托珠单抗对免疫沉积相关膜增殖性肾小球肾炎和小管间质性肾炎合并多中心Castleman病的疗效显著

Clinical nephrology. Case studies Pub Date : 2024-12-20 eCollection Date: 2024-01-01 DOI:10.5414/CNCS111337
Hisashi Sugimoto, Naoki Sawa, Daisuke Ikuma, Yuki Oba, Hiroki Mizuno, Akinari Sekine, Masayuki Yamanouchi, Eiko Hasegawa, Tatsuya Suwabe, Takehiko Wada, Kei Kono, Keiichi Kinowaki, Kenichi Ohashi, Kazuho Honda, Yukiko Kanetsuna, Kensuke Joh, Yutaka Yamaguchi, Yoshifumi Ubara
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引用次数: 0

摘要

一名47岁女性,12年贫血史,高c反应蛋白(CRP)水平,因疲劳加重和盗汗入住我院。她有高水平的免疫球蛋白G (IgG;4182 mg/dL)、IgA (630.6 mg/dL)和CRP (7.44 mg/dL);血红蛋白水平低(8.9 g/dL);尿蛋白(11.83 g/d);尿液沉淀物(每高倍视场20 - 29个红细胞)。根据临床表现和淋巴结活检,我们诊断为多中心Castleman病(MCD)。肾活检标本光镜显示多种肾病,包括月牙形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。免疫荧光和电镜显示igg阳性沉积在上皮下区域,系膜区域和管基底膜。患者的临床表现包括肾脏疾病在tocilizumab治疗后得到改善。MCD被认为是由异常高水平的白细胞介素-6引起的。Tocilizumab,一种IL-6受体拮抗剂,在该患者中有效,表明免疫复合物相关的肾脏发现也与MCD有关。
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Significant response to tocilizumab in a case of immune deposits-related membranoproliferative glomerulonephritis and tubulointerstitial nephritis complicated by multicentric Castleman's disease.

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.

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