| Clinical Features of Dermatomyositis/Polymyositis(DM/PM) with lung Involvement. |
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Gun Min park, Chang Min Choi, Sang Won Um, Yong Il Hwang, Jae Joon Yim, Jae Ho Lee, Chul Gyu Yoo, Ghoon Taek Lee, Hee Soon Chung, Young Wook Song, Young Whan Kim, Sung Koo Han, Young Soo Shim |
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| Abstract |
BACKGROUND
Although lung involvement has been reported in 5 to 46% of dermatomyositis/polymyositis (DM/PM) patients, reports of the condition in Korea are rare. This study evaluated the clinical features of lung involvement in DM/PM patients. METHODS: The medical records, laboratory results and radiologic findings of 79 DM/PM patients, who attended the Seoul National University Hospital (SNUH) between 1989 and 1999, were reviewed retrospectively. RESULTS: A total 79 patients of whom 24 patients(33%) showed lung involvement, were enrolled in this study. More patients with lung involvement were female(F:M=11:1), and older compared with those without lung involvement. Patients with lung involvement presented with dyspnea(79%), coughing(67%), an elevated ESR, and CK/LD. Anti-Jo 1 antibody test was positive in 30%, which is significantly higher in patients with lung involvement. A simple chest X-ray of the patients with lung involvement exhibited reticular opacity(50%), reticulonodular opacity(30%), patchy opacity(29%), nodular opacity(13%) and linear opacity(4%). HRCT(n=24) showed ground glass opacity(75%), linear or septal thickening(50%), patchy consolidation(42%), honey-combing(33%) and nodular opacity(17%). The pulmonary function test showed a restrictive ventilatory pattern(77%) and a lower diffusing capacity(62%). The patients were followed up during a mean duration of 30±28 months. They were treated with steroid only(50%) or a combination of steroids and cytotoxic agents(46%). Muscle symptoms were improved in 89% with treatment, but an improvement in the respiratory symptoms or in the pulmonary function test was rare. Patients with lung involvement had a higher mortality rate(21%) than those without lung involvement(10%) during the follow-up periods. CONCLUSION: DM/PM patients with lung involvement were mostly female, older and had a higher positive rate Anti-Jo 1 antibodies, but there was no significant difference in prognosis. |
| Key Words:
Dermatomyositis, Polymyositis, Interstitial lung disease, Lung involvement, Anti-Jo 1 antibody |
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