一例在使用利妥昔单抗治疗极小变化肾病综合征完全缓解期间被诊断为 IgA 肾病的病例。

IF 1 Q4 UROLOGY & NEPHROLOGY CEN Case Reports Pub Date : 2024-05-22 DOI:10.1007/s13730-024-00885-z
Mana Nishikawa, Noriaki Shimada, Motoyasu Kurahashi, Kentaro Watanabe, Motoko Kanzaki, Kosuke Fukuoka, Kenichiro Asano
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引用次数: 0

摘要

我们在此报告了一例 IgA 肾病病例,患者是一名 20 岁的男性,通过使用利妥昔单抗(RTX),他的微小病变肾病综合征(MCNS)得到了完全缓解。他在 4 岁时被诊断出患有肾病综合征。在频繁复发后,他在 8 岁时根据肾活检结果被诊断为 MCNS。13 岁时,他开始接受 RTX 治疗,以维持类固醇治疗后的完全缓解。MCNS 复发了两次,其中一次 RTX 给药间隔时间较长。每次复发时,他都使用类固醇药物诱导缓解,并使用 RTX 维持缓解。第 7 次使用 RTX 5 个月后,血清 IgA 水平开始上升。第 9 次使用 RTX 后,尽管尿蛋白水平显示病情完全缓解,但他仍出现了微量血尿。在第 10 次给药时,还观察到尿蛋白和红细胞。在首次使用 RTX 84 个月后进行了肾活检,患者被诊断为 IgA 肾病并发症。RTX 被认为不是治疗 IgA 肾病的有效方法。究其原因,是因为即使在使用 RTX 后,IgA1 也不会减少,因为 RTX 可能无法清除黏膜中的 B 细胞,而且由于 CD20 长寿命浆细胞的存在,IgA 的生成也可能会继续。即使使用 RTX,如果尿检发现肾小球肾炎,也必须考虑 IgA 肾病的可能性。
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A case diagnosed with IgA nephropathy during a complete remission of minimal change nephrotic syndrome treated with rituximab.

We herein report a case of IgA nephropathy in a 20-year-old male who maintained a complete remission of minimal change nephrotic syndrome (MCNS) through the administration of rituximab (RTX). He was diagnosed with nephrotic syndrome at 4 years of age. After he relapsed frequently, he was diagnosed with MCNS at 8 years of age based on the findings of a kidney biopsy. At 13 years of age, RTX therapy was initiated to maintain a complete remission after steroid treatment. MCNS recurred twice, including the time in which the interval between the RTX administrations was long. Whenever he relapsed, remission induction was achieved using steroids, and remission maintenance was achieved using RTX. Five months after the 7th RTX administration, the serum IgA level started to increase. After the 9th RTX administration, he demonstrated microhematuria despite the urinary protein level indicating complete remission. At the 10th administration, the urinary protein and the red-blood cell casts were also observed. A renal biopsy was performed 84 months after the initial administration of RTX, and the patient was diagnosed with complications of IgA nephropathy. RTX is not considered to be a useful treatment for IgA nephropathy. The reasons for this are due to the fact that IgA1 does not decrease even following the administration of RTX, because B cells residing in the mucosa may not be deleted by RTX, and IgA production may also continue due to the presence of CD20- long-lived plasma cells. Even when administering RTX, if there are findings of glomerulonephritis on urine testing, the possibility of IgA nephropathy must be considered.

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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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