Clinical Features of Dermatomyositis/Polymyositis(DM/PM) with lung Involvement

park, Gun Min; Choi, Chang Min; Um, Sang Won; Hwang, Yong Il; Yim, Jae Joon; Lee, Jae Ho; Yoo, Chul Gyu; Lee, Ghoon Taek; Chung, Hee Soon; Song, Young Wook; Kim, Young Whan; Han, Sung Koo; Shim, Young Soo

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.