# [IMACS FORM 08: MYOSITIS DAMAGE INDEX (MDI) - 2001]{.underline}

**[Please see the instructions and Myositis Damage Index Glossary of
Terms Prior to Assessment (pp. 5-8).]{.underline}**

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

+-------+---------------------------------+---------------------------+
| ###   | **(Absent) (Maximum)**          | ###### Ma                 |
| ####  |                                 | ximum Value Guidelines (E |
| [MUSC |                                 | xamples of maximal score) |
| LE]{. |                                 |                           |
| under |                                 | Severe muscle atrophy or  |
| line} |                                 | weakness resulting in     |
|       |                                 | being bed bound and an    |
| **[D  |                                 | inability to perform self |
| AMAGE |                                 | care                      |
| ]{.un |                                 |                           |
| derli |                                 |                           |
| ne}** |                                 |                           |
+-------+---------------------------------+---------------------------+

1\. Muscle atrophy (clinical) 0 1 NA

2\. Muscle weakness not attributable to active muscle disease 0 1 NA

######### 3. Muscle dysfunction: decrease in aerobic exercise capacity 0 1 NA 

######### 

######### 4. Muscle atrophy assessed by radiographic methods 0 1 NA 

######### 5. Low serum creatinine 0 1 NA

Creatinine value = \_\_\_\_ mg/dL or umol/L; lower limit normal value =
\_\_\_\_\_mg/dL or umol/L

+------+----------------------------------+---------------------------+
| ##   | **(Absent) (Maximum)**           | ###### Ma                 |
| #### |                                  | ximum Value Guidelines (E |
| # [S |                                  | xamples of maximal score) |
| KELE |                                  |                           |
| TAL] |                                  | -Life threatening         |
| {.un |                                  | fractures from            |
| derl |                                  | osteoporosis              |
| ine} |                                  |                           |
|      |                                  | -Avascular necrosis       |
| **[D |                                  | requiring arthroplasty    |
| AMAG |                                  |                           |
| E]{. |                                  |                           |
| unde |                                  |                           |
| rlin |                                  |                           |
| e}** |                                  |                           |
+------+----------------------------------+---------------------------+

6\. Joint contractures 0 1 NA

7\. Osteoporosis with fracture or vertebral collapse (excluding
avascular necrosis) 0 1 NA

8\. Avascular necrosis 0 1 NA

9\. Deforming arthropathy (including reducible deformities,

excluding avascular necrosis and contractures) 0 1 NA

######### 

######### 10. Osteoporosis without clinical fracture, requiring treatment of osteoporosis 0 1 NA

*11. Limitation of motion (Passive Joint ROM in degrees):*

#########  a. Elbow extension (degrees): R\_\_\_\_\_\_L\_\_\_\_\_\_\_ 0 1 NA

*b. Hip flexion (degrees): R\_\_\_\_\_\_L\_\_\_\_\_\_\_ 0 1 NA*

*c. Knee extension (degrees): R\_\_\_\_\_\_L\_\_\_\_\_\_\_ 0 1 NA*

d\. Ankle dorsiflesion (degrees) R\_\_\_\_\_\_L\_\_\_\_\_\_\_ 0 1 NA

+-------+---------------------------------+----------------------------+
| #     | **(Absent) (Maximum)**          | ######                     |
| ##### |                                 | Maximum Value Guidelines ( |
| # [CU |                                 | Examples of maximal score) |
| TANEO |                                 |                            |
| US]{. |                                 | Calcinosis with extensive  |
| under |                                 | subcutaneous exoskeleton   |
| line} |                                 | resulting in extreme loss  |
|       |                                 | of function (bedridden,    |
| **[D  |                                 | inability for self care)   |
| AMAGE |                                 |                            |
| ]{.un |                                 |                            |
| derli |                                 |                            |
| ne}** |                                 |                            |
+-------+---------------------------------+----------------------------+

