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一例伴有单克隆IgA1沉积的肝肾小球硬化症

A Case of Hepatic Glomerulosclerosis with Monoclonal IgA1- Deposits.

作者信息

Okabayashi Yusuke, Tsuboi Nobuo, Nakaosa Naoko, Haruhara Kotaro, Kanzaki Go, Koike Kentaro, Shimizu Akihiro, Fukui Akira, Okonogi Hideo, Miyazaki Yoichi, Kawamura Tetsuya, Ogura Makoto, Shimizu Akira, Yokoo Takashi

机构信息

Division of Nephrology and Hypertension, Department of Internal Medicine, The Jikei University School of Medicine, Tokyo, Japan.

Department of Analytic Human Pathology, Nippon Medical School, Tokyo, Japan.

出版信息

Case Rep Nephrol. 2018 Oct 1;2018:4748357. doi: 10.1155/2018/4748357. eCollection 2018.

Abstract

Glomerular immunoglobulin A (IgA) deposition is a common finding in hepatic glomerulosclerosis; thus, this disease is also called hepatic IgA nephropathy. However, only a small number of patients with hepatic IgA nephropathy have active glomerular lesions, so functional decline is slow in most cases. In this report, we describe a 60-year-old man who developed nephrotic syndrome and progressive renal impairment during follow-up for alcoholic liver cirrhosis. A renal biopsy showed a membranoproliferative glomerulonephritis-like pattern; diffuse double-contours of the glomerular basement membrane and focal active glomerular lesions with moderate-to-severe endocapillary proliferation and fibrocellular crescents. Immunofluorescence findings revealed granular staining for monoclonal IgA1- and C3 on the peripheral capillary walls. Laboratory examinations did not reveal any definitive evidence of myeloproliferative disorders. Therefore, this case may represent a previously unrecognized etiology of renal injury in relation to liver cirrhosis that is characterized by monoclonal IgA1- deposits and proliferative glomerulonephritis.

摘要

肾小球免疫球蛋白A(IgA)沉积是肝性肾小球硬化症的常见表现;因此,这种疾病也被称为肝性IgA肾病。然而,只有少数肝性IgA肾病患者存在活动性肾小球病变,所以在大多数情况下功能下降缓慢。在本报告中,我们描述了一名60岁男性,他在酒精性肝硬化随访期间出现了肾病综合征和进行性肾功能损害。肾活检显示为膜增生性肾小球肾炎样模式;肾小球基底膜呈弥漫性双轨征,伴有局灶性活动性肾小球病变,伴有中度至重度毛细血管内增生和纤维细胞性新月体形成。免疫荧光检查结果显示外周毛细血管壁上有单克隆IgA1和C3的颗粒状染色。实验室检查未发现骨髓增殖性疾病的确切证据。因此,该病例可能代表了一种与肝硬化相关的、以前未被认识的肾损伤病因,其特征为单克隆IgA1沉积和增殖性肾小球肾炎。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/5a62/6188736/3ab4ba17847b/CRIN2018-4748357.001.jpg

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