多发性肌炎／皮肌炎合并急性或亚急性间质性肺炎的临床分析

目的：分析总结多发性肌炎/皮肌炎（PM/DM）合并急性或亚急性间质性肺炎的临床特点.方法：回顾性分析15例PM/DM合并急性或亚急性间质性肺炎患者的临床资料.结果：15例患者中有7例肺部症状出现在肺外症状之前,5例肺部症状出现与肺外症状的首发时间几乎一致,3例肺部症状出现在肺外症状之后,但间隔不超过1个月.有肌酶升高者12例,其中CK升高者4例,AST升高者8例,LDH升高者10例.15例患者在病程早期X线和/或HRCT检查均发现不同程度的肺纤维化,后期纤维化程度迅速进展、恶化,呈弥漫性改变.11例患者中有5例行纤支镜肺活检术,病理结果均为弥漫性肺泡损害（DAD）.所有患者均出现严重感染,ARDS,其中7例经积极抢救无效死亡,8例治疗过程中家属放弃治疗.结论：多发性肌炎/皮肌炎合并急性或亚急性间质性肺炎患者间质性肺病症状出现早,进展快,死亡率极高,在发病早期及时识别诊断对于疾病的控制至关重要.

Clinical analysis of polymyositis/dermatomyositis with acute or subacute interstitial pneumonia

Objective： To analyze and summarize the clinical features of polymyositis/dermatomyositis （ PM/ DM） with acute or subacute interstitial pneumonia. Methods： Clinical data of 15 patients with PM/DM complicated by acute or subacute interstitial pneumonia were analyzed retrospectively. Results： Among the 15 patients,7 had pulmonary symptoms before other symptoms ,5 had pulmonary symptoms concurrent with other symptoms,and 3 had pulmonary symptoms within 1 month after experiencing other symptoms. 12 patients were found with elevated creatases （4 of CK,8 of AST and 10 of LDH）. X-ray and/or HRCT of all the 15 patients showed various degrees of pulmonary fibrosis in early stage, and the fibrosis rapidly progressed and aggravated,with diffuse damage. 5 patients underwent lung biopsy via fiberoptic bronchoscope, results of which were all diffuse alveolar damage （DAD）. All patients suffered from severe infection and ARDS ,7 patients died after active rescue measures and family members of 8 patients gave up their treatment. Conclusions： Polymyositis/dermatomyositis complicated by acute or subacute interstitial pneumonia have early respiratory symptoms, and progresses rapidly with a high mortality, so that early diagnosis is extremely important for disease control.