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托珠单抗对一例合并多中心Castleman病的免疫沉积物相关膜增生性肾小球肾炎和肾小管间质性肾炎有显著疗效。

Significant response to tocilizumab in a case of immune deposits-related membranoproliferative glomerulonephritis and tubulointerstitial nephritis complicated by multicentric Castleman's disease.

作者信息

Sugimoto Hisashi, Sawa Naoki, Ikuma Daisuke, Oba Yuki, Mizuno Hiroki, Sekine Akinari, Yamanouchi Masayuki, Hasegawa Eiko, Suwabe Tatsuya, Wada Takehiko, Kono Kei, Kinowaki Keiichi, Ohashi Kenichi, Honda Kazuho, Kanetsuna Yukiko, Joh Kensuke, Yamaguchi Yutaka, Ubara Yoshifumi

机构信息

Nephrology Center and the Okinaka Memorial Institute for Medical Research.

Department of Pathology, Toranomon Hospital.

出版信息

Clin Nephrol Case Stud. 2024 Dec 20;12:73-82. doi: 10.5414/CNCS111337. eCollection 2024.

Abstract

A 47-year-old woman with a 12-year history of anemia and high C-reactive protein (CRP) levels was admitted to our hospital with worsening fatigue and night sweats. She had high levels of immunoglobulin G (IgG; 4182 mg/dL), IgA (630.6 mg/dL), and CRP (7.44 mg/dL); a low hemoglobin level (8.9 g/dL); urinary protein (11.83 g/day); and urinary sediment (20 - 29 red blood cells per high power field). On the basis of the clinical findings and biopsied lymph nodes, we diagnosed multicentric Castleman's disease (MCD). Light microscopy of kidney biopsy samples revealed various nephropathies, including membranoproliferative glomerulonephritis with crescentic formation and focal segmental sclerosis and tubulointerstitial nephritis. Immunofluorescence and electron microscopy revealed IgG-positive deposits in the subepithelial areas, mesangial areas, and tubular basement membrane. The patient's clinical findings including kidney disease improved after treatment with tocilizumab. MCD is considered to be caused by abnormally high levels of interleukin (IL)-6. Tocilizumab, an IL-6 receptor antagonist, was effective in this patient, indicating that the immune complex-related kidney findings were also related to MCD.

摘要

一名有12年贫血病史且C反应蛋白(CRP)水平升高的47岁女性因疲劳加重和盗汗入院。她的免疫球蛋白G(IgG;4182mg/dL)、IgA(630.6mg/dL)和CRP(7.44mg/dL)水平升高;血红蛋白水平低(8.9g/dL);尿蛋白(11.83g/天);以及尿沉渣(每高倍视野20 - 29个红细胞)。根据临床表现和活检淋巴结,我们诊断为多中心Castleman病(MCD)。肾脏活检样本的光镜检查显示了各种肾病,包括伴有新月体形成的膜增生性肾小球肾炎、局灶节段性硬化和肾小管间质性肾炎。免疫荧光和电子显微镜检查显示IgG阳性沉积物位于上皮下区域、系膜区域和肾小管基底膜。使用托珠单抗治疗后,患者包括肾病在内的临床表现有所改善。MCD被认为是由白细胞介素(IL)-6水平异常升高引起的。托珠单抗是一种IL-6受体拮抗剂,对该患者有效,表明免疫复合物相关的肾脏表现也与MCD有关。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/bc59/11706227/ade1c4271a97/CNCS-12-073-01.jpg

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