Interstitial Lung Disease in Dermatomyositis and polymyositis-analysis of 23 Cases

Abstract

Objective: To characterize the clinical, laboratory, immunological, image, and pathological features inpatients of dermatomyositis (DM)/polymyositis (PM) with interstitial lung disease (ILD). Methods: We searched the data base of National Taiwan University Hospital between 1989 and 2006 for DM/PM patients with ILD. The medical records were reviewed. Results: Twenty-three DM/PM patients fulfilling Bohan and Peter's criteria with image-proved ILD were enrolled. Seven, 16 and no patients were classified into the rapid progression, slow progression and asymptomatic group, respectively. There were significantly more DM-ILD patients compared to PM-ILD patients in the rapid progression group than in the slow progression group (4/3 vs. 2/14, p=0.045). In the rapid progression group, 6 patients (85.71%) died and only one patient, receiving aggressive combination of immunosuppressive agents, survived at the end of study with improved pulmonary function test results. The survival of the rapid progression group was significantly lower than the slow progression group (mean survival time: 6±4 months vs. 69 ± 8 months, p=0.001; frequency of death due to ILD progression: 85.71% vs. 25%, p=0.019). Conclusion: There are significantly more DM-ILD patients with rapid progression of pulmonary symptoms than PM-ILD patients. DM/PM patients with rapid progressive ILD are with poorer prognosis, despite with intensive immunosuppressive therapy.