Table 1.
Comparison of demographics, clinical and laboratory data, primary pulmonary function parameters, and ILD subtypes in patients with MDA5+DM between the survival and non-survival groups
| 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 |
Bold text highlights the significance
MDA5+DM Anti-MDA5 dermatomyositis, ALT Alanine transaminase, AST Aspartate transaminase, LDH Lactate dehydrogenase, CK Creatine kinase, CRP C-reactive protein, ESR Erythrocyte sedimentation rate, CTDs Connective tissue diseases, FVC forced vital capacity, FEV1 Forced expiratory volume in 1 s, Dlco, diffusing capacity of the lungs for carbon monoxide, TLC Total lung capacity, % pred Percent predicted values, RP-ILD Rapidly progressive interstitial lung disease, C-ILD Chronic interstitial lung disease
aData available for 17 patients
bData available for 62 patients
cData available for 14 patients
dData available for 61 patients