Clinical Features of Dermatomyositis/Polymyositis(DM/PM) with lung Involvement.
10.4046/trd.2001.51.4.354
- Author:
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
- Publication Type:Original Article
- Keywords:
Dermatomyositis;
Polymyositis;
Interstitial lung disease;
Lung involvement;
Anti-Jo 1 antibody
- MeSH:
Antibodies;
Dermatomyositis;
Female;
Follow-Up Studies;
Glass;
Humans;
Korea;
Lung Diseases, Interstitial;
Lung*;
Medical Records;
Mortality;
Polymyositis;
Prognosis;
Respiratory Function Tests;
Retrospective Studies;
Seoul;
Steroids;
Thorax
- From:Tuberculosis and Respiratory Diseases
2001;51(4):354-363
- CountryRepublic of Korea
- Language:Korean
-
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.
