临床荟萃

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IgM肾病的临床病理与治疗观察

  

  1. 1.承德市中心医院  肾内科,河北 承德 067000; 2.沈阳军区总医院 肾内科,辽宁 沈阳  110016
  • 出版日期:2017-07-05 发布日期:2017-07-11
  • 通讯作者: 通信作者:郑红光,Email: zhenghongguang528@163.com
  • 基金资助:
    辽宁省科技攻关项目(2015225010)

Study on clinicopathological and treatment  of  IgM  nephropathy

  1. 1.Department of Nephrology,Central Hospital of Chengde, Chengde  067000,China;
    2.Department of Nephrology,General Hospital of  Shenyang Military Region,Shenyang 110016,China
  • Online:2017-07-05 Published:2017-07-11
  • Contact: Corresponding author:Zheng Hongguang,Email: zhenghongguang528@163.com

摘要: 目的  探讨IgM肾病患者的发病规律并对其治疗情况进行总结。方法  对经肾活检证实的52例IgM肾病患者的临床病理资料及治疗情况进行分析。结果  IgM肾病发病率占成人原发性肾小球疾病的4.9%(52/1 045),多表现为肾病综合征。平均发病年龄(20.1±10.3)岁,男女之比1.6∶1。起病时合并高血压16例(30.7%),起病时合并急性肾衰竭5例(9.6%)。52例患者24小时尿蛋白定量(4.44±1.48) g;血白蛋白(16.68±4.65)  g/L;血胆固醇(12.75±3.64)  mmol/L;血甘油三酯(4.98±1.43)   mmol/L。52例患者光镜下见轻中度系膜增生40例(76.9%);重度系膜增生3例(5.7%);局灶节段硬化9例(17.3%)。免疫荧光见单纯IgM沉积型34例(65.3%);IgM+C3沉积12例(23.0%)。对糖皮质激素初治敏感14例(26.9%);对糖皮质激素依赖26例(50%);对糖皮质激素抵抗12例(23.0%)。合并应用环磷酰胺治疗后临床缓解30例(57.6%);无效19例(36.5%);恶化3例(5.7%)。结论  IgM肾病多见于青壮年男性,多表现为肾病综合征;光镜下多见系膜轻中度增生,免疫荧光以单纯IgM沉积为主;对糖皮质激素治疗的初治反应以激素依赖多见,合并应用环磷酰胺后临床缓解率提高。

关键词: 肾小球肾炎, IGM, 治疗, 临床研究性, 病理学, 临床

Abstract: Objective  To analyze the pathogenesis regularity and treatment of IgM nephropathy in adults with kidney disease.Methods  The clinicopathological and treatment  data were collected from 52 patients of IgM nephropathy confirmed by renal biopsy.Results  A  prevalence  of  IgM nephropathy of  4.9%(52/1 045)  accounted for all primary glomerular diseases on renal biopsies. The majority of the patients presented with nephrotic syndrome. The mean age was (20.1±10.3) years old. The malefemale ratio was 1.6∶1. 16 cases with hypertension at onset accounted for 30.7%.There were 5 patients with acute renal failure. The mean 24hour urine protein level was (4.44±1.48) g. The mean albumin  level was (16.68±4.65)  g/L. The mean cholesterol level was (12.75±3.64) mmol/L. The mean triglycerides level was (12.75±3.64) mmol/L. The most common morphologic change consisted of mild and moderate mesangial cell proliferaion of the glomeruli,found in 40 biopsies (76.9%),severe hyperplasia  in  3 biopsies(5.7%). The morphological pattern of FSGS was found in 9 cases(17.3%). IgM was the sole localizing immunoglobulin,and it was the predominant globulin in  34 cases(65.3%),IgM+C3 accounted for  23.0%(12/52). 14 cases( 26.9%)  were sensitive to glucocorticoid. There were 26 cases(50%) of glucocorticoid dependence. There were 12 cases(23%) of glucocorticoid resistance. Combined with cyclophosphamide treatment, clinical remission in 30 cases, the total clinical remission rate was 57.6%; invalid cases were 19 cases( 36.5%); 3 worsening cases(5.7%). Conclusion  A high incidence with IgM  nephropathy in young men. The majority of the patients presented with idiopathic nephrotic syndrome. The most common morphologic change consisted of mild and moderate mesangial cell proliferaion of the glomeruli.Predominant IgM deposition in mesangium was the sole localizing immunoglobulin. Glucocorticoid dependent was the most    common response. Clinical complete remission rate was increased in glucocorticoid with cyclophosphamide.

Key words: glomerulonephritis, IGM;therapies, investigational;pathology;therapeutic effect