**Glossary for the myositis disease activity assessment TOOL (MDAAT)**

**[CONSTITUTIONAL]{.underline}**

1.  *[Pyrexia:]{.underline}* refers to a **documented** fever in excess
    of 38°C

2.  [*Weight loss*:]{.underline} unintentional weight loss of greater
    than 5% in body weight

3.  *[Fatigue:]{.underline}* sufficiently severe to interfere with
    normal daily activities not attributable to another cause

### **CUTANEOUS DISEASE ACTIVITY**

4.  *[Cutaneous ulceration:]{.underline}* extensive injury to dermis or
    deeper due to dermatomyositis

5.  *[Erythroderma:]{.underline}* generalized, widespread confluent
    erythema involving both sun-exposed and non sun-exposed skin with
    \>50% of body surface area involved

6.  *[Panniculitis:]{.underline}* tender erythematous or violaceous
    nodules or depressions due to inflammation in the subcutaneous fat

7.  *[Erythematous rashes]{.underline}* (includes malar rash, facial
    erythema, linear extensor erythema, V-sign, shawl sign, periungual
    erythema, sun-exposed or non sun-exposed erythema): (a) **Secondary
    change:** *erosions:* slightly depressed lesions with denuded
    epithelium; *vesiculobullous*: fluid-filled lesions ≤ 0.5cm
    (vesicles) or ≥ 0.5cm (bullae); *necrosis:* dead or devitalized
    tissue manifested as a black eschar, ulceration or wet or dry
    gangrene. (b) **Without secondary change:** erythema without
    accompanying changes to epidermis or dermis

8.  *[Heliotrope rash:]{.underline}* purple, lilac-colored or
    erythematous patches over eyelids or in a periorbital distribution,
    often associated with periorbital edema

9.  *[Gottron\'s papules or sign:]{.underline}* erythematous to
    violaceous papules, plaques or macules (sign) over extensor surfaces
    of joints, which are sometimes scaly

10. [*Periungual capillary changes:*]{.underline} 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

11. *[Alopecia:]{.underline}*

> a\) Diffuse: non-scarring, non-erythematous widespread alopecia
>
> b\) Focal: patchy alopecia with scaling and erythema localized to
> areas of inflammation (i.e. scalp rash of dermatomyositis)

12. *[Mechanic\'s hands:]{.underline}* hyperkeratosis and scaling with
    frequent fissuring and cracking along the lateral and palmar aspects
    of the fingers

### 

### **SKELETAL DISEASE ACTIVITY**

13. *[Arthritis:]{.underline}* active joint inflammation marked by
    tenderness, warmth or swelling

*a)* *Severe:* arthritis of **two or more** joints with clinically
significant loss of the functional range of movement and requiring
assistance with activities of daily living

*b) Moderate:* arthritis of **one or more** joints with some loss of
functional range of movement, but not requiring assistance with
activities of daily living

c\) *Mild*: arthritis of **one or more** joints with neither loss of
range of motion nor impaired activities of daily living

14\. [*Arthralgia*:]{.underline} joint pain with or without stiffness
but due to an inflammatory process in two or more joints

### **GASTROINTESTINAL DISEASE ACTIVITY**

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

> **If dysphagia has been stable and unchanged and is present for \> 6
> months, this is most likely to represent a damage item and should be
> scored as such unless there is good evidence of reversibility.**

*a) Severe:* accompanied by aspiration pneumonia, nasal regurgitation,
or difficulty in protecting the airway

*b) Moderate:* frequent or moderate symptomatic difficulty swallowing,
chewing or eating

*c) Mild*: occasional dysphagia [or]{.underline} asymptomatic dysphagia
noted on objective testing; can eat regular diet

16\. *[Abdominal pain:]{.underline}* pain in the abdominal area related
to the myositis disease process

> *a) Severe:* requiring hospitalization, treatment, [or]{.underline}
> bowel rest with nothing per oral route (NPO)
>
> *b) Moderate:* requiring treatment, but not hospitalization or NPO
>
> *c) Mild:* no intervention required and does not interfere with
> function

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

17\. *[Respiratory muscle weakness **without** interstitial lung disease
(ILD):]{.underline}* Shortness of breath worsened with exertion
[or]{.underline} hypoventilation on pulmonary function testing in the
absence of intrinsic lung disease

18\. *[Active reversible ILD:]{.underline}* Previously documented by
radiography or pathology. Only active features are graded, not those
based on pulmonary fibrosis or irreversible features.

\- In patients with new or significant deterioration in symptoms a
complete evaluation with pulmonary function testing (PFTs) and
radiography (chest x-ray or high resolution CT scan) is required. If
further investigation is necessary to elucidate the cause of symptoms,
then defer scoring until results are available.

\- 18a can always be answered clinically, but 18b and 18c may not be
answered if radiography and PFTs (respectively) have not been recently
performed.

> \- If there has been no significant change since last visit - score 2.
> If results normalize - score 0. If patient is clinically better but no
> recent radiography/PFTs are available, score as NA.
>
> **Any percentage change in PFTs is based on a change in the absolute
> value.** As a guide a significant change in PFTs is defined as a ≥10%
> change in FVC (minimum 200 ml for adults) or ≥15% in DLCO (minimum
> 3ml/min/mmHg for adults). However, in some patients smaller changes in
> FVC or DLCO may be suggestive of worsening disease in the context of
> worsening symptoms or imaging studies.

19\. [*Dysphonia*:]{.underline} alteration in voice quality, resonance,
articulation or speech rate from normal

> *a) Moderate to severe*: persistent voice symptoms or those that
> interfere with communication
>
> *b) Mild*: intermittent voice symptoms not interfering with
> communication

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

20\. *[Pericarditis:]{.underline}* Inflammation of the pericardium
defined clinically or by electrocardiogram (EKG) or echocardiogram

21\. [*Myocarditis*:]{.underline} Inflammation of the myocardium defined
clinically or with echocardiographic or other objective evidence

22\. *[Arrhythmia:]{.underline}* clinical or electrocardiographic
evidence of irregular heart beat

> *a) Severe arrhythmia:* symptomatic and requiring therapy or other
> intervention, excluding sinus tachycardia
>
> *b) Other cardiac arrhythmias*: symptomatic, but not requiring
> intervention, excluding sinus tachycardia

23\. *[Sinus tachycardia:]{.underline}* resting heart rate \> 100 beats
per minute in an adult patient or greater than upper limit of
age-appropriate normal value in a pediatric patient

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

24\. Specify feature that is felt to be due to the myositis disease
process. Then on the VAS rate the severity of this feature: 'max' would
be severe involvement with requirement for intensive care in the case of
a systemic feature, or extensive/generalized cutaneous involvement.

**[EXTRAMUSCULAR GLOBAL ASSESSMENT]{.underline}**

Overall evaluation for the disease activity in all extramuscular systems
(**excluding** muscle disease activity). **This is an IMACS Core Set
Measure.**

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

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

a\) *Severe muscle inflammation:* requiring assistance with activities
of daily living and severe loss of function

b\) *Moderate muscle inflammation*: not requiring assistance with
activities of daily living with some loss of function

c\) *Mild muscle inflammation:* little or no loss of function

26\. [*Myalgia*:]{.underline} muscle pain or tenderness

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

Physician judgment of overall disease activity based on and laboratory
assessments. **This is an IMACS Core Set Measure.**