IgG4相关性疾病泌尿系统损害的临床特点分析  

Clinical feature of renal impairment caused by IgG4 related disease with renal and urinary lesions

作　　者：陈罡[1] 郑可[1] 叶文玲[1] 文煜冰[1] 蔡建芳[1] 袁群生[1] 李明喜[1] 张文[2] 李雪梅[1] 

机构地区：[1]中国医学科学院北京协和医学院北京协和医院肾内科,北京100730 [2]中国医学科学院北京协和医学院北京协和医院免疫内科,北京100730

出　　处：《中华肾脏病杂志》2015年第1期7-12,共6页Chinese Journal of Nephrology

基　　金：国家科技支撑计划（2011BA110802,2012BAJ18B03）;首都医学发展科研基金（2011-4001-08）

摘　　要：目的 总结和分析IgG4相关性疾病（IgG4-RD）泌尿系统损害的临床特点.方法 收集24例IgG4相关性疾病泌尿系统损害患者的临床资料,回顾性分析患者肾脏受累、治疗前后Scr、Hb、炎性反应指标和IgG4亚类水平的变化情况.结果 2010年8月至2014年5月经北京协和医学院肾内科确诊的IgG4相关性疾病泌尿系统损害病例24例,平均受累器官数目（4.38±1.55）个.治疗前血清IgG4[M（范围）]为12 750（1 460～ 59 400） mg/L,均伴炎性反应指标明显升高.20例患者有检测尿蛋白量,其中19例尿蛋白量升高,6例伴肾病综合征.21例患者Scr升高,均值为（410.48±352.17） μmol/L,其中3例需接受透析治疗.14例患者伴贫血,8例伴单侧或双侧肾脏体积缩小.21例Scr升高的患者中,11例患者肾功能不全因肾后性梗阻所致,3例病理证实为肾小管间质肾炎.与治疗前相比,24例患者糖皮质激素联合或不联合免疫抑制剂治疗1周后Scr明显下降（P＜0.05）,治疗1个月后Scr下降更为明显（P＜0.01）,3例透析患者均脱离透析.血沉于治疗1个月后亦明显下降（P＜0.01）.IgG4亚类水平在治疗1个月时无显著下降（P＞0.05）,治疗2个月时明显下降（P＜0.01）.结论 IgG4相关疾病合并肾功能不全并不少见,对中至大量糖皮质激素治疗反应良好,治疗后Scr下降早于血沉和IgG4亚类.Objective To analyze the clinical features of renal and urinary lesions in IgG4 related disease （IgG4-RD）.Methods Clinical data of 24 cases of lgG4-RD patients were retrospectively analyzed,including serum creatinine,hemoglobin,inflammatory factors,and serum IgG4 level before and after treatment.Results Twenty four cases of IgG4-RD with renal and urinary lesions were diagnosed in Peking Union Medical College Hospital from Aug 2010 to May 2014,including 21 males and 3 females.The average age of the patients was （63.79±12.13） years old,and the average number of organs involvement was （4.38±1.55）.Elevated inflammatory factors and serum IgG4 level were detected in all patients.Elevated urine protein was detected in 19 cases and 6 cases were diagnosed as nephritic syndrome among 20 patients.Increased Scr were detected in 21 cases with an average value of （410.48±352.17） μmol/L and 3 cases had been treated with hemodialysis.Anemia was found in 14 cases and 8 cases were detected with single or bilateral kidney atrophy.Among the 21 cases with elevated Scr,11 cases were caused by postrenal obstruction and 3 cases were pathologically proved as tubulointerstitial nephritis.Received steroid treatment,Scr level declined dramatically one week later （P 〈 0.05） and further declined one month later （P 〈 0.01）; erythrocyte sedimentation rate declined significantly one month later （P 〈 0.01） ; serum IgG4 level did not decline significantly after one month （P=0.146）,but declined significantly after two months （P 〈 0.01）.Conclusions Renal insufficiency is not rare in IgG4-RD with renal and urinary lesions,and it is sensitive to steroid therapy; the decline in Scr can be seen earlier than the change in inflammatory factors and serum IgG4 level.

关 键 词：肾功能不全 急性 泌尿系疾病 IgG4相关疾病 急性肾损伤 

分 类 号：R593.2[医药卫生—内科学]