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一例罕见的伴有系膜IgA沉积的4型快速进展性肾小球肾炎(非典型)病例报告

A Rare Case of Type 4 Rapidly Progressive Glomerulonephritis (Atypical) with Mesangial IgA Deposits: A Case Report.

作者信息

Bhuwania Puneet, Veerappan Ilangovan, Sethuraman Ramaswami

机构信息

Department of Nephrology, KG Hospital and PG Institute, Coimbatore, Tamil Nadu, India.

出版信息

Indian J Nephrol. 2021 Sep-Oct;31(5):488-491. doi: 10.4103/ijn.IJN_364_20. Epub 2021 Apr 2.

Abstract

Rapidly progressive glomerulonephritis can result from glomerular deposition of anti-GBM antibody, immune complexes, or may involve pauci-immune mechanisms. The coexistence of IgA nephropathy, anti-GBM, and anti-neutrophilic cytoplasmic antibodies is unheard of, and the pathogenic role of these antibodies in IgA nephropathy or vice versa remains unclear. Herein, we describe a case of a patient with type 4 rapidly progressive glomerulonephritis who was found to have significant mesangial IgA deposits. The prognosis of this remains unclear but our patient responded well to cytotoxic therapy and plasmapheresis and achieved remission by 6 months. The findings suggest an overlap syndrome of IgA nephropathy-associated type 4 crescentic glomerulonephritis that resembles the former histologically and the latter in its potential to respond to aggressive therapy if detected relatively early in its course.

摘要

快速进展性肾小球肾炎可由抗肾小球基底膜(GBM)抗体、免疫复合物在肾小球沉积引起,或可能涉及寡免疫机制。IgA肾病、抗GBM抗体和抗中性粒细胞胞浆抗体并存的情况闻所未闻,这些抗体在IgA肾病中的致病作用或反之亦然仍不清楚。在此,我们描述一例4型快速进展性肾小球肾炎患者,该患者被发现有显著的系膜IgA沉积。其预后尚不清楚,但我们的患者对细胞毒性治疗和血浆置换反应良好,并在6个月时实现缓解。这些发现提示了一种IgA肾病相关的4型新月体性肾小球肾炎重叠综合征,其在组织学上类似于前者,且如果在病程中相对早期检测到,则有可能对积极治疗产生反应。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ccfd/8597790/c8d3f9b9e869/IJN-31-488-g001.jpg

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