12\. Calcinosis: 0 1 NA

*a. Calcinosis*, *superficial plaques or nodules* *0 1 NA*

b*. Calcinosis, tumoral 0 1 NA*

*c. Calcinosis, planar 0 1 NA*

*d. Calcinosis, exoskeleton/calcinosis universalis 0 1 NA*

13\. Alopecia 0 1 NA

14\. Cutaneous scarring or atrophy 0 1 NA

15\. Poikiloderma 0 1 NA

16\. Lipodystrophy 0 1 NA

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

+-----------+-----------------------------+---------------------------+
| ##        | **(Absent) (Maximum)**      | ###### Ma                 |
| ##### [GA |                             | ximum Value Guidelines (E |
| STROINTES |                             | xamples of maximal score) |
| TINAL]{.u |                             |                           |
| nderline} |                             | Extreme dysfunction       |
|           |                             | requiring total           |
| ######    |                             | parenteral nutrition      |
| ## DAMAGE |                             |                           |
+-----------+-----------------------------+---------------------------+

17\. Dysphagia 0 1 NA

18\. Gastrointestinal dysmotility, constipation, diarrhea or abdominal
pain 0 1 NA

19\. Infarction or resection of bowel or other gastrointestinal organs 0
1 NA

######### 20. Steatosis 0 1 NA

+--------+-------------------------------+----------------------------+
| #      | **(Absent) (Maximum)**        | ######                     |
| ###### |                               | Maximum Value Guidelines ( |
|  [PULM |                               | Examples of maximal score) |
| ONARY] |                               |                            |
| {.unde |                               | \- Fibrosis requiring      |
| rline} |                               | ventilatory support; or    |
|        |                               |                            |
| **[DAM |                               | -Pulmonary hypertension    |
| AGE]{. |                               | resulting in extreme loss  |
| underl |                               | of function (bedridden,    |
| ine}** |                               | inability for self care)   |
+--------+-------------------------------+----------------------------+

21\. Dysphonia 0 1 NA

22\. Impaired lung function due to respiratory muscle damage 0 1 NA

23\. Pulmonary fibrosis 0 1 NA

24\. Pulmonary hypertension 0 1 NA

*25. Diminished lung function:*

*a. DL-CO/Va (Carbon monoxide diffusing capacity corrected for alveolar
volume)(% Predicted =\_\_\_\_\_%) 0 1 NA*

#########  b. FEV1 (forced expiratory volume in 1 second) (% predicted = \_\_\_\_\_\_%) 0 1 NA

+----------+-------------------------------+--------------------------+
| ####     | **(Absent) (Maximum)**        | ###### Maxi              |
| ### [CAR |                               | mum Value Guidelines (Ex |
| DIOVASCU |                               | amples of maximal score) |
| LAR]{.un |                               |                          |
| derline} |                               | \--Myocardial            |
|          |                               | dysfunction resulting in |
| **[DAMAG |                               | extreme loss of function |
| E]{.unde |                               | (bedridden, inability    |
| rline}** |                               | for self care)           |
+----------+-------------------------------+--------------------------+

26\. Hypertension requiring treatment \> 6 months 0 1 NA

27\. Ventricular dysfunction / cardiomyopathy 0 1 NA

[Assessed in Adult Patients \> = 18 years of age:]{.underline}

28\. Angina or coronary artery bypass 0 1 NA

29\. Myocardial infarction 0 1 NA

+--------+---------------------------------+---------------------------+
| *      | **(Absent) (Maximum)**          | ###### Ma                 |
| *[PERI |                                 | ximum Value Guidelines (E |
| PHERAL |                                 | xamples of maximal score) |
| VASCU  |                                 |                           |
| LAR]{. |                                 | \- Limb loss; or          |
| underl |                                 | -Thrombosis requiring     |
| ine}** |                                 | intensive care unit (ICU) |
|        |                                 | care; or-Severe disease   |
| **[DAM |                                 | resulting in extreme loss |
| AGE]{. |                                 | of function (bedridden,   |
| underl |                                 | inability for self care)  |
| ine}** |                                 |                           |
+--------+---------------------------------+---------------------------+

30\. Tissue or pulp loss 0 1 NA

31\. Digit or limb loss or resection 0 1 NA

32\. Venous or arterial thrombosis with swelling, ulceration or venous
stasis 0 1 NA

[Assessed in Adult Patients \> = 18 years of age:]{.underline}

33\. Claudication 0 1 NA

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

+-------+---------------------------------+---------------------------+
| #     | **(Absent) (Maximum)**          | ###### Ma                 |
| ##### |                                 | ximum Value Guidelines (E |
| # [EN |                                 | xamples of maximal score) |
| DOCRI |                                 |                           |
| NE]{. |                                 | Extreme disease resulting |
| under |                                 | in ICU care or life       |
| line} |                                 | threatening               |
|       |                                 | complications, such as    |
| ##### |                                 | ICU stay for seizures or  |
| ### D |                                 | DKA; renal failure,       |
| AMAGE |                                 | amputation                |
+-------+---------------------------------+---------------------------+

