The Clinical Features Of Dermatomyositis In Association With Interstitial Lung Disease

OBJECTIVEInterstitial lung disease (ILD) is common in patients with Dermatomyositis (DM), and these patients are difficult to be treated with a poor prognosis and high mortality rate. This study is to determine the clinical features and lung findings of 27 patients with Dermatomyositis associated with Interstitial Lung Disease.METHODS128 cases with DM collected from our hospital from 1997 to 2004 were evaluated retrospectively. The clinical manifestations and laboratory findings of 27 DM patients with ILD were analyzed and compared with those of 101 DM patients without ELD.RESULTSAs compared with the patients without ILD, the patients with ILD had shorter average courses of illness (4.1months vs. 11.2months, P<0.01), more common to have fever (43.8% vs. 20.8%, P<0.01), crackles in the lower zone of lung (40.6% vs. 7.3%, P<0.01), arthritis (59.4% vs. 26.0%, P<0.01), combine to use immuno-suppressive drugs (56.3% vs. 35.4%, P<0.01) during the course of the illness;meanwhile, these cases were less likely to have dysphagia (12.5% vs. 37.5%, P<0.01), and have lower average serum albumin and albumin/globulin (3.06g/dl vs. 3.66g/dl, 1.13 vs. 1.34, P<0.01). The predominant findings on the Chest X-ray and high-resolution computed tomography (HRCT) of the lungs in 27 DM patients with ILD were reticular and/or linear opacities, increased and coarsened lung markings, ground-glass opacity, speckle and/or patch opacities.There were no significant differences in other aspects (such as heliotrope rash, Gottronvs sign, myalgia, myasthenia, positive antinuclear antibodies and myogenic change in EMG and so on) between the two groups.Among 27 patients with DM-ILD, 12(45.6%) patients died, 8 (13.2%) patients improves, 7(11.3%) patients withdrew corticosteroid after remission. Among 101 patients without ILD, 30(42.6%) cases died. There was a significant difference in the death rate (P<0.01) between the tow groups.CONCLUSIONSThe present data demonstrates that interstitial lung disease might occur before, simultaneous or after the involvement of skin and/or muscle, and be frequently happened in the early illness courses in patients with PM/DM. The illness courses of these cases with ILD are shorter. Arthritis, fever, crackles in the lower zone of lungsare significantly increased while dysphagia, combined tumors, average serum albumin, albumin/globulin are significantly decreased. These cases are difficult to be treated with a high mortality rate, and the pulmonary infection is the main reason. The findings on HRCT of the lungs had early diagnostic value in the patients with ELD.