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[系膜增生性肾小球肾炎合并多中心性Castleman病病例]

[Case of mesangial proliferative glomerulonephritis complicated with multicentric Castleman's disease].

作者信息

Fujiwara Hiroaki, Mise Naobumi, Ishimoto Yu, Kotera Nagaaki, Tanaka Mototsugu, Tanaka Shinji, Kurita Noriaki, Fujii Akiko, Yamaguchi Yutaka, Sugimoto Tokuichiro

机构信息

Department of Internal Medicine, Mitsui Memorial Hospital, Tokyo, Japan.

出版信息

Nihon Jinzo Gakkai Shi. 2011;53(2):189-94.

Abstract

We report a case of a 47-year-old man with multicentric Castleman's disease (MCD) and progressive renal dysfunction due to mesangial proliferative glomerulonephritis, possibly from IgA nephropathy. At age 36 years, he was referred to a hematologist due to hypergammaglobulinemia. Because of systemic lymph node swelling, he underwent right cervical lymph node biopsy at age 41 years and MCD (plasma cell type)was diagnosed. During this period, microscopic hematuria and persistent proteinuria occurred and his renal function deteriorated (serum creatinine (Cr) rising from 0.7 mg/dL to 1.4 mg/dL). Treatment with intravenous methylprednisolone at the