Table 2.
Clinical disease features, N = 119a
| Muscle Enzyme Elevation, N (%) | 99 (83.2) |
| MMT8b, median (IQR) | 63.5 (51.0–75.0), N = 32 |
| CMASc, median (IQR) | 43.5 (30.5–51.0), N = 34 |
| Skin Manifestations, N (%) | |
| 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) |
| CATd Score, median (IQR) | 4.0 (2.0–5.0) |
| CDASIe Activity Score, median (IQR) | 5.0 (3.0–10.0), N = 52 |
| Calcinosis, N (%) | 4 (3.4) |
| Overlap features, N (%) | |
| Raynaud phenomenon | 8 (5.0) |
| Sclerodactyly | 5 (3.1) |
| Constitutional symptoms, N (%) | |
| Fever | 13 (10.9) |
| Weight loss | 22 (18.5) |
| Fatigue | 71 (59.7) |
| Arthritis, N (%) | 33 (27.7) |
| Gastrointestinal symptoms, N (%) | |
| Abdominal Pain | 6 (5) |
| Dysphagia | 21 (17.6) |
| Pulmonary symptoms, N (%) | |
| 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 |
aN = 119 unless indicated in the table where missing data reduced the total number of patients analyzed
bManual-Muscle Testing 8
cChildhood Myositis Assessment Scale
dCutaneous Activity Tool
eCutaneous Dermatomyositis Area and Severity Instrument