Non-survival group (N = 32) | Survival group (N = 94) | P-value | |
---|---|---|---|
Female, no. (%) | 14 (43.8) | 64 (68.1) | 0.014 |
Age at onset, years, median [IQR] | 59 [55, 56] | 48 [39, 57] | < 0.001 |
Diagnosis time, months, median [IQR] | 1.0 [1.0, 3.0] | 2.0 [1.0, 5.0] | 0.036 |
Survival time, months, median [IQR] | 3.6 [1.7, 7.2] | 30.3 [15.0, 46.3] | < 0.001 |
Cause of death, no. (%) | |||
Respiratory failure | 30 (93.8) | – | – |
Gastrointestinal hemorrhage | 1 (3.1) | – | – |
Unknown | 1 (3.1) | – | – |
Laboratory data, median [IQR] | |||
ALT, U/L | 71 [30, 133] | 44 [24, 88] | 0.069 |
AST, U/L | 76 [48, 153] | 46 [29, 87] | 0.008 |
LDH, U/L | 372 [284, 478] | 301 [237, 368] | 0.008 |
CK, U/L | 85 [43, 276] | 60 [36, 103] | 0.079 |
Creatinine, μmol/L | 56 [41, 60] | 51 [41, 60] | 0.385 |
Ferritin, ng/mL | 1301 [523, 1500] | 532 [241, 1215] | 0.003 |
CRP, mg/L | 14 [6, 25] | 6 [3, 14] | 0.002 |
ESR, mm/h | 38 [28, 69] | 28 [19, 47] | 0.011 |
Total lymphocyte count, /µL | 605 [390, 785] | 745 [500, 1198] | 0.030 |
CD3+T lymphocyte count, /µL | 362 [254, 527] | 454 [292, 759] | 0.025 |
CD4+T lymphocyte count, /µL | 249 [118, 385] | 249 [162, 444] | 0.241 |
CD8+T lymphocyte count, /µL | 119 [66, 150] | 145 [83, 253] | 0.029 |
Clinical characteristics, no. (%) | |||
Myodynia | 10 (31.3) | 27 (28.7) | 0.786 |
Muscle weakness | 9 (28.1) | 26 (27.7) | 0.960 |
Heliotropic rash | 19 (59.4) | 57 (60.6) | 0.900 |
V sign | 12 (37.5) | 42 (44.7) | 0.478 |
Shawl sign | 9 (28.1) | 23 (24.5) | 0.681 |
Gottron’s sign | 25 (78.1) | 75 (79.8) | 0.841 |
Skin ulcer | 5 (15.6) | 9 (9.6) | 0.347 |
Mechanics hand | 10 (31.3) | 33 (35.1) | 0.691 |
Periungual erythema | 8 (25.0) | 23 (24.5) | 0.952 |
Dysphagia | 3 (9.4) | 7 (7.4) | 1.000 |
Malignancy | 3 (9.4) | 3 (9.4) | 0.348 |
Overlap CTDs | 0 (0) | 7 (7.4) | 0.190 |
Pulmonary function | |||
FVC, % pred, mean ± SD | 66 ± 21a | 75 ± 19b | 0.091 |
FEV1, % pred, mean ± SD | 66 ± 19a | 74 ± 18b | 0.093 |
DLco, % pred, mean ± SD | 49 ± 33c | 56 ± 17d | 0.489 |
TLC, % pred, median [IQR] | 67 [56, 78]c | 75 [64, 86]d | 0.213 |
ILD subtypes, no. (%) | |||
RP-ILD | 29 (90.6) | 9 (9.6) | < 0.001 |
C-ILD | 2 (6.3) | 79 (84.0) | < 0.001 |
Non-ILD | 1 (3.2) | 6 (6.4) | 0.804 |