Department of Nephrology, Kurashiki Central Hospital, 1-1-1 Miwa, Kurashiki, Okayama, 7108602, Japan.
CEN Case Rep. 2024 Dec;13(6):561-564. doi: 10.1007/s13730-024-00885-z. Epub 2024 May 22.
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.
我们在此报告一例 20 岁男性 IgA 肾病病例,该患者通过使用利妥昔单抗(RTX)治疗维持微小病变性肾病综合征(MCNS)完全缓解。他在 4 岁时被诊断为肾病综合征。在频繁复发后,他在 8 岁时被诊断为 MCNS,这是基于肾活检的结果。在 13 岁时,开始使用 RTX 治疗以维持类固醇治疗后的完全缓解。MCNS 复发了两次,包括 RTX 给药间隔时间较长的时候。每当他复发时,都会使用类固醇诱导缓解,并用 RTX 维持缓解。在第 7 次 RTX 给药后 5 个月,血清 IgA 水平开始升高。在第 9 次 RTX 给药后,尽管尿蛋白水平表明完全缓解,但他出现了镜下血尿。在第 10 次给药时,还观察到尿蛋白和红细胞管型。在 RTX 初始给药后 84 个月进行了肾活检,该患者被诊断为 IgA 肾病的并发症。RTX 不被认为是 IgA 肾病的有效治疗方法。原因是即使给予 RTX,IgA1 也不会减少,因为 RTX 可能不会删除粘膜中的 B 细胞,并且由于存在 CD20 长寿浆细胞,IgA 产生也可能继续。即使在给予 RTX 时,如果尿液检查中存在肾小球肾炎的发现,也必须考虑 IgA 肾病的可能性。