Clinical Characteristics | Values |
---|---|
Age at onset, yrs, mean (SD) | 44.9 (14.9) |
Sex | |
Male | 16 (29.6) |
Female | 38 (70.4) |
Race | |
Black | 26 (48.2) |
White | 22 (40.7) |
Hispanic | 4 (7.4) |
Asian | 2 (3.7) |
Smoking history | |
Never | 38 (70.4) |
Former | 12 (22.2) |
Active | 4 (7.4) |
Myositis | |
Polymyositis | 30 (55.6) |
Dermatomyositis | 20 (37.0) |
CADM | 4 (7.4) |
Baseline CK, U/l, mean (SD) | 2933.4 (4724.8) |
Baseline aldolase, mean (SD) | 31.6 (32.2) |
Serologic data | |
ANA | 32 (59.3) |
Anti-Jo1 | 27 (50.0) |
SSA | 22 (40.7) |
Mi-2 | 3 (5.6) |
PL-7 | 1 (1.9) |
SRP | 2 (3.7) |
Ku | 1 (1.9) |
PFT, %, mean (SD) | |
FVC | 61.7 (19.6) |
TLC | 68.6 (17.1) |
DLCO | 57.9 (24.1) |
CT chest | |
Honeycombing absent | 38 (70.4) |
Honeycombing present | 16 (29.6) |
Lung histopathology | |
OP | 5 (35.7) |
NSIP | 3 (21.4) |
UIP | 3 (21.4) |
Others* | 3 (21.4) |
Conventional DMARD | |
AZA | 35 (64.8) |
MTX | 17 (31.5) |
MMF | 11 (20.4) |
↵* COP with NSIP, COP with hypersensitivity pneumonitis, and COP with diffuse alveolar damage. MA-ILD: myositis-associated interstitial lung disease; CADM: clinically amyopathic dermatomyositis; CK: creatine kinase; ANA: antinuclear antibody; SRP: signal recognition particle; PFT: pulmonary function test; FVC: forced vital capacity; TLC: total lung capacity; CT: computed tomography; OP: organizing pneumonia; COP: cryptogenic OP; NSIP: nonspecific interstitial pneumonia; UIP: usual inter-stitial pneumonia; DMARD: disease-modifying antirheumatic drugs; AZA: azathioprine; MTX: methotrexate; MMF: mycophenolate mofetil.