Abstract: Objective To study the clinical features and significance of the patients with dermatomyositis(DM)associated with interstitial lung disease(ILD).Methods 216 cases of DM were collected from the Peking Union Medical College Hospital from 1970 to 1997.The criteria for the diagnosis of DM were based on the report of Bohan and Peter in 1975.The mean follow-up period of these patients was 5.8±4.7 years(ranged from 2 to 27 years).The clinical manifestations and laboratory findings of 32 DM patients with ILD were analyzed and compared with those of 184 DM patients without ILD.Results As compared with DM patients without ILD,the DM patients with ILD were more likely to have fever(28.2%vs.6.5%,P<0.01)in the initial symptoms,during the course of the illness the DM patients with ILD were more common to have fever(71.9%vs34.2%,P<0.01),arthritis(62.5%vs34.2%,P<0.01),skin ulcer(31.3%.vs.8.7%,P<0.01)and positive rheumatoid factor(24.1%vs.8.1%,P<0.05).There were no significant differences in heliotrope rash,Gottron's sign,dysphagia,myalgia,myasthenia,positive antinuclear antibodies and myogenic change in EMG between the two groups.Among 32 DM patients with ILD,13(40.6%)patients died,15(46.9%)patients improved,4(12.5%)patients withdrew corticosteroid after remission.Among 184 DM patients without ILD,30(16.3%)cases died.There was a singnificant difference in the death rate(P<0.01)between the two groups.Conclusion Fever,arthritis,skin ulcer and positive rheumatoid factor were significantly increased in DM patients with ILD.These cases are difficult to be treated with a high death rate.

Key words: Dermatomyositis, Lung diseases,interstitial