Total (n = 87) | Mi-2 (+) (n = 17) | Mi-2 (−) (n = 70) | P value | |
---|---|---|---|---|
Age at disease diagnosis (years) | 42.4 ± 13.8 | 43.1 ± 13.7 | 42.3 ± 14.0 | 0.819 |
Gender: female | 64 (73.6) | 13 (76.5) | 41 (72.9) | 1.000 |
Ethnicity: white | 69 (79.3) | 15 (88.2) | 44 (77.1) | 0.696 |
Disease duration (years) | 4.3 [2.9–7.1] | 4.5 [3.0–6.0] | 4.3 [2.5–7.5] | 0.945 |
Duration of follow-up time (years) | 4.3 [2.9–7.1] | 4.5 [3.0–6.0] | 4.3 [2.5–7.5] | 0.945 |
Constitutional symptoms (baseline) | 52 (59.8) | 11 (64.7) | 41 (58.6) | 0.786 |
Cumulative manifestations | ||||
Cutaneous manifestations | ||||
Gottron’s papules/sign | 84 (96.6) | 17 (100.0) | 67 (95.7) | 1.000 |
Heliotrope rash | 79 (90.8) | 15 (88.2) | 64 (91.4) | 0.651 |
Facial rash | 58 (66.7) | 14 (82.4) | 44 (62.9) | 0.247 |
Raynaud’s phenomenon | 40 (46.0) | 10 (58.8) | 30 (42.9) | 0.294 |
“V-neck” sign | 39 (44.8) | 9 (52.9) | 30 (42.9) | 0.412 |
“Shawl” sign | 21 (24.1) | 5 (29.4) | 16 (22.9) | 0.752 |
Vasculitis | 19 (21.8) | 1 (5.9) | 18 (25.7) | 0.103 |
Ulcers | 13 (14.9) | 3 (17.6) | 10 (14.3) | 0.715 |
Calcinosis | 3 (3.4) | 0 | 3 (4.3) | – |
Dysphagia | 43 (49.4) | 11 (64.7) | 32 (45.7) | 0.279 |
Articular involvement | 31 (35.6) | 3 (17.6) | 28 (40.0) | 0.094 |
Pulmonary involvement | 31 (35.6) | 1 (5.9) | 30 (42.9) | 0.004 |
Dyspnea | 29 (33.3) | 1 (5.9) | 28 (40.0) | 0.008 |
Incipient interstitial lung disease | 26 (29.9) | 1 (5.9) | 25 (35.7) | 0.017 |
Pulmonary fibrosis | 7 (8.0) | 0 | 7 (10.0) | – |
“Ground glass” | 11 (12.6) | 0 | 11 (15.7) | – |
Pneumomediastinum | 2 (2.3) | 0 | 2 (2.9) | – |
Creatine phosphokinase (U/L) | 992 [148–8040] | 6240 [3800–9148] | 484 [115–4880] | 0.001 |
Aldolase (U/L) | 18.5 [6.6–60.7] | 60.0 [35.0–138.0] | 12.1 [6.3–40.0] | 0.002 |