木村病合并肾脏损害的病例分析及文献复习  

Kimura disease with renal impairment:case series and literature review

作　　者：胡蓉蓉[1] 张磊[1] 马杰[1] 乐偲[1] 文煜冰[1] 叶葳[1] 叶文玲[1] 郑可[1] 秦岩[1] 陈丽萌[1] 李雪梅[1] Hu Rongrong;Zhang Lei;Ma Jie;Yue Cai;Wen Yubing;Ye Wei;Ye Wenling;Zheng Ke;Qin Yan;Chen Limeng;Li Xuemei(Department of Nephrology,Peking Union Medical College Hospital,Chinese Academy of Medical Sciences,Beijing 100730,China)

机构地区：[1]中国医学科学院北京协和医院肾内科,北京100730

出　　处：《中华肾脏病杂志》2022年第3期196-202,共7页Chinese Journal of Nephrology

基　　金：北京市科技计划(D181100000118001)。

摘　　要：目的分析木村病患者肾脏改变的临床与病理特点、治疗与预后, 提高临床医师对木村病肾脏表现的认识。方法回顾性分析1980年1月至2020年8月北京协和医院诊断明确且资料详细的木村病患者的临床资料, 并根据有无肾脏受累分为肾脏损害组与无肾脏损害组, 进行对照比较, 对表现为肾病综合征的患者进行随访。结果共经病理诊断明确木村病60例, 男性48例, 中位年龄33(3, 62)岁, 中位病程36(12, 111)个月。完善尿常规和肾功能检测的49例患者中, 合并肾脏损害18例, 主要表现为蛋白尿和/或镜下血尿。肾脏损害组(n=18)和无肾脏损害组(n=31)患者在起病年龄、性别比例、头面部以外脏器受累比例等方面差异均无统计学意义(均P>0.05)。与无肾脏损害组比较, 肾脏损害组患者病程较长, 超敏C反应蛋白与红细胞沉降率水平较高, 血嗜酸性粒细胞总数及总IgE中位值较低, 但差异均无统计学意义(均P>0.05)。在肾脏受累的患者中, 达到肾病综合征诊断标准6例, 其中5例患者完善了肾活检检查, 肾脏病理诊断结果为膜性肾病2例、微小病变型肾病3例, 肾活检组织中均未显示间质嗜酸性粒细胞浸润;此类患者对激素和(或)免疫抑制剂治疗反应良好, 均可达到肾病综合征缓解;治疗肾病的同时, 木村病导致的淋巴结肿大也得到良好的控制。结论木村病患者可合并多种肾脏病变表现, 表现为肾病综合征患者的病理可为膜性肾病或微小病变型肾病。经过积极的激素和免疫抑制剂治疗, 肾病综合征均可达到完全缓解, 淋巴结肿大控制良好。木村病和肾脏病变之间的关联机制尚需更深入的研究来确定。Objective To analyze the clinical and pathological characteristics,treatment and prognosis of renal changes in patients with Kimura disease and improve the clinicians′understanding on renal manifestations of Kimura disease.Methods The clinical data of Kimura disease patients with definite diagnosis and detailed data in Peking Union Medical College Hospital from January 1980 to August 2020 were retrospectively analyzed.The patients were divided into renal impairment group and non-renal impairment group according to whether the kidney was involved or not and the related clinical data between the two groups were compared.The patients presenting with nephrotic syndrome were followed up.Results There were 60 patients with Kimura disease confirmed by pathological diagnosis with 48 males.The median age was 33(3,62)years old,and the median duration was 36(12,111)months.There were 18 cases complicated with renal injury in 49 patients with complete routine urine and renal function examination and the main manifestations of renal injury were proteinuria and/or microscopic hematuria.There was no significant difference at age,sex and absolute value of eosinophils between the two groups(all P>0.05).Compared with the renal inpairment group,patients in non-renal inpairment group had longer course of disease,higher levels of hypersensitive C-reactive protein and erythrocyte sedimentation rate,and lower median values of total eosinophils and total IgE,but there was no statistically significant difference(all P>0.05).Among the patients with renal involvement,6 patients met the diagnostic criteria for nephrotic syndrome,and 5 of them completed renal biopsies.The renal pathological diagnosis was membranous nephropathy in 2 cases and minimal change disease in 3 cases,and no interstitial eosinophil infiltration was found in renal biopsy tissues.These patients had a good response to glucocorticoids and/or immunosuppressive therapy,and achieved complete remission of nephrotic syndrome;at the same time,lymphadenopathy caused by Kimura d

关 键 词：肾病综合征 嗜酸粒细胞增多 免疫球蛋白E 木村病 

分 类 号：R692[医药卫生—泌尿科学]