【摘要】 目的 探讨新诊断的多发性肌炎（PM）、皮肌炎（DM）和无肌病性皮肌炎（ADM）肺部病变发生率、临床特点及相关因素。 方法 回顾性分析2008年1月—2010年7月新确诊的206例PM、DM、ADM临床表现、肺部影像学、肺功能、超声心动图和实验室指标。 结果 206例患者中合并肺部病变156例，以肺间质病变（ILD）最多见，占患者总数的51.46%。在性别、病程、是否吸烟方面合并ILD与无ILD患者相比差异无统计学意义，合并ILD患者年龄大于无ILD组。合并ILD患者呼吸困难、发热、雷诺现象、关节炎或关节痛概率增加。合并ILD组白蛋白低于无ILD组，血沉和免疫球蛋白IgM高于无ILD组。急性ILD组中女性患者及出现雷诺现象的概率高于慢性组。206例患者中死亡13例，其中周围型肺癌1例，特发性血小板减少并颅内出血1例，严重肺部感染11例；死亡患者中10例伴肺间质纤维化。合并急性ILD患者死亡率较慢性组高2倍。 结论 PM、DM、ADM患者肺部病变发生率高，以ILD多见，发热、年龄大、白蛋白降低、血沉升高、雷诺现象及关节炎或关节痛都是合并ILD的相关因素。合并急性ILD患者预后差，死亡者常合并肺部感染。【Abstract】 Objective To explore the prevalence, clinical features, and predictive factors of pulmonary involvement in newly diagnosed polymyositis (PM), dermatomyositis (DM) and amyopathic dermatomyositis (ADM), in order to carry out early diagnosis and treatment, and improve the prognosis. Methods The clinical manifestations, chest imaging, pulmonary function test, ultrasonic cardiography and laboratory results of 206 inpatients with PM, DM and ADM in West China Hospital of Sichuan University from January 2008 to July 2010 were reviewed retrospectively. Results One hundred and fifty-six out of 206 patients developed PM/DM/ADM associated lung diseases, including 106 cases (51.46%) of interstitial lung disease (ILD). There was no significant difference in gender, disease duration, and smoking or not between the ILD and non-ILD group, but patients in the ILD group were significantly older than non-ILD group. The results also showed that patients with ILD were much more likely to have symptoms of breathing difficulties, fever, Raynaud phenomenon and arthritis/arthralgia. The patients with ILD had lower level of albumin but higher levels of ESR and IgM; In the group of acute ILD, female patients and the ratio of Raynaud phenomenon were higher than those in the chronicity group. Of the 206 patients, 13 patients died, including 1 death of peripheral lung cancer, 1 of essential thrombocytopenia and intracranial hemorrhages, and 11 of severe lung infection, and 10 in these patients developed ILD. Mortality in patients with acute ILD was 2 times higher than the chronicity group. Conclusion The prevalence of lung diseases is high in patients with PM, DM and ADM. ILD is the main pulmonary involvement, and fever, older age at onset, hypoalbuminemia, higher values of blood sedimentation, Raynaud phenomenon and arthritis or arthralgia were the predictive factors for developing ILD. Patients with acute ILD have poor prognosis. Death cases often have pulmonary infections.
Melanoma differentiation associated gene 5 (MDA5) antibody is a kind of myositis-specific autoantibodies, which can be used as a biological marker for dermatomyositis (DM) patients. It has been shown that this antibody is closely related to the occurrence of interstilung disease (ILD) in DM patients, which may lead to rapidly progressive interstitial lung disease in DM patients with MDA5 antibody positive, leading to a higher mortality rate. Therefore, the early detection, diagnosis and treatment of this disease have attracted the attention of clinical workers. In this paper, the progress of diagnosis and treatment of MDA5 antibody-positive DM with ILD is reviewed.