病例报告:对一名抗 GBM 抗体和 ANCA 双血清阳性患者采用包括阿伐戈班在内的积极综合疗法有效治疗坏死性新月体肾小球肾炎。

IF 1 Q4 UROLOGY & NEPHROLOGY
Rina Tanaka, Takumi Toishi, Reiji Masaki, Hideaki Aihara, Sumie Sakamoto, Mari Ikeda, Tomohiko Inoue, Atsuro Kawaji, Masatoshi Matsunami, Junko Fukuda, Mamiko Ohara, Hiroshi Kuji, Daisuke Ichikawa, Tomo Suzuki
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引用次数: 0

摘要

抗肾小球基底膜(anti-GBM)肾炎通常伴有抗中性粒细胞胞浆抗体(ANCA),其预后很差,即使采取积极的治疗方法,通常也需要进行肾脏替代治疗(KRT)。在此,我们介绍了一例抗 GBM 抗体和 ANCA 双血清阳性的坏死性新月体肾小球肾炎患者,该患者在接受了包括阿伐戈班在内的积极治疗后成功实现了透析独立。患者是一名 77 岁的女性,患有快速进展性肾小球肾炎,髓过氧化物酶-ANCA 和抗 GBM 抗体双血清阳性。肾活检发现弥漫性细胞新月体坏死,免疫荧光染色显示免疫球蛋白(Ig)G1 和 IgG3 阳性,组织学诊断为抗肾小球基底膜肾炎。我们的治疗方法包括糖皮质激素、利妥昔单抗、小剂量环磷酰胺和阿伐潘辅助血浆置换的新组合。患者需要进行临时血液透析,尽管组织学预后不佳,但患者在12次透析后成功中止了透析。该病例强调,对于严重的抗-GBM肾炎,即使没有肺部受累,也应考虑积极的治疗策略,包括阿伐戈班,以避免KRT的需要。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Effective management of necrotizing crescentic glomerulonephritis using an aggressive combination therapy including avacopan in a patient double-seropositive for anti-GBM antibodies and ANCA: a case report.

The prognosis of anti-glomerular basement membrane (anti-GBM) nephritis, often accompanied by the presence of antineutrophil cytoplasmic antibodies (ANCA), is poor, and even with aggressive therapeutic approaches, kidney replacement therapy (KRT) is typically required. Here, we present a case of necrotizing crescentic glomerulonephritis in a patient double-seropositive for anti-GBM antibodies and ANCA who successfully achieved dialysis independence following aggressive treatment, including avacopan. The patient was a 77-year-old woman with rapidly progressive glomerulonephritis and double seropositivity for myeloperoxidase-ANCA and anti-GBM antibodies. A kidney biopsy revealed diffuse cellular crescents with necrosis and immunoglobin (Ig)G1 and IgG3 positivity on immunofluorescence staining, leading to a histological diagnosis of anti-glomerular basement membrane nephritis. Our treatment approach involved a novel combination of glucocorticoids, rituximab, low-dose cyclophosphamide, and plasma exchange complemented by avacopan. Temporary hemodialysis was required, and the patient successfully discontinued dialysis after 12 sessions despite a poor histological prognosis. This case underscores the significance of considering aggressive therapeutic strategies, including avacopan, for severe anti-GBM nephritis, even in the absence of lung involvement, to avert the need for KRT.

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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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