Table 2.
Clinical features, ever present | Anti-HSC70 positive (N = 22) | Anti-HSC70 negative (N = 41) | P-value |
---|---|---|---|
Skin involvement, n/N (%) | |||
Cutaneous ulcer | 13/22 (59) | 7/41 (17) | <0.002 |
Periungual capillary dilatation | 22/22 (100) | 38/41 (93) | 0.5 |
Malar erythemaa | 18/22 (82) | 22/41 (54) | <0.04 |
Oedema | 9/22 (41) | 7/41 (17) | 0.07 |
Calcinosis | 12/22 (55) | 16/41 (39) | 0.3 |
Systemic involvement, n/N (%) | |||
Weight loss | 11/22 (50) | 21/41 (51) | 1.0 |
Fever | 11/22 (50) | 7/41 (17) | <0.009 |
Muscle involvement, n/N (%) | |||
Distal weakness | 16/22 (73) | 27/41 (66) | 0.8 |
Proximal and distal weakness | 16/22 (73) | 27/41 (66) | 0.8 |
Muscle atrophy | 12/22 (55) | 16/41 (39) | 0.3 |
Lung involvement, n/N (%) | |||
Dyspnea on exertion | 13/22 (59) | 15/41 (37) | 0.1 |
Dyspnea at rest | 1/22 (5) | 9/41 (22) | 0.1 |
Interstitial lung disease | 4/22 (18) | 2/41 (5) | 0.2 |
Joint involvement, n/N (%) | |||
Arthralgia | 16/22 (73) | 33/41 (80) | 0.5 |
Arthritis | 14/22 (64) | 24/41 (59) | 0.8 |
Gastrointestinal involvement, n/N (%) | |||
Regurgitation | 8/22 (36) | 11/41 (27) | 0.6 |
Abdominal paina | 13/22 (59) | 13/39 (33) | 0.06 |
Early total symptom score, median (IQR) | 0.33 (0.23–0.38) | 0.28 (0.20–0.35) | 0.2 |
Early muscle score | 0.43 (0.29–0.57) | 0.43 (0.29–0.57) | 0.5 |
Early joint score | 0.50 (0.50–1.0) | 0.50 (0.50–1.0) | 0.9 |
Early cutaneous score | 0.38 (0.33–0.44) | 0.38 (0.31–0.44) | 0.9 |
Early gastrointestinal score | 0.13 (0–0.25) | 0 (0–0.13) | 0.09 |
Early pulmonary score | 0.20 (0–0.20) | 0 (0–0.20) | <0.04 |
Early constitutional score | 0.38 (0.25–0.69) | 0.25 (0.25–0.5) | 0.6 |
Muscle enzymes (highest value), median (IQR), IU/l | |||
AST | 122 (48–202) | 85 (46–127) | 0.3 |
ALT | 58 (38–107) | 54 (30–127) | 0.7 |
LDHb | 488 (346–808) | 266 (207–377) | <0.002 |
CK | 1154 (286–3537) | 800 (278–3082) | 0.7 |
Severity at onset, median (IQR) | 2.0 (2.0–3.0) | 2.0 (2.0–3.0) | 1.0 |
Categorical variables were analysed by Fisher's exact tests and are presented as count/total (percentage). Continuous variables were analysed by the Mann–Whitney U-test and are expressed as median (IQR [Q1–Q3]).
In those with the history during the follow-up period after onset of first symptom.
All values of LDH were adjusted for the NIH upper limit of normal for LDH in adults, which is 226 U/l. ALT: alanine aminotransferase; AST: aspartate aminotransferase; CK: creatine kinase; IQR: interquartile range; LDH: lactate dehydrogenase.