Kimura 病伴肾病综合征2例临床分析并文献回顾

Kimura′s disease complicated with nephrotic syndrome: a clinical analysis of 2 cases and literature review

目的探讨Kimura病合并肾脏损害的临床特点,结合文献复习以期提高对该病的诊治水平。方法对2015年5月和2014年8月分别收治的2例合并肾脏损害的Kimura病患者的临床资料进行分析,检索Pubmed、中国知网数据库检索2000年以来成人Kimura病合并肾损害的病例报道,进行文献回顾。结果 2例均为男性,31岁(例1)和17岁(例2),均以“肾病综合征”初诊,例1合并淋巴结肿大,例2合并左腮腺区肿物。例1肾脏病理示肾小球微小病变,用肾上腺糖皮质激素(激素)治疗后病情控制,随访42个月无复发;例2肾脏病理示新月体性肾炎,用激素联合吗替麦考酚酯,病情改善,但药物减量中出现病情反复。检索结果,2000年至2018年11月,国内外已报道Kimura病合并肾脏损害病例共16例。结论 Kimura病合并肾脏损害罕见,多发于年轻男性,大部首发症状为皮下肿物,系膜增生性肾小球肾炎为最常见病理类型,大多患者应用激素治疗,病情可获缓解。Kimura病易复发,合并肾脏损害也可复发。

Objective To investigate the clinical characteristics of Kimura′s disease complicated with renal lesions,summarize the experiences of diagnosis and treatment and review the literature in order to improve the understanding of the diagnosis and treatment of Kimura′s disease.Methods The clinical data of 2 patients with Kimura′s disease complicated with kidney damage admitted in May 2015 and August 2014 were analyzed.The case reports of adult Kimura′s disease with kidney damage were retrieved from databases of Pubmed and CNKI since 2000,and the literature was reviewed.Results Two cases of male were 31 and 17 years old,respectively,and were early diagnosed as "nephrotic syndrome".There were enlarged lymph nodes in case 1 and mass in the left parotid gland in case 2.Case 1 presented with minor glomerular abnormalities,which were controlled after adrenocortical hormone(hormone) treatment,and no recurrence was found during 42 months follow-up.Case 2 showed crescentic glomerulonephritis,which was improved by prednisone combining with mycophenolate mofetil(MMF),but recurrence occurred in drug reduction.According to the search,from 2000 to November 2018,16 cases of Kimura′s disease with kidney damage have been reported at home and abroad.Conclusions Kimura′s disease with renal lesions is rare,mostly occurring in young men.Subcutaneous masses are the common first symptoms.Mesangial proliferative glomerulonephritis is the most common pathological type.Most patients can be relieved by hormone therapy.Kimura′s disease or complicated with kidney damage is easy to recur.