with erythema

CATEGORY D

Previously involved and no medication is currently required (in
remission).

CATEGORY E

No previous involvement

**[3) SKELETAL DISEASE ACTIVITY]{.underline}**

CATEGORY A

Severe active polyarthritis with major loss of function (requiring
assistance with activities of daily living), requiring category A
therapy (disease modifying treatment in high dose).

CATEGORY B

> Moderately active arthritis with moderate loss of function requiring
> category B therapy (i.e., moderate doses of corticosteroids other
> immunosuppressive agents or use of antimalarials, NSAIDs or topical
> steroids or tacrolimus).

CATEGORY C

Any one of:

> 1\. Mild arthritis with no to mild loss of function, requiring only
> symptomatic therapy or therapy at doses below the level required in
> category B
>
> 2\. Polyarthralgias

CATEGORY D

Previously involved and no medication is currently required (in
remission).

CATEGORY E

No previous involvement

**[4) GASTROINTESTINAL DISEASE ACTIVITY]{.underline}**

CATEGORY A

Any one of the following features with severe activity requiring
category A therapy (disease modifying treatment in high dose):

> 1\. Gastrointestinal ulceration (with abdominal pain or GI blood loss)
>
> 2\. Moderate to severe dysphagia
>
> 3\. Severe abdominal pain \[requiring hospitalization, treatment,
> [or]{.underline} bowel rest with nothing per oral route (NPO).

CATEGORY B

> Any one of the following features with moderate activity requiring
> category B therapy (i.e., moderate doses of corticosteroids other
> immunosuppressive agents or use of antimalarials, NSAIDs or topical
> steroids or tacrolimus):
>
> 1\. Category A gastrointestinal signs or symptoms of moderate activity
> with therapy at doses below the level required in category A

2\. Mild dysphagia

> 3\. Moderate abdominal pain \[requiring treatment, but not
> hospitalization or NPO\].

CATEGORY C

Any one of the following features with mild and stable activity
requiring category C therapy:

> 1\. Category A or B gastrointestinal signs or symptoms with mild and
> stable activity, requiring only symptomatic therapy or therapy at
> doses below the level required in category B
>
> 2\. Mild abdominal pain \[no intervention required and does not
> interfere with function

CATEGORY D

Previous involvement and no medication currently required (i.e., in
remission)

CATEGORY E

No previous involvement

**[5) PULMONARY DISEASE ACTIVITY]{.underline}**

CATEGORY A

Any one of the following clinical features with severe activity
requiring category A therapy (disease modifying treatment in high dose):

> 1\. Dyspnea at rest from muscle weakness
>
> 2\. Active interstitial lung disease (ILD) with shortness of breath at
> rest or FVC (Forced Vital Capacity) or DLCO (Carbon monoxide diffusion
> capacity) \< 50% predicted or requirement for oxygen use or assisted
> ventilation

CATEGORY B

######### Any one of the following clinical features with moderate activity requiring category B therapy (i.e., moderate doses of corticosteroids other immunosuppressive agents or use of antimalarials, NSAIDs or topical steroids or tacrolimus):

1.  Category A pulmonary signs or symptoms of moderate disease activity
    with therapy at doses below the level required in category A

######### 2. Dyspnea on exertion from muscle weakness

######### 3. Active ILD with shortness of breath with normal activity or FVC or DLCO 50-- 70% predicted 

4\. Moderate to severe dysphonia

CATEGORY C

######### Any one of the following clinical features with mild and stable activity requiring category C therapy:

> 1\. Category A or B pulmonary signs or symptoms which are mild and
> stable activity, requiring only symptomatic therapy or therapy at
> doses below the level required in category B

2\. Active ILD with shortness of breath with exertional activity
[or]{.underline} FVC or DLCO \> 70% predicted

> 3\. Mild dysphonia

CATEGORY D

Previous involvement and no medication currently required (i.e., in
remission)

CATEGORY E

No previous involvement

**[6) CARDIOVASCULAR DISEASE ACTIVITY]{.underline}**

CATEGORY A

Any one of the following clinical features with severe activity
requiring category A therapy (disease modifying treatment in high dose):

> 1\. Echocardiography or biopsy evidence of myocarditis or pericarditis
> with clinical symptoms
>
> 2\. Severe cardiac arrhythmias

CATEGORY B

######### Any one of the following clinical features with moderate activity requiring category B therapy (i.e., moderate doses of corticosteroids other immunosuppressive agents or use of antimalarials, NSAIDs or topical steroids or tacrolimus):

> 1\. Category A cardiovascular signs or symptoms with moderate activity
> with therapy at doses below the level required in category A

######### 2. Myocarditis or pericarditis without clinical signs

> 3\. Other cardiac arrhythmias

CATEGORY C

#########  Any one of the following features with mild and stable activity requiring category C therapy:

> 1\. Category A or B signs or symptoms with mild and stable activity,
> requiring only symptomatic therapy or therapy at doses below the level
> required in category B
>
> 2\. Sinus tachycardia

CATEGORY D

Previous involvement and no therapy is currently required (i.e., in
remission)

CATEGORY E

No previous involvement

**[7) MUSCLE DISEASE ACTIVITY]{.underline}**

CATEGORY A

Severe muscle inflammation resulting overall in major loss of function
(requiring assistance with activities of daily living) based upon manual
muscle strength testing, functional assessments, laboratory or other
testing.

CATEGORY B

> Moderate muscle inflammation resulting overall in moderate loss of
> function (not requiring assistance with activities of daily living)
> based upon manual muscle strength testing, functional assessments,
> laboratory or other testing.

CATEGORY C

Any one of:

> 1\. Stable mild muscle inflammation, resulting overall in little or no
> loss of function \-- based upon manual muscle strength testing,
> functional assessments, laboratory or other testing
>
> 2\. Myalgia

CATEGORY D

Previous muscle inflammation and no medication currently required (i.e.,
in remission)

CATEGORY E

No evidence of previous muscle inflammation.