系统性红斑狼疮足细胞病七例临床特征分析

Clinical characteristics and outcomes of 7 patients with lupus podocytopathies

目的 回顾性分析狼疮足细胞病(LP)患者的临床及实验室特征、治疗反应及预后.方法 选取2011年1月至2019年5月无锡市人民医院住院期间行肾活检证实的LP病例7例,回顾性分析其临床及实验室特征、治疗反应及预后.结果 7例LP中6例为女性,平均发病年龄(33±12)岁,SLE平均病程(69±64)个月,其中3例为初发初诊,6例为初次诊断狼疮肾损害.6例患者表现为肾病综合征(NS);2例表现为急性肾功能不全(ARF).LP患者肾脏表现与SLE疾病活动度不平行,自身抗体阳性率、低补体发生率均较低.经激素、免疫抑制剂治疗后6例患者肾脏病变缓解;随访过程中,2例患者出现肾脏复发.结论 LP以生育期女性好发,NS或伴AKI是其主要临床表现,肾脏表现与肾外表现不平行,激素、免疫抑制剂治疗敏感,部分患者治疗后可出现复发.

Objective To investigate the clinical,treatment responses and outcomes of SLE patients with lupus podocytopathy(LP).Methods Seven hospitalized cases in Wuxi people's hospital were diagnosed with LP based on the renal biopsy study during January 2011 to May 2019.Their clinical,immunological and pathological features,treatment responses and prognosis were analyzed.Results Six cases were women.The mean onset age was(33±12),and the mean duration of systemic lupus erythematosus(SLE)was(69±64)months.Among these patients,3 cases were initially diagnosed of SLE,and 6 with kidney damage caused by SLE.Six cases presented as nephrotic syndrome(NS)in which 2 cases were complicated with acute kidney injury(AKI).The extrarenal manifestations were not parallel to the renal manifestations as the positive rate of autoantibody and the incidence of hypocomplementemia were both low.After treatments with glucocorticoids and immunosuppressants,renal disease of 6 cases was improved.During follow-up,2 cases developed renal disease recurrence.Conclusion LP tends to occur in women in reproductive age,and NS or AKI is the main clinical manifestations.Renal manifestations are not parallel to extra-renal manifestations.Glucocorticoids and immunosuppressive therapy is sensitive,and some patients may relapse after treatment.