第21卷 　 第二期 　 Vol. 21, No. 2, December, 2007

Original Aiticles 原著

Interstitial lung disease in dermatomyositis and polymyositis – analysis of 23 cases

Kuo-Yi Weng1, Ping-Ning Hsu2,3, Song-Chou Hsieh2, Chia-Li Yu2,4

1Ten Chan Hospital, Chung Li, Taiwan 2Devision of Allergy, Immunology and Rheumatology, Department of Internal Medicine, National Taiwan University Hospital, Taipei, Taiwan 3Graduate Institute of Immunology, and 4Graduate Institute of Molecular Medicine, College of Medicine, National Taiwan University, Taipei, Taiwan

Objective: To characterize the clinical, laboratory, immunological, image, and pathological features in patients 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.

keyword Polymyositis, dermatomyositis, interstitial lung disease