IgM肾病；我们还能忽视它吗？
IgM nephropathy; can we still ignore it.
作者信息
Vanikar Aruna
机构信息
Department of Pathology, Laboratory Medicine and Transfusion Services and Department of Immunohematology, GR Doshi and KM Mehta Institute of Kidney Diseases & Research Centre, Gujarat, India.
出版信息
J Nephropathol. 2013 Apr;2(2):98-103. doi: 10.12860/JNP.2013.16. Epub 2013 Apr 1.
CONTEXT
IgM nephropathy (IgMN) is a relatively less recognized clinico-immunopathological entity in the domain of glomerulonephritis , often thought to be a bridge between minimal change disease and focal segmental glomerulosclerosis.
EVIDENCE ACQUISITIONS
Directory of Open Access Journals (DOAJ), Google Scholar, Pubmed (NLM), LISTA (EBSCO) and Web of Science has been searched.
RESULTS
IgM nephropathy can present as nephritic syndrome or less commonly with subnephrotic proteinuria or rarely hematuria. About 30% patients respond to steroids whereas others are steroid dependent / resistant. They should be given a trial of Rituximab or stem cell therapy.
CONCLUSIONS
IgM nephropathy (IgMN) is an important and rather neglected pathology responsible for renal morbidity in children and adults in developing countries as compared to developed nations with incidence of 2-18.5% of native biopsies. Abnormal T-cell function with hyperfunctioning suppressor T-cells are believed to be responsible for this disease entity. Approximately one third of the patients are steroid responders where as the remaining two thirds are steroid resistant or dependent. Therapeutic trials including cell therapies targeting suppressor T-cells are required.
背景
IgM肾病（IgMN）在肾小球肾炎领域是一种相对不太被认识的临床免疫病理实体，常被认为是微小病变病和局灶节段性肾小球硬化之间的桥梁。
证据收集
检索了开放获取期刊目录（DOAJ）、谷歌学术、PubMed（美国国立医学图书馆）、LISTA（EBSCO）和科学引文索引。
结果
IgM肾病可表现为肾炎综合征，较少见的是亚肾病性蛋白尿，罕见血尿。约30%的患者对类固醇有反应，而其他患者则为类固醇依赖/抵抗型。应给予他们利妥昔单抗或干细胞治疗的试验。
结论
与发达国家相比，IgM肾病（IgMN）在发展中国家是一种导致儿童和成人肾脏发病的重要且相当被忽视的病理情况，在肾活检中发生率为2% - 18.5%。异常的T细胞功能以及功能亢进的抑制性T细胞被认为是导致这种疾病实体的原因。大约三分之一的患者对类固醇有反应，而其余三分之二是类固醇抵抗或依赖型。需要进行包括针对抑制性T细胞的细胞治疗在内的治疗试验。
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