Factor | No. of Patients | HR | 95% CI | |
---|---|---|---|---|
Demographics | ||||
Sex | Malea | 52 | — | — |
Female | 117 | 0.71 | 0.26-1.94 | |
Age, yrs | < 15 | 0 | NA | NA |
15 to < 30 | 3 | NA | NA | |
30 to < 40 | 6 | NA | NA | |
40 to < 50 | 31 | 0.82 | 0.22-3.09 | |
50 to < 60 | 63 | 0.62 | 0.20-1.91 | |
≥ 65a | 66 | — | — | |
Weight, kg | < 30 | 0 | NA | NA |
30 to < 50a | 63 | — | — | |
50 to < 70 | 80 | 0.86 | 0.29-2.56 | |
≥ 70 | 21 | 1.48 | 0.37-5.92 | |
Inpatient/outpatient | Inpatient | 143 | 2.89 | 0.38-21.89 |
Outpatienta | 26 | — | — | |
Smoking | Noa | 130 | — | — |
Yes | 38 | 1.66 | 0.58-4.78 | |
Dysphagia | Absencea | 159 | — | — |
Presence | 10 | NA | NA | |
Underlying disease | ||||
Idiopathic inflammatory myopathies | PMa | 46 | — | — |
CADM | 61 | 9.04 | 1.18-69.51 | |
Classic DM | 62 | 2.09 | 0.22-20.05 | |
Disease duration, yrs | < 1a | 121 | — | — |
1 to < 5 | 25 | NA | NA | |
≥ 5 | 11 | 0.78 | 0.10-5.99 | |
Symptoms other than muscle, lung, and skin | Absencea | 64 | — | — |
Presence | 105 | 1.35 | 0.47–3.89 | |
Raynaud phenomenon | Absencea | 149 | — | — |
Presence | 20 | 0.99 | 0.22–4.34 | |
Arthritis or joint pain | Absencea | 100 | — | — |
Presence | 69 | 1.12 | 0.42–3.01 | |
Cardiopathy | Absencea | 161 | — | — |
Presence | 8 | 3.01 | 0.68–13.28 | |
Fever | Absencea | 134 | — | — |
Presence | 35 | 2.55 | 0.93–7.03 | |
IP | ||||
IP duration | < 1 montha | 26 | — | — |
1 to < 3 months | 40 | 0.42 | 0.12–1.48 | |
3 to < 6 months | 25 | NA | NA | |
6 to < 12 months | 20 | 0.39 | 0.08–1.92 | |
1 to < 3 yrs | 15 | NA | NA | |
≥ 3 yrs | 19 | 0.43 | 0.09–2.13 | |
Primary/relapse | Primarya | 134 | — | — |
Relapse | 35 | 0.86 | 0.24–3.00 | |
Radiographically speculated histological type | NSIPa | 130 | — | — |
UIP | 11 | NA | NA | |
OP | 17 | 0.51 | 0.07–3.88 | |
DAD | 5 | 4.58 | 1.03–20.38 | |
SaO2, % | < 85 | 1 | NA | NA |
85 to < 90 | 2 | NA | NA | |
90 to < 95 | 22 | 7.53 | 2.73–20.82 | |
≥ 95a | 120 | — | — | |
Oxygen administration | Absencea | 151 | — | — |
Presence | 18 | 3.82 | 1.23–11.88 | |
Ferritin, ng/mL | < 500a | 92 | — | — |
500 to < 1500 | 21 | 8.61 | 2.51–29.45 | |
1500 to < 2500 | 3 | NA | NA | |
2500 to < 3500 | 2 | NA | NA | |
≥ 3500 | 0 | NA | NA | |
Anti-Jo-1 antibody | Positive | 27 | 0.26 | 0.03–2.01 |
Negativea | 112 | — | — | |
Anti-ARS antibody | Positive | 74 | 0.12 | 0.03–0.53 |
Negativea | 58 | — | — | |
Anti-MDA5 antibody | Positive | 28 | 8.16 | 1.03–64.47 |
Negativea | 21 | — | — | |
KL-6, U/mL | < 1000a | 83 | — | — |
≥ 1000 | 71 | 0.62 | 0.21–1.84 | |
SP-D, ng/mL | < 100a | 30 | — | — |
≥ 100 | 50 | 0.59 | 0.17–2.05 | |
Mode of onset | Subacutea | 73 | — | — |
Acute exacerbation | 22 | 1.00 | 0.28–3.65 | |
Chronic | 46 | 0.48 | 0.13–1.74 | |
Asymptomatic | 28 | NA | NA |
↵aReference. For categories with no events (death), HR and 95% CI are shown as NA. Anti-ARS: antiaminoacyl tRNA synthetase; anti-MDA5: antimelanoma differentiation-associated gene 5; CADM: clinically amyopathic dermatomyositis; DAD: diffuse alveolar damage; DM: dermatomyositis; HR: hazard ratio; IP: interstitial pneumonia; KL-6: Krebs von den Lungen 6; NA: not available; NSIP: nonspecific interstitial pneumonia; OP: organizing pneumonia; PM: polymyositis; SaO2: arterial oxygen saturation; SP-D: surfactant protein D; UIP: usual interstitial pneumonia.