目的:研究中国人多发性肌炎/皮肌炎(PM/DM)合并肾脏损害的临床特点.方法:报道1例皮肌炎合并肾脏损害病例的临床特点,并检索中国人PM/DM合并肾脏损害的文献报道进行系统分析.结果:共纳入28例病例.其中男10例,女18例,男:女比为1:1.8.PM/DM合并肾脏病变时,急性肾损伤和慢性肾脏病变均存在.肾脏损害病理类型:局灶节段性肾小球硬化(40％)、系膜增生性肾小球肾炎(20％)、轻微肾小球病变(20％)、肾小管间质性肾炎(10％)、IgA肾病(10％).28例PM/DM患者中,25例(89.3％)治疗后好转,尿蛋白转阴,肾功能正常;1例(3.6％)死亡;1例(3.6％)发展为终末期肾病,1例(3.6％)预后不详.结论:多发性肌炎/皮肌炎合并肾脏病变的临床表现,主要包括急性肾损伤和慢性肾脏病变.早期诊断与早期治疗是改善患者预后的关键.%Objective:To study the clinical characteristics of the polymyositis/dermatomyositis(PM/DM)patients with renal involvement in China.Methods:Here we reported a case of dermatomyositis complicated with renal involvement,and to retrieve all PM/DM with renal involvement reported in the literature from Chinese database.Results:Twenty-eight cases were enrolled in our study.Among these patients there were 10 males and 18 females,with a male to female ratio of 1:1.8.PM/DM combined with renal disease has both acute kidney injury and chronic kidney disease.Renal pathological types included:focal segmental glomerulosclerosis (40％),mesangial proliferative glomerulonephritis (20％),minor glomerular lesions (20％),tubulointerstitial nephritis (10％),IgA nephropathy (10％).Among the 28 PM/DM patients,25 cases (89.3％)improved after treatment,urine protein negative and normal renal function;1 case (3.6％)died;1 case (3.6％)developed end-stage renal disease,1 case (3.6％)of prognosis is unknown.Conclusion:The clinical manifestations of polymyositis/dermatomyositis complicated with renal lesions include acute kidney injury and chronic kidney disease.Early diagnosis and treatment is the key to improve the prognosis of patients.
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