Table 1.

Clinical characteristics of patients with MA-ILD (n = 54). Values are n (%) unless otherwise specified.

Clinical CharacteristicsValues
Age at onset, yrs, mean (SD)44.9 (14.9)
Sex
  Male16 (29.6)
  Female38 (70.4)
Race
  Black26 (48.2)
  White22 (40.7)
  Hispanic4 (7.4)
  Asian2 (3.7)
Smoking history
  Never38 (70.4)
  Former12 (22.2)
  Active4 (7.4)
Myositis
  Polymyositis30 (55.6)
  Dermatomyositis20 (37.0)
  CADM4 (7.4)
Baseline CK, U/l, mean (SD)2933.4 (4724.8)
Baseline aldolase, mean (SD)31.6 (32.2)
Serologic data
  ANA32 (59.3)
  Anti-Jo127 (50.0)
  SSA22 (40.7)
  Mi-23 (5.6)
  PL-71 (1.9)
  SRP2 (3.7)
  Ku1 (1.9)
PFT, %, mean (SD)
  FVC61.7 (19.6)
  TLC68.6 (17.1)
  DLCO57.9 (24.1)
CT chest
  Honeycombing absent38 (70.4)
  Honeycombing present16 (29.6)
Lung histopathology
  OP5 (35.7)
  NSIP3 (21.4)
  UIP3 (21.4)
  Others*3 (21.4)
Conventional DMARD
  AZA35 (64.8)
  MTX17 (31.5)
  MMF11 (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.