[Assessed in Patients with diagnosis before 18 years of
age:]{.underline}

34\. Growth failure 0 1 NA

35\. Delay in development of secondary sexual characteristics 0 1 NA

[Assessed in Both Pediatric and Adult Patients:]{.underline}

36\. Hirsutism or Hypertrichosis 0 1 NA

37\. Irregular menses 0 1 NA

38\. Primary or Secondary Amenorrhea 0 1 NA

39\. Diabetes 0 1 NA

######### 40. Hyperlipidemia (regardless of treatment) 0 1 NA

[Assessed in Adult Patients (\> = 18 years of age and adolescent
patients when applicable)]{.underline}

41\. Infertility (female or male) 0 1 NA

42\. Sexual dysfunction 0 1 NA

+------+----------------------------------+---------------------------+
| #### | **(Absent) (Maximum)**           | ###### Ma                 |
| ###  |                                  | ximum Value Guidelines (E |
| [OCU |                                  | xamples of maximal score) |
| LAR] |                                  |                           |
| {.un |                                  | Complete loss of vision   |
| derl |                                  | in both eyes              |
| ine} |                                  |                           |
|      |                                  |                           |
| **[D |                                  |                           |
| AMAG |                                  |                           |
| E]{. |                                  |                           |
| unde |                                  |                           |
| rlin |                                  |                           |
| e}** |                                  |                           |
+------+----------------------------------+---------------------------+

43\. Cataract resulting in visual loss 0 1 NA

44\. Visual loss, other, not secondary to cataracts 0 1 NA

+------+----------------------------------+---------------------------+
| ###  | **(Absent) (Maximum)**           | ###### Ma                 |
| #### |                                  | ximum Value Guidelines (E |
|  [IN |                                  | xamples of maximal score) |
| FECT |                                  |                           |
| ION] |                                  | Infection resulting in    |
| {.un |                                  | septic shock or life      |
| derl |                                  | threatening complications |
| ine} |                                  |                           |
+------+----------------------------------+---------------------------+

45\. Chronic infection 0 1 NA

Specify:\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_

Specify:\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_

46\. Multiple infections 0 1 NA

Specify:\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_

Specify:\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_

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

+-------+--------------------------------+----------------------------+
| ##    | **(Absent) (Maximum)**         | ######                     |
| ##### |                                | Maximum Value Guidelines ( |
|  [MAL |                                | Examples of maximal score) |
| IGNAN |                                |                            |
| CY]{. |                                | Malignancy resulting in    |
| under |                                | ICU care or life           |
| line} |                                | threatening complications  |
+-------+--------------------------------+----------------------------+

47\. Any form of cancer, Specify type, grade and
stage:\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ 0 1
NA

+------+---------------------------------+----------------------------+
| ###  | **(Absent) (Maximum)**          | ######                     |
| #### |                                 | Maximum Value Guidelines ( |
|  [OT |                                 | Examples of maximal score) |
| HER  |                                 |                            |
| DAMA |                                 | Extreme disease damage     |
| GE,  |                                 | resulting in ICU care or   |
| spec |                                 | life threatening           |
| ify] |                                 | complications              |
| {.un |                                 |                            |
| derl |                                 |                            |
| ine} |                                 |                            |
+------+---------------------------------+----------------------------+

48\. *Death 0 1 NA*

*Cause and
date:\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_*

49\.
Specify:\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_

50\.
Specify:\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_

+------+---------------------------------+----------------------------+
| ###  | **(Absent) (Maximum)**          | ######                     |
| #### |                                 | Maximum Value Guidelines ( |
|  [GL |                                 | Examples of maximal score) |
| OBAL |                                 |                            |
|  DAM |                                 | None                       |
| AGE] |                                 |                            |
| {.un |                                 |                            |
| derl |                                 |                            |
| ine} |                                 |                            |
+------+---------------------------------+----------------------------+

