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205例多发性肌炎和皮肌炎临床分析

唐福林 唐宝玮 王丹彤

摘 要:对205例PM/DM的临床分析表明:PM/DM除以肌无力为首发症状外,尚可以发热、呼吸困难、吞咽困难、雷诺征、心肌炎、关节炎及肿瘤就诊。PM/DM内脏受损部位有:心脏36.5%(54/148);肺部26.3%(54/205)(其中肺间质病变9.0%,肺纤维化5.0%);消化系27.8%(57/205);血液系15.5%(32/205);神经系9.7%(20/205),几乎无肾损害。在诊断PM/DM平均11个月后有9例合并肿瘤;13例重叠综合征:5例干燥综合征,4例类风湿关节炎,1例系统性红斑狼疮,3例系统性硬化。48例患者测定抗Jo-1抗体,6例阳性,其中4例伴肺间质病变。CK可视为病情活动的指标。
关键词:多发性肌炎 皮肌炎 抗Jo-1抗体

Clinical analysis of 205 polymyositis and dermatomyositis cases

Tang Fulin
(Rheumatology Department, Peking Union Medical College Hospital,Beijing 100730)
Tang Baowei
(Rheumatology Department, Peking Union Medical College Hospital,Beijing 100730)
Wang Dantong
(Rheumatology Department, Peking Union Medical College Hospital,Beijing 100730)

Abstract:The clinical manifestations of 205 PM/DM patients were analyzed. Besides the most common presenting complaint, muscle weakness other complaints included fever, dyspnea, dysphagia, Raynaud s phenomenon, myocarditis, arthritis and even malignant disease. Organs involved in PM/DM were heart 36.5%(54/148); lungs 26.3%(54/205)[interstitial pulmonary diseases 9.0%,lung fibrosis 5.0%]; gastrointestinal tract 27.8%(57/205); hemotologic system 15.5% (32/205);neurologic system 9.7%(20/205). Almost no renal involvement in this group was noticed. Totally, 9 cases of malignant disease were found. The mean interval between the diagnoses of PM/DM and malignant tumor was 11 months. Overlap syndromes were 13 cases: 5 Sjogren s syndrome; 4 rheumatoid arthritis; 1 systemic lupus erythematosis; 3 systemic sclerosis. Among 48 cases, anti-Jo-1 antibody were detected in 6 cases,4 of them were suffering of interstitial pulmonary disease. Serum level of creatine kinase can be recognized as an index of this disease activity.
Key words:Polymyositis  Dermatomyositis  Anti-Jo-1 antibody▲

  多发性肌炎(polymyositis, PM)是一组原因不明的以横纹肌为主要受损的全身性自身免疫性疾病,伴皮肤损害的称皮肌炎(dermatomyositis, DM)。随着临床免疫学的进展,对本病的认识日趋全面。但由于本病

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