IgG4相关肾病临床及病理特征分析

Analysis of clinical and histological features of IgG4-related kidney disease

目的:通过分析IgG4相关肾病(IgG4-related kidney disease,IgG4-RKD)的临床及病理特征,为临床医师早期认识疾病,合理诊断、治疗疾病提供参考。方法:回顾性分析2017年4月至2020年3月首次就诊于我科的9例Ig G4-RKD患者的一般情况、实验室检查、病理学检查结果及治疗随访情况。结果:9例患者的发病年龄50~71岁,其中男性7例,女性2例。首次就诊的原因为血肌酐升高(6例)、胰腺占位(2例)和泪腺肿大(1例)。仅累及肾脏者1例,累及2、3及4个脏器者(含肾脏)分别为6、2及2例。血清学特征为IgG4(8/8)、IgG(8/9)、血肌酐(8/9)、红细胞沉降率(erythrocyte sedimentation rate,ESR)(7/8)和IgE升高(5/9),补体C3(8/9)、补体C4降低(3/9),尿液异常表现为尿蛋白升高(8/9)、尿α微球蛋白升高(8/8)、低尿钙(5/9)。7例患者行肾穿刺,均有IgG4沉积,6例有席纹样改变。单用糖皮质激素治疗2例,糖皮质激素加环磷酰胺3例,糖皮质激素加利妥昔单抗3例,血浆置换加糖皮质激素1例,随访过程中血肌酐均出现不同程度的下降。结论:IgG4-RKD临床表现多样,常累及多系统和多器官,且可同时累及肾小球及肾小管间质,导致肾功能衰竭,故需要各科医师提高对该疾病的认识,早期甄别,早期诊断,早期治疗,避免进展至肾功能衰竭。

Objective:To analyze the clinical and pathological features of IgG4 related kidney disease(IgG4-RKD),and to provide reference for clinicians to recognize the disease in early stage,diagnose and treat the disease rationally.Methods The general information,laboratory examination index,pathological examination result,treatment,and follow-up in the patients with IgG4-RKD who firstly visited our department from April 2017 to March 2020 were collected and retro-spectively analyzed.Results The age of 9 patients with IgG4-RKD ranged from 50 to 71 years old,including 7 males and 2 females.The reasons of the first visit were elevated serum creatinine(6 cases)or pancreatic mass(2 cases)and lacrimal gland enlargement(1 case).One case only had kidney damage;6,2 and 2 cases were found 2,3 or 4 organ damages,re-spectively(including kidney).The serological characteristics were as follows:the indexes including IgG4(8/8),IgG(8/9),serum creatinine(8/9),erythrocyte sedimentation rate(ESR)(7/8),and IgE(5/9)all increased,while complement C3(8/9)and complement C4(3/9)decreased.Abnormal urine tests showed low urinary calcium(5/9),and increased urinary protein(8/9)andα-microglobulin(8/8).Seven patients underwent renal puncture,the results showed all cases had IgG4 deposi-tion,and 6 cases had fine pattern alterations.There were 2 cases treated with glucocorticoid alone,3 cases with glucocor-ticoid plus cyclophosphamide,3 cases with glucocorticoid plus rituximab;and 1 case received plasma exchange combined with glucocorticoid.Conclusions The clinical manifestations of Ig G4-RKD are diverse,usually involving multiple systems and organs,and the rate of misdiagnosis is high.Clinicians need to recognize and diagnose the disease as early as possible and conduct effective treatment to delay progression of the disease.