# 

# 

# 

# 

# [IMACS MYOSITIS DAMAGE INDEX (MDI) - 2001]{.underline}

**(Modification of the SLICC/ACR Damage Index, Brit J Rheum 1996; 35:
248-54)**

Damage is defined as persistent changes in anatomy, physiology,
pathology or function, which are present for at least 6 months. Damage
may be the result of prior active disease (causing scarring, fibrosis
and atrophy), complications of therapy, co-morbid conditions, or other
events. A portion of disease damage is disease chronicity. Features of
damage are ascertained by clinical assessment and must be present for at
least 6 months (or the pathology that led to the feature must have been
present for at least 6 months) despite prior immunosuppressive or other
therapy, including exercise and rehabilitation. Only items present since
date of diagnosis should be included. Damage is often permanent and
cumulative. Damage scores most often increase over time, but in some
cases may decline (i.e., a manifestation which was previously present
which has currently resolved would receive a score of 0 in the present
assessment).

For each organ system, please assess the severity and extent of damage
exhibited by the patient at this time. To assess the severity, please
rate your overall assessment of the current disease damage for each of
the systems below by drawing a vertical mark on the 10-cm. line
according to the following scale: -Left end of line = no evidence of
disease damage,

> -Midpoint of line = moderate disease damage, and
>
> -Right end of line = extreme or maximum disease damage.
>
> -Please write in NA if the system cannot be assessed.

To assess the extent of damage in that organ system, please indicate the
following for the specific items that are assessed:

> -A score of 0 indicates the item has never been present.
>
> -A score of 1 indicates the damage manifestation at a single point in
> time that has been present for at least six months.
>
> -NA = cannot be assessed.

*At the end of each organ system section, items in italics represent
specialised objective testing items that are optional for the assessment
and will be scored separately in an extended score. When absolute values
are requested, please provide these even if they are within normal
limits*.

**[IMACS MYOSITIS DAMAGE INDEX Scoring System]{.underline}**

> The MDI has three proposed scores: an **extent of damage score**, a
> **severity of damage score** and an **extended damage score.**

The proposed scoring system for the **MDI extent of damage score** is
the sum of all 0 or 1 scores for the eleven individual organ systems
(MUSCLE, SKELETAL, CUTANEOUS, GASTROINTESTINAL, PULMONARY,
CARDIOVASACULAR, PERIPHERAL VASCULAR, ENDOCRINE, OCULAR, INFECTION,
MALIGNANCY) divided by the total possible score (range = 0 -- 35 in
children, 0-37 for adolescents and 0-38 in adults). If one or more items
were not assessed, the resulting score would be divided by the maximum
possible score of the assessed items. The categories of OTHER DAMAGE and
global DAMAGE are not included in the MDI extent of damage score but are
scored separately.

The proposed **MDI severity of damage score** is the sum of the 10 cm
visual analogue scale scores for each of the eleven individual organ
systems (MUSCLE, SKELETAL, CUTANEOUS, GASTROINTESTINAL, PULMONARY,
CARDIOVASACULAR, PERIPHERAL VASCULAR, ENDOCRINE, OCULAR, INFECTION,
MALIGNANCY) divided by the total possible score (range = 0 -- 110). If
one or more organ systems were not assessed, the resulting score would
be divided by the maximum possible score of the assessed items. The
categories of OTHER DAMAGE and GLOBAL DAMAGE are not included in the MDI
severity score but are scored separately.

The proposed **MDI extended damage score** is the sum of the optional
items listed in italics under the systems MUSCLE, SKELETAL,
GASTROINTESTINAL, PULMONARY, ENDOCRINE divided by the total possible
score. Each optional item is scored 0 or 1, providing a range of 0 -- 16
for the extended damage score. If one or more items were not assessed,
the resulting score would be divided by the maximum possible score
assessed.

# [IMACS MYOSITIS DAMAGE INDEX Glossary of Terms]{.underline}

### **MUSCLE DAMAGE**

1.  *Muscle atrophy*: decreased muscle mass assessed by clinical exam

2.  *Muscle weakness not attributable to active muscle disease:*
    weakness present for at least 6 months, demonstrated on clinical
    examination, not thought to be due to active muscle inflammation
    based on assessments of clinical and laboratory measures, such as
    serum muscle enzymes, magnetic resonance imaging, or repeat muscle
    biopsies.

