| Interstitial lung disease associated with polymyositis:response to cyclophosphamide and prednisolone combination treatment. |
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Jong Ho Moon1, Jun Young Park1, Sang Moo Lee1, Hyeon Tae Kim1, Soo Taek Uh1, Yeon Tae Chung1, Yong Hoon Kim1, Choon Sik Park1, Kyung Soo Lee2, Dae Yaung Kan3 |
1Department of lntemal Medicine, College of Medicine, Soonchunhyang University, Seoul, Korea
2Department of Radiology, College of Medicine, Soonchunhyang University, Seoul, Korea
3Department of Pathology, College of Medicine, Chungnam National University, Chungnam, Korea |
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| Abstract |
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Polymyositis (PM) is an inflammatory connective tissue disease involving predominantly skeletal muscles, characterized by symmetrical, proximal muscle weakness, inflammation, and frequently, degeneration. Interstitial lung disease in association with PM occurs in 5-10% of cases and carries an especially grave prognosis. Although the cause of l> mg involvement in PM is not known, the underlying pathologic process in the lung is an immune-mediated inflammation of alveolar structures, alveolitis. It is of interest, therefore, that cyclophosphamide, an immune-modulating agent, has been reported to be effective in the treatment of PM We report a case of corticosteroid-resistant PM associated with interstitial lung disease, success. fully treated with cyclophosphamide. A 37-year-old female was presented with 8 months duration of cough, exertional dyspnea, and muscle weakness. She had typical symptoms, physical findings, and elevated muscle enzyme levels in serum with characteristic findings of muscle biopsy. She also had a typical interstitial lung disease pattern on chest X-ray and high-resolution CT with restrictive pattern on pulmonary function test. The findings of transbronchial lung biopsy was compatible with interstitial lung disease. She failed to respond to corticosteroid initially. Subsequently steroids and cyclophosphamide were given with excellent clinical improvement. |
| Key Words:
Interstitial lung disease, Polymyositis, Corticosteroid, Cyclophosphamide |
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