Interstitial lung disease in patients with polymyositis, dermatomyositis and amyopathic dermatomyositis.
OBJECTIVE
To assess the prevalence, characteristics and prognostic factors of interstitial lung disease (ILD) in Korean patients with polymyositis (PM), dermatomyositis (DM) and amyopathic dermatomyositis (ADM).


METHODS
We reviewed the medical records of 72 consecutive PM and DM patients, including six patients with ADM, who were seen at the Rheumatology Clinic of Seoul National University Hospital between 1984 and 2003.


RESULTS
Twenty-nine PM/DM patients (40.3%) developed ILD. Anti-Jo-1 antibody and arthralgia were associated with the presence of ILD (P = 0.022 and P = 0.041, respectively), whereas dysphagia was more frequently found in patients without ILD (P = 0.041). Lung biopsies revealed diffuse alveolar damage (DAD) (n = 2), usual interstitial pneumonia (UIP) with DAD (n = 2), UIP (n = 1), and non-specific interstitial pneumonia (n = 2). Of the 29 patients, 11 (37.9%) died. The mean survival time in ILD patients was significantly shorter than in those without ILD (13.8+/-1.8 vs 19.2+/-0.9 yr, P = 0.017). Poor survival in ILD patients was associated with a Hamman-Rich-like presentation (P = 0.0000), ADM features (P = 0.0001) and an initial forced vital capacity (FVC) < or =60% (P = 0.024).


CONCLUSIONS
ILD was observed in 40.3% of Korean PM/DM patients and was associated with poor survival. A Hamman-Rich-like presentation, ADM features and an initial FVC < or =60% were associated with poor survival in ILD.
Department of Internal Medicine, Seoul National University Hospital, 28 Yongon-dong, Chongno-gu, Seoul 110-744, Korea.
E H
EH
Kang
E B
EB
Lee
K C
KC
Shin
C H
CH
Im
D H
DH
Chung
S K
SK
Han
Y W
YW
Song
D000328:Adult; D003882:Dermatomyositis / Q000150:complications; D018450:Disease Progression; D004812:Epidemiologic Methods; D005260:Female; D006801:Humans; D017563:Lung Diseases, Interstitial / Q000000981:diagnostic imaging / Q000209:etiology* / Q000503:physiopathology; D008297:Male; D008875:Middle Aged; D017285:Polymyositis / Q000150:complications*; D011379:Prognosis; D014057:Tomography, X-Ray Computed; D014797:Vital Capacity