Characteristics | ANA | Anti-ARS | Anti-MDA-5 | Anti-Mi-2 | Anti-Ro-52 |
---|---|---|---|---|---|
(n = 62) | (n = 13) | (n = 10) | (n = 10) | (n = 26) | |
Age at diagnosis (years) | 47.9 (16.1) | 47.2 (11.1)d | 45.8 (17.2) | 50.5 (12.6)d | 49 (13.9) |
Female gender | 40 (64.5) | 9 (69.2)e | 4 (40.0)a,e | 8 (80.0)e | 16 (61.5) |
White ethnicity | 49 (79.0) | 11 (84.6)e | 7 (70.0)e | 8 (80.0)e | 19 (73.1) |
Fever/weight loss | 56 (90.3)e | 12 (92.3)e | 10 (100)e | 10 (100)e | 25 (96.2)e |
Gottron’s sign | 61 (98.4)e | 12 (92.3)e | 10 (100)e | 10 (100)e | 26 (100)e |
Heliotrope rash | 57 (91.9)a,e | 9 (69.2)e | 9 (90.0)e | 7 (70.0)e | 21 (80.8) |
“V”-neck sign | 51 (82.3) | 5 (38.5)b,e | 10 (100)e | 8 (80.0)e | 20 (76.9)e |
“Shawl” sign | 37 (59.7) | 4 (30.8)a,e | 8 (80.0)e | 8 (80.0)e | 14 (53.8) |
Raynaud’s phenomenon | 42 (67.7)a | 7 (53.8)e | 7 (70.0)e | 7 (70.0)e | 19 (73.1) |
“Mechanics’ hands” | 15 (24.2) | 7 (53.8)a,e | 4 (40.0)e | 0e | 11 (42.3)b |
Digital ulcers | 13 (21.0) | 0e | 5 (50.0)b,e | 2 (20.0)e | 4 (15.4)e |
Vasculitis | 20 (32.3) | 1 (7.7)a,e | 8 (80.0)b,e | 3 (30.0)e | 9 (34.6) |
Calcinosis | 2 (3.2)e | 0e | 0e | 0e | 0e |
Muscle weakness | 19 (30.6) | 3 (23.1)e | 2 (20.0)e | 5 (50.0)e | 5 (19.2) |
Dysphagia | 45 (72.6) | 8 (61.5)e | 8 (80.0)e | 7 (70.0)e | 20 (76.9) |
Arthritis | 21 (33.9) | 8 (61.5)e | 7 (70.0)a,e | 1 (10.0)e | 15 (57.7)a |
ILD | 23 (37.1) | 9 (69.2)b,e | 4 (40.0)e | 0a,e | 13 (50.0) |
RP-ILD | 10 (16.1)e | 4 (30.8)e | 2 (20.0)e | 0e | 8 (30.8)e |
Maximum CPK (U/L) | 1245 (189–3931)d | 585 (216–5323)d | 158 (91–857)a,d | 4783 (3366–8915)b,d | 301 (117–2781)d |
ANA | – | 8 (61.5)e | 8 (80.0)e | 9 (90.0)e | 18 (69.2) |
Anti-ARS | 8 (12.9)c,e | – | 0e | 0e | 8 (30.8)e |
Anti-MDA-5 | 8 (12.9)c,e | 0e | – | 0e | 7 (26.9)a,e |
Anti-Mi-2 | 9 (14.5)c,e | 0e | 0e | – | 0a,e |
Anti-Ro-52 | 18 (29.0)c | 8 (61.5)a,e | 7 (70.0)a,e | 0a,e | – |
Severe infections | 32 (51.6) | 5 (38.5) | 5 (50.0)e | 5 (50.0)e | 12 (46.2) |
Neoplasia | 12 (19.4) | 2 (15.4)e | 1 (10.0)e | 0e | 5 (19.2) |
Complete clinical response | 31 (50.0) | 8 (61.5) | 4 (40.0)e | 6 (60.0)e | 14 (53.8) |
Disease remission | 22 (35.5) | 4 (30.8)e | 1 (10.0)e | 4 (40.0)e | 7 (26.9) |
Active disease | 9 (14.5) | 1 (7.7)e | 5 (50.0)a,e | 0e | 5 (19.2)e |
Death | 6 (9.7)e | 2 (15.4)e | 0e | 0e | 3 (11.5)e |