3.  *Muscle dysfunction*, decrease in aerobic exercise tolerance by
    clinical history **[or]{.underline}** assessed by aerobic exercise
    testing, due to muscle damage and not attributable to cardiac,
    pulmonary, psychologic or other factors.

4.  *Muscle atrophy:* assessed by radiographic methods, including T1
    MRI, CT scan, DEXA scan (body composition) (optional assessment

**[SKELETAL DAMAGE]{.underline}**

5.  *Joint contractures*: fixed limitation in the normal range of motion
    of joints, in the absence of synovitis, excluding reducible
    deformities, avascular necrosis and deforming arthropathy.

6.  *Osteoporosis with fracture or vertebral collapse (excluding
    avascular necrosis):* demonstrated by any imaging technique.

7.  *Avascular necrosis:* demonstrated by any imaging technique.

8.  *Osteoporosis without clinical fracture:* requiring treatment of
    osteoporosis beyond calcium and vitamin D (prophylactic) therapy,
    demonstrated by any imaging technique (OPTIONAL ASSESSMENT)

**[CUTANEOUS DAMAGE]{.underline}**

9.  *Calcinosis:* dystrophic calcification, observed clinically or
    radiologically in the skin, subcutaneous tissue, fascia, or muscle.
    Calcinosis can be clinically or radiographically distinguished into
    4 subtypes (Blane CE et al, 1984, AJR, 142: 397-400): (*the 4
    subtypes are part of the OPTIONAL ASSESSMENT*)

    a.  *Superficial plaques or nodules:* circumscribed lesions confined
        to the cutaneous or subcutaneous tissue

    b.  *Tumoral:* large circumscribed nodules, which are intramuscular.
        Can ulcerate if subcutaneous.

    c.  *Planar or fascial:* linear accumulations of calcinosis that are
        along the fascial plane of subcutaneous tissue or muscles.

    d.  *Exoskeleton/calcinosis universalis:* widespread sheets of
        calcium in the muscle, fascia and subcutaneous tissue, often
        encasing the patient. Can ulcerate when subcutaneous.

10. *Alopecia:* hair loss with scarring present

## Cutaneous scarring or atrophy: dermal or epidermal, with or without telangiectasia 

11. *Poikiloderma:* fine speckled pattern of hyper- and hypopigmented
    macules interspersed with telangiectasias and cutaneous atrophy
    *(requires all 3 features)* usually in areas of photosensitivity

12. *Lipodystrophy:* loss of subcutaneous fat (localised or widely
    distributed) observed clinically or radiologically.

### **GASTROINTESTINAL DAMAGE**

13. *Dysphagia:* persistent difficulty swallowing by history or
    persistent changes documented by radiography or other objective
    measures.

14. *Infarction or resection of bowel or other GI organs*, by history.

15. *Steatosis (OPTIONAL ASSESSMENT):* persistent fatty changes of
    liver, documented by ultrasound, CT scan or biopsy on at least 1
    occasion, with persistent changes on reexamination

#### **PULMONARY DAMAGE**

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

17. *Impaired lung function due to respiratory muscle damage:* shortness
    of breath not thought to be due to active muscle inflammation or
    intrinsic pulmonary disease

18. *Pulmonary fibrosis:* shortness of breath **[or]{.underline}** rales
    on physical exam for at least 6 months, with previously documented
    abnormal chest radiograph, computed tomography scan, or biopsy
    evidence of interstitial lung disease (ILD)/pulmonary fibrosis.

19. *Pulmonary hypertension:* Right ventricular prominence,
    **[or]{.underline}** loud P2, **[or]{.underline}** by direct
    measurement of pulmonary pressures (greater than 10% above upper
    limit of normal).

### **CARDIOVASCULAR DAMAGE**

20. *Hypertension:* Diagnosed by *b*lood pressure \> 95% of upper limits
    of normal for age and gender, requiring treatment \> 6 months

21. *Ventricular dysfunction/Cardiomyopathy:* ventricular dysfunction
    documented clinically or by echocardiography.

[Assessed only in adult patients:]{.underline}

22. *Angina:* episodes of angina present for a period of at least 6
    months.

23. *Myocardial infarction*: documented by electrocardiogram and
    enzymes.

## **[PERIPHERAL VASCULAR DAMAGE]{.underline}**

## Tissue or pulp loss: tissue loss such as pulp space loss or loss less than entire digit. 

[Assessed only in adult patients:]{.underline}

24. *Claudication:* by history.

