Comparison of clinical characteristics between mortality group and survival group.
| Characteristics | Mortality Group, n = 14 | Survival Group, n = 4 | p | p-adjusted |
|---|---|---|---|---|
| Age, yrs, median (IQR) | 62.0 (42.0–66.5) | 66.0 (64.0–72.5) | 0.101 | 0.612 |
| Sex, male/female | 4/10 | 1/3 | 1.000 | 1.000 |
| Course of disease, mos, median (IQR) | 4.0 (2.0–16.8) | 1.0 (1.0–4.0) | 0.101 | 0.612 |
| Duration of diagnosis delay, mos, median (IQR) | 2.5 (2.0–3.8) | 1.0 (1.0–4.0) | 0.192 | 0.864 |
| On-admission disease activity, median (IQR) | 13.0 (9.8–16.0) | 9.0 (6.8–10.5) | 0.035 | 0.612 |
| Clinical features | ||||
| Fever | 14 (100.0) | 4 (100.0) | NA | NA |
| Lymphadenectasis | 6 (42.9) | 3 (75.0) | 0.576 | 1.000 |
| Splenomegaly | 10 (71.4) | 3 (75.0) | 1.000 | 1.000 |
| Hepatomegaly | 2 (14.3) | 0 (0.0) | 1.000 | 1.000 |
| Hyperferritinemia (ferritin ≥ 500 ng/ml) | 14 (100.0) | 4 (100.0) | NA | NA |
| Neutropenia (neutrophil count < 1.0 × 109/l) | 4 (28.6) | 1 (25.0) | 1.000 | 1.000 |
| Anemia (hemoglobin < 90 g/l) | 14 (100.0) | 3 (75.0) | 0.222 | 0.888 |
| Thrombocytopenia (platelet count < 100 × 109/l) | 14 (100.0) | 4 (100.0) | NA | NA |
| Hypofibrinogenemia (fibrinogen ≤ 1.5 g/l) | 10 (71.4) | 2 (50.0) | 0.569 | 1.000 |
| Hypertriglyceridemia (triglyceride ≥ 3.0 mmol/l) | 7 (50.0) | 3 (75.0) | 0.588 | 1.000 |
| Bone marrow involvement (hemophagocytosis in bone marrow) | 8 (57.1) | 1 (25.0) | 0.576 | 1.000 |
| ILD | 14 (100.0) | 4 (100.0) | NA | NA |
| AE-ILD | 10 (71.4) | 3 (75.0) | 1.000 | 1.000 |
| GI hemorrhage | 7 (50.0) | 0 (0.0) | 0.119 | 0.612 |
| Cardiac involvement | 3 (21.4) | 0 (0.0) | 1.000 | 1.000 |
| Carcinoma | 1 (7.1) | 1 (25.0) | 0.405 | 1.000 |
| Infection | 13 (92.9) | 2 (50.0) | 0.108 | 0.612 |
| Bacterial infection | 9 (64.3) | 0 (0.0) | 0.082 | 0.612 |
| Fungal infection | 5 (35.7) | 2 (50.0) | 1.000 | 1.000 |
| Tuberculosis infection | 1 (7.1) | 0 (0.0) | 1.000 | 1.000 |
| Viral infection | 1 (7.1) | 0 (0.0) | 1.000 | 1.000 |
| Medications | ||||
| Steroids | 14 (100.0) | 4 (100.0) | NA | NA |
| MMF | 1 (7.1) | 0 (0.0) | 1.000 | 1.000 |
| Thalidomide | 2 (14.3) | 0 (0.0) | 1.000 | 1.000 |
| Hydroxychloroquine | 2 (14.3) | 1 (25.0) | 1.000 | 1.000 |
| Cyclosporine | 1 (7.1) | 0 (0.0) | 1.000 | 1.000 |
| Azathioprine | 0 (0.0) | 0 (0.0) | NA | NA |
| Methotrexate | 1 (7.1) | 1 (25.0) | 0.405 | 1.000 |
| IG | 6 (42.9) | 1 (25.0) | 1.000 | 1.000 |
| Cyclophosphamide | 0 (0.0) | 0 (0.0) | NA | NA |
| Steroid monotherapy | 5 (35.7) | 1 (25.0) | 1.000 | 1.000 |
| Steroid+DMARD | 3 (21.4) | 2 (50.0) | 0.533 | 1.000 |
| Steroid+IG | 4 (28.6) | 1 (25.0) | 1.000 | 1.000 |
| Steroid+DMARD+IG | 2 (14.3) | 0 (0.0) | 1.000 | 1.000 |
| DST-based antibiotics | 6/9 (66.7) | 0/0 | NA | NA |
| Third-line antibiotics | 6/9 (66.7) | 0/0 | NA | NA |
| IV antifungal drugs | 5/5 (100) | 2/2 (100) | NA | NA |
| Prophylactic SMZ | 1 (7.1) | 1 (25.0) | 0.405 | 1.000 |
| IIM subtypes | ||||
| DM | 9 (64.3) | 2 (50.0) | 1.000 | 1.000 |
| PM | 5 (35.7) | 0 (0.0) | 0.278 | 1.000 |
| CADM | 0 (0.0) | 2 (50.0) | 0.039 | 0.612 |
Data are n (%) unless otherwise indicated. P-adjusted: adjusted p value after false discovery rate correction; IQR: interquartile range; ILD: interstitial lung disease; NA: not applicable; AE-ILD: acute exacerbation of interstitial lung disease; GI: gastrointestinal; MMF: mycophenolate mofetil; IG: immunoglobulin; DMARD: disease-modifying antirheumatic drugs; DST-based antibiotics: antibiotics based on drug-resistant testing; IV: intravenous; prophylactic SMZ: prophylactic application of sulfamethoxazole; IIM: idiopathic inflammatory myopathy; DM: dermatomyositis; PM: polymyositis; CADM: clinically amyopathic dermatomyositis.