Table 2 Clinical disease features, N = 119^a

Muscle Enzyme Elevation, N (%)

99 (83.2)

MMT8^b, median (IQR)

63.5 (51.0–75.0), N = 32

CMAS^c, median (IQR)

43.5 (30.5–51.0), N = 34

 Gottron’s papules or sign

90 (75.6)

 Malar or facial erythema

78 (65.5)

 Periungal capillary loop changes

70 (58.8)

 Heliotrope Rash

64 (53.8)

 Linear Erythema

33 (27.7)

 Cuticular overgrowth

24 (20.2)

 Non-sun exposed erythema

23 (19.3)

 Extensive cutaneous erythema

17 (14.3)

 Shawl Sign

13 (10.9)

 Cutaneous Ulceration

13 (10.9)

 Subcutaneous edema

11 (9.2)

 V sign

10 (8.4)

 Mucus membrane lesions

6 (5)

 Livedo reticularis

6 (5)

 Mechanic’s hands

6 (5)

 Alopecia

4 (3.4)

CAT^d Score, median (IQR)

4.0 (2.0–5.0)

CDASI^e Activity Score, median (IQR)

5.0 (3.0–10.0), N = 52

Calcinosis, N (%)

4 (3.4)

 Raynaud phenomenon

8 (5.0)

 Sclerodactyly

5 (3.1)

 Fever

13 (10.9)

 Weight loss

22 (18.5)

 Fatigue

71 (59.7)

Arthritis, N (%)

33 (27.7)

 Abdominal Pain

6 (5)

 Dysphagia

21 (17.6)

 Interstitial lung disease

1 (0.8)

 Dysphonia

14 (11.8)

Cardiovascular involvement, N (%)

1 (0.8)

Physician Global Assessment, median (IQR)

4.0 (2.5–5.0), N = 106

Physician Global Muscle Disease Activity, median (IQR)

3.0 (1.0–5.0), N = 78

Physician Global Skin Disease Activity, median (IQR)

2.0 (1.0–4.0), N = 71

Physician Global Extramuscular Disease activity, median (IQR)

0.25 (0.0–3.25), N = 72

 Constitutional

2.0 (0.0–2.0), N = 84

 Skeletal

0.0 (0.0–2.0), N = 88

 Gastrointestinal

0.0 (0.0–0.4), N = 90

 Pulmonary

0.0 (0.0–0.0), N = 89

 Cardiovascular

0.0 (0.0–0.0), N = 88