Maeshima Akito, Nakasatomi Masao, Henmi Daizo, Yamashita Shin, Kaneko Yoriaki, Kuroiwa Takashi, Hiromura Keiju, Nojima Yoshihisa
Department of Medicine and Clinical Science, Gunma University Graduate School of Medicine, 3-39-15 Showa, Maebashi, 371-8511, Japan.
CEN Case Rep. 2012 May;1(1):7-11. doi: 10.1007/s13730-012-0004-7. Epub 2012 Mar 17.
Castleman's disease is a benign lymphoproliferative disorder in which interleukin-6 (IL-6), a pleiotropic proinflammatory cytokine, is thought to play a pathogenetic role. Presented is the case of a 72-year-old man with Castleman's disease who exhibited progressive renal dysfunction with proteinuria. Renal biopsy revealed mesangial hypercellularity and matrix expansion in most glomeruli and peritubular inflammatory cell infiltration. Immunofluorescence studies showed intense deposition of IgG in a granular pattern along the glomerular basement membrane. Histological features were compatible with membranoproliferative glomerulonephritis accompanied by interstitial inflammatory cell infiltration. Immunohistological analysis showed that IL-6 was abundantly expressed by tubular cells and interstitial macrophages, suggesting involvement of IL-6 in the renal injury. As a result of administration of tocilizumab, a humanized anti-IL-6 receptor antibody, the patient experienced clinical and biochemical improvement of Castleman's disease, including marked reduction of proteinuria and stabilization of renal function. These findings suggest the efficacy of tocilizumab against Castleman's disease and its renal complications.
卡斯特曼病是一种良性淋巴增生性疾病,其中白细胞介素-6(IL-6),一种多效性促炎细胞因子,被认为发挥致病作用。本文介绍了一例72岁患有卡斯特曼病的男性患者,该患者出现进行性肾功能不全伴蛋白尿。肾活检显示大多数肾小球系膜细胞增多和基质扩张以及肾小管周围炎性细胞浸润。免疫荧光研究显示IgG沿肾小球基底膜呈颗粒状强烈沉积。组织学特征符合伴有间质炎性细胞浸润的膜增生性肾小球肾炎。免疫组织学分析显示IL-6在肾小管细胞和间质巨噬细胞中大量表达,提示IL-6参与肾损伤。给予人源化抗IL-6受体抗体托珠单抗后,患者卡斯特曼病的临床和生化指标得到改善,包括蛋白尿显著减少和肾功能稳定。这些发现提示托珠单抗对卡斯特曼病及其肾脏并发症有效。