Table 1. Clinical characteristics of the patients with DM-IP and DM without IP.
Characteristics | IP (n = 21) | Without IP (n = 5) |
---|---|---|
Age, years | 61 (36–78) | 76 (39–77) |
Female, n (%) | 15 (71) | 2 (40) |
CADM, n (%) | 14 (67) | 3 (60) |
RPIP, n (%) | 14 (67) | |
Disease duration, months | 2.5 (0.5–32) | |
Positive anti-MDA5-Ab, n (%) | 8 (38) | |
Positive anti-ARS-Ab, n (%) | 9 (43) | |
CK, IU/l | 168 (17–6049) | 489 (62–1851) |
LD, IU/l | 330 (180–894) | 251 (196–541) |
CRP, mg/dl | 0.95 (0.04–13.45) | 0.2 (0.05–5.86) |
KL–6, U/ml | 1084 (423–3898) | 187 (137–219) |
Ferritin, ng/ml | 419.9 (28–23272.5) | 287 (87.9–508.2) |
AaDO2, mmHg | 41.9 (5.9–113.6) | |
%VC | 75.5 (48.1–93.1) | |
%Dlco, ml/min/mmHg | 32.7 (8.6–48.6) | |
Total GGO score | 15 (10–23) | |
PSL (n = 21), mg/day | 50 (32.5–80) | 50 (45–50) d |
CSA (n = 13), mg/day | 250 (175–375) a | |
TAC (n = 7), mg/day | 3 (2–6) b | |
Total IVCY (n = 14), mg | 1700 (300–4900) c | |
Fatal outcome due to IP, n (%) | 4 (19) |
Values indicate the median (interquartile range). DM: dermatomyositis; IP: interstitial pneumonia; CADM: clinically amyopathic DM; RPIP: rapid progressive IP; Disease duration: disease duration from onset of respiratory symptoms of IP to initiation of treatments; MDA5: anti-melanoma differentiation-associated gene 5; Ab: antibody; ARS: aminoacyl-tRNA synthetase; CK: creatine kinase; LD: lactate dehydrogenase; CRP: C-reactive protein; AaDO2: alveolar-arterial oxygen difference; VC: vital capacity; Dlco: diffusion capacity of the lung for carbon monoxide; GGO: ground-glass opacity; PSL: prednisolone; CSA: cyclosporine; TAC: tacrolimus; IVCY: intravenous pulse cyclophosphamide.
an = 13.
bn = 7.
cn = 14.
dn = 3.