Patients | Age at Diagnosis, Yrs, Sex | Diagnosis | Rashes | Muscle Involvement | Pulmonary | Survival/prognosis | Other Key Features |
---|---|---|---|---|---|---|---|
1 | 47 M | Classic DM | Nonspecific rash on extremities | Mild proximal muscle weakness | ILD (RPILD) | Developed respiratory failure requiring intubation and died within 1 month | Myalgia, dysphagia, abnormal nailfold capillary |
2 | 38 M | Classic DM | Heliotrope rash, Gottron papules, necrotic ulcers | Mild proximal muscle weakness | No ILD | Alive (4-yr followup) | Dysphagia |
3 | 56 F | Classic DM | Gottron papules, cutaneous ulcers, digital tip ulceration with gangrene, heliotrope rash, palmar papules | Mild proximal muscle weakness | ILD | Alive (3-yr followup) | Sicca, RP, dysphagia, abnormal nailfold capillary, arthralgia |
4 | 39 F | Classic DM | Heliotrope rash, Gottron papule/sign | Mild proximal muscle weakness | No ILD | Alive (11-yr followup) | Myalgia, arthritis, abnormal nailfold capillary |
5 | 55 M | Classic DM | Mechanic’s hand, heliotrope rash | Mild proximal muscle weakness | ILD (RPILD) | Developed respiratory failure requiring intubation and died within 1 yr | RP, arthralgia, abnormal nailfold capillary |
6 | 46 M | Classic DM | Gottron papules | Moderate proximal muscle weakness | ILD (RPILD) and PAH | Respiratory status stabilized and patient remained alive (2-yr followup) | Myalgia, dysphagia, abnormal nailfold capillary |
7 | 9 F | JDM | NA | Moderate proximal muscle weakness | No ILD | Alive (14-yr followup) | Dysphagia |
8 | 2 F | JDM | NA | Severe proximal muscle weakness | No ILD | Alive (1-yr followup) | |
9 | 41 F | CADM | Gottron sign, malar rash, palmar papules | No weakness | ILD (RPILD) | Developed respiratory failure requiring intubation and died within 1 month | |
10 | 56 M | CADM | Gottron sign, violaceous rashes over extensor areas of hand, forearm, elbow, and knees, periorbital edema/rash and V-neck rash, livedoid rash | No weakness | ILD (RPILD) | Developed respiratory failure requiring intubation and died within 1 month | |
11 | 18 M | CADM | Gottron sign, digital tip ulcerations and cutaneous ulcerations, heliotrope rash | No weakness | No ILD | Alive (2-yr followup) | Abnormal capillary microscopy |
12 | 49 F | CADM | Gottron papules/sign, heliotrope rash | No weakness | No ILD | Alive (5-yr followup) | Abnormal nailfold capillary, mechanic’s hand |
13 | 58 F | CADM | Palmar papules and cutaneous ulcerations, Gottron papules/sign, heliotrope rash | No weakness | ILD (RPILD) | Developed respiratory failure requiring intubation and died within 1 yr | Abnormal nailfold capillary, arthralgia, arthritis, myalgia |
14 | 44 F | CADM | Nonspecific (biopsy-proven) | No weakness | No ILD | Alive (19-yr followup) | |
15 | 54 F | CADM | Gottron papules, severe cutaneous ulcers | No weakness | No ILD | Alive (8-yr followup) | Sicca, RP, abnormal nailfold capillary |
16 | 70 M | CADM | Gottron papules, cutaneous ulcers, digital tip ulceration, palmar papules | No weakness | ILD (RPILD) | Respiratory status stabilized and patient remained alive (1-yr followup) | Abnormal nailfold capillary |
Anti-MDA5: antimelanoma differentiation-associated gene 5 antibody; CADM: clinically amyopathic dermatomyositis; DM: dermatomyositis; JDM: juvenile DM; ILD: interstitial lung disease; RPILD: rapidly progressive ILD; NA: specific rash details not available; RP: Raynaud phenomenon; PAH: pulmonary arterial hypertension.