### **ENDOCRINE DAMAGE**

[Assessed only in Patients \< 18 years of age:]{.underline}

25. *Growth failure:* Two of the following three features*:*

```{=html}
<!-- -->
```
a.  Less than 3^rd^ percentile height for age

b.  Growth velocity over 6 months less than 3^rd^ percentile for age

c.  Crossing at least 2 centiles (5%, 10%, 25%, 50%, 75%, 95%) on growth
    chart

> (See
> <http://www.cdc.gov/nchs/about/major/nhanes/growthcharts/clinical_charts.htm>
> to obtain growth charts for U.S. patients)

26. *Delay in development of secondary sexual characteristics:* greater
    than 2 standard deviations beyond mean for age in Tanner staging
    (see Harriet Lane Handbook: A Manual for Pediatric House Officers,
    Ed. G.K. Sidberry, **[or]{.underline}** Tanner, JM and Davies, PS,
    *J Pediatr* 1985; 107:317-29 for age-defined mean values for North
    American children).

[Assessed in both pediatric and adult patients:]{.underline}

27. *Hirsutism:* excessive terminal hair growth in an adult male
    distribution, i.e., lip, chin, chest, back. This is to be assessed
    only in girls, women and pre-pubertal boys. *Hyptertrichosis:*
    Generalised increase in body hair.

28. *Irregular menses:* Missing more than one ovulatory menstrual cycle
    (i.e., 24 -- 37 days in duration) in one year, assessed at least 3
    years after menarche or before menopause.

29. *Primary amenorrhea:* the absence of any menstruation within 4.5
    years of reaching puberty (i.e., in a girl without genital
    malformations who has reached her complete morphological
    development, including a bone age of at least 15 years, breasts for
    at least 2 years, and adult type pubic hair)

30. *Secondary amenorrhea*: cessation of menstrual periods before
    menopause, after initially menstruating

31. *Diabetes mellitus:* fasting glucose \> 140 mg/dl or 2 hour glucose
    in Oral Glucose Tolerance Test \> 200 mg/dl

32. *Hyperlipidemia (OPTIONAL ASSESSMENT):* Cholesterol or lipoprotein
    levels \>95% of age appropriate reference ranges

33. References: Summary of the Second Report of the National Cholesterol
    Education Program (NCEP) Expert Panel on Detection, Evaluation, and
    Treatment of High Blood Cholesterol in Adults (Adult Treatment Panel
    II). JAMA. 1993; 269:3015-3023; "Report of the expert panel on blood
    cholesterol levels in children and adolescents" by the National
    Cholesterol Education program (NCEP), NIH Publication No 91-2732,
    September 1991.

[Assessed in adult patients and adolescents when
applicable:]{.underline}

34. *Infertility (female or male):* no pregnancy after one year
    attempting to conceive via regular sexual intercourse

35. *Sexual dysfunction:* patient dissatisfaction with sexual function
    (female or male)

#### **OCULAR DAMAGE**

36. *Cataract:* lens opacity in either eye, ever, whether primary or
    secondary to steroid therapy, documented by ophthalmoscopy, and
    resulting in visual loss.

37. *Visual loss, other*: legal blindness, with vision less than or
    equal to 20/200.

### **INFECTION**

38. Chronic infection: Infection (presumptive diagnosis with or without
    positive culture) requiring \> 6 months of antimicrobial treatment,
    OR persisting with or without clinical symptoms \> 6 months and
    associated with disability (absence from school, work, day-care)

39. *Multiple infections:* \> 3 infections (at same or different sites,
    with same or different organisms) requiring antimicrobial treatment
    or disability (absence from school, work, day-care) over a 6 month
    period

### 

### **MALIGNANCY**

40. Documented by pathology, excluding dysplasias, including myositis
    associated malignancies.

### **OTHER**

41. **Death** should be recorded under OTHER, and the date and cause of
    death noted (OPTIONAL ASSESSMENT)

42. Any other features of myositis damage not listed but felt to be of
    importance to the patient or physician. Please specify the feature
    in as much detail as possible. (These are not scored)

######## GLOBAL DAMAGE ASSESSMENT

43. Your expert clinical judgement of the totality of disease damage in
    all systems