20例IgG4相关性肾病临床特征分析

An Analysis of Clinical Characteristics of 20 Cases of IgG4-Related Kidney Disease

目的通过分析20例IgG4相关性肾病(IgG4-related kidney disease,IgG4-RKD)患者的临床特点,为临床医生对该病的早期诊治提供参考。方法回顾性分析2014年4月至2019年12月首次就诊于四川大学华西医院的20例IgG4-RKD患者的一般资料、相关实验室指标以及治疗随访情况。结果20例IgG4-RKD患者年龄范围为15~74岁,中位发病年龄54.5岁,男女比例为2.33∶1。就诊原因前三位为肾功能异常(30.0%)、全身或局部不同程度的水肿(25.0%)和不明原因的咳嗽咳痰(20.0%)。只累及肾脏、累及2个、3个、5个、6个器官(含肾脏)的IgG4-RKD患者分别占35.0%、45.0%、5.0%、10.0%、5.0%。肾外器官受累前三位为淋巴结(40.0%)、肺(20.0%)、颌下腺(20.0%)。20例IgG4-RKD患者的主要血清学特征为:IgG4升高(100.0%),IgG升高(90.0%),IgG4/IgG比值中位数为0.35(0.17~1.02),IgG4倍数参考值(1.35 g/L)中位数为5.04(2.96~28.15)g/L,白蛋白降低(95.0%),球蛋白升高(65.0%),血肌酐升高(60.0%),胱抑素-C升高(85.0%),血红蛋白降低(90.0%),eGFR降低(64.71%),IgE升高(75.0%),补体3降低(66.67%),补体4降低(46.67%)。单用糖皮质激素治疗的患者15例(75.0%),糖皮质激素加免疫抑制剂治疗的患者5例(25.0%)。有随访的17例患者中,治疗后病情明显好转的16例(94.1%),治疗效果不理想的1例(5.9%)。结论IgG4-RKD以中老年男性发病为主,临床表现不特异,常和其他组织器官合并受累,应结合血清学检测结果对疾病进行综合评估。同时,加强血清学IgG4检测在筛查、诊断及治疗监测中的应用。

Objective To analyze the clinical characteristics of 20 cases of IgG4-related kidney disease(IgG4-RKD),so to provide a reference for clinicians to diagnose and treat the disease during the early stage.Methods A retrospective analysis was conducted using the general information,related laboratory indicators,treatment and follow-up of 20 IgG4-RKD patients who were first seen in the West China Hospital of Sichuan University from April,2014 to November,2019.Results Twenty IgG4-RKD patients ranged in age from 15 to 74 years,with a median onset age of 54.5 years,and the male to female ratio was 2.33:1.The top three reasons for the consultation were abnormal renal function(30.0%),systemic or local edema of different degrees(25.0%),and unexplained cough and sputum(20.0%).The numbers of IgG4-RKD patients involved in one,two,three,five,and six organs(including kidney)were 7,9,9,2,and 1,respectively.Lymph nodes(40.0%),lungs(20.0%),and submandibular glands(20.0%)were the top three extra-renal organs involved.The main serological characteristics of 20 patients with IgG4-RKD were:IgG4 increased in 100%of patients;IgG increased in 90.0%of patients;and the median odds ratio of IgG4/IgG was 0.35(0.17-1.02);the median fold increase in IgG4 from the reference value(1.35 g/L)was 5.04(2.96-28.15).Albumin decreased in 95.0%of patients,globulin increased in 65.0%of patients,serum creatinine increased in 60.00%of patients,cystatin-C increased in 85.0%of patients,hemoglobin decreased in 90.0%of patients,eGFR decreased in 64.71%of patients.Also,IgE increased in 75.00%of patients,complement 3 decreased in 66.67%of patients,and complement 4 decreased in 46.67%of patients.15 patients(75.0%)were treated with glucocorticoids alone,and 5 patients(25.00%)were treated with glucocorticoids plus immunosuppressants.Of the 17 patients who were followed up,16 patients(94.1%)showed a marked improvement after treatment,and 1 patient(5.9%)was not treated well.Conclusion IgG4-RKD mainly occurs in middle-aged and elderly men,and its clinical manifestations are not specific.It is often associated with other tissues and organs.A comprehensive evaluation of the disease should be combined with the results of serological testing.At the same time,the application of serological IgG4 detection in screening,diagnosis and treatment monitoring should be strengthened.