皮质类固醇和环孢素A有效联合治疗免疫球蛋白A肾病伴膜增生性肾小球肾炎1例

IF 1 Q4 UROLOGY & NEPHROLOGY
Saeko Hatanaka, Go Kanzaki, Kentaro Koike, Hiroyuki Ueda, Nanae Matsuo, Yukio Maruyama, Nobuo Tsuboi, Takashi Yokoo
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引用次数: 0

摘要

免疫球蛋白A肾病(IgAN)是世界范围内最常见的原发性肾小球肾炎,具有异质性的组织病理表型。尽管在儿童和成人中已经报道了具有膜增生性肾小球肾炎(MPGN)样特征的IgAN,但这种罕见IgAN亚型的治疗策略尚未建立。在这里,我们提出的情况下,56岁的男子没有肾脏疾病的历史,谁最初提出了肾病综合征。肾活检显示mpn样特征,继发性血清学检查阴性。免疫荧光染色显示肾小球系膜和毛细血管壁IgA阳性。半乳糖缺乏的IgA1染色显示与IgA染色相似的分布模式。电镜显示系膜和内皮下区域有紊乱的结构沉积物。根据临床和组织病理学结果,患者被诊断为原发性IgAN。经皮质类固醇和环孢素A (CsA)联合治疗6个月后,肾病综合征完全消失。尽管皮质类固醇和CsA逐渐减少,血尿和蛋白尿在多年的随访中仍处于完全缓解状态。该病例证明了将具有mpgn样特征的IgAN作为一种可能受益于强化免疫抑制治疗的组织病理学亚型的重要性。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Effective combination of corticosteroid and cyclosporine A for immunoglobulin A nephropathy with membranoproliferative glomerulonephritis features: a case report.

Immunoglobulin A nephropathy (IgAN) is the most common primary glomerulonephritis worldwide with heterogeneous histopathological phenotypes. Although IgAN with membranoproliferative glomerulonephritis (MPGN)-like features has been reported in children and adults, treatment strategies for this rare IgAN subtype have not been established. Here, we present the case of a 56-year-old man with no history of kidney disease who initially presented with nephrotic syndrome. Renal biopsy revealed MPGN-like features with a negative serological workup for secondary causes. Immunofluorescent staining was predominantly positive for IgA in the glomerular mesangial and capillary walls. Galactose-deficient IgA1 staining showed a distribution pattern similar to IgA staining. Electron microscopy revealed disorganized structural deposits in the mesangial and subendothelial regions. Based on clinical and histopathological findings, the patient was diagnosed with primary IgAN. The nephrotic syndrome resolved completely after six months of combined corticosteroids and cyclosporine A (CsA) therapy. Although corticosteroids and CsA were tapered off, hematuria and proteinuria remained in complete remission for years of follow-up. This case demonstrates the importance of recognizing IgAN with MPGN-like features as a histopathological subtype that may benefit from intensive immunosuppressive therapy.

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来源期刊
CEN Case Reports
CEN Case Reports UROLOGY & NEPHROLOGY-
CiteScore
1.90
自引率
0.00%
发文量
80
期刊介绍: Clinical and Experimental Nephrology (CEN) Case Reports is a peer-reviewed online-only journal, officially published biannually by the Japanese Society of Nephrology (JSN).  The journal publishes original case reports in nephrology and related areas.  The purpose of CEN Case Reports is to provide clinicians and researchers with a forum in which to disseminate their personal experience to a wide readership and to review interesting cases encountered by colleagues all over the world, from whom contributions are welcomed.
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