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目的通过对以急性间质性肺炎为主要症状的多发性肌炎/皮肌炎(PM/DM)的临床特点的分析,提高对该病诊治的认识。方法结合相关文献对2009年10月至2015年6月在我院以急性间质性肺炎为主要症状的8例PM/DM患者的临床资料进行分析。结果 8例患者中,男2例,女6例,平均年龄为(54.8±9.2)岁。主要表现为发热(8例)、气促(8例)、进行性呼吸困难(8例)、干咳(6例)、肌力减退(8例),7例有典型皮疹。8例肌电图检查呈神经源性或肌源性损害,7例肌肉活检符合肌炎,胸部高分辨率CT(HRCT)表现为双肺弥漫磨玻璃样改变或实变影。全部患者入院第1 d即使用无创正压机械通气,并于入院后第(2.3±1.4)d予大剂量甲基泼尼松龙单用或联合静脉环磷酰胺治疗。6例患者临床症状好转,2例死亡。结论发热、气促、进行性呼吸困难、干咳、肌力减退为PM/DM合并急性间质性肺炎的主要症状,大部分有典型皮疹,结合HRCT、肌电图及肌肉活检特点可诊断本病,早期使用无创正压机械通气及大剂量免疫抑制剂为主的综合治疗可改善预后。
OBJECTIVE: To improve the understanding of the diagnosis and treatment of polymyositis / dermatomyositis (PM / DM) characterized by acute interstitial pneumonia. Methods The clinical data of 8 PM / DM patients with acute interstitial pneumonia as the main symptom in our hospital from October 2009 to June 2015 were analyzed in combination with related literatures. Results Among 8 patients, there were 2 males and 6 females with an average age of (54.8 ± 9.2) years. Mainly manifested as fever (8 cases), shortness of breath (8 cases), progressive dyspnea (8 cases), dry cough (6 cases), muscle weakness (8 cases), and 7 cases with typical rash. 8 cases of neuromuscular examination showed neurogenic or myogenic damage, 7 cases of muscle biopsy in line with myositis, chest high resolution CT (HRCT) performance of diffuse lung glass-like changes or consolidation. All patients were on non-invasive positive pressure mechanical ventilation on day 1 and were treated with high-dose methylprednisolone or intravenous cyclophosphamide 2.3 (1.4) d after admission. 6 patients improved clinical symptoms, 2 patients died. Conclusions Fever, shortness of breath, progressive dyspnea, dry cough and decreased muscle strength are the main symptoms of PM / DM complicated with acute interstitial pneumonia. Most of them have a typical rash. Combined with the features of HRCT, EMG and muscle biopsy, the disease can be diagnosed , Early use of non-invasive positive pressure mechanical ventilation and high-dose immunosuppressive agents based comprehensive treatment can improve the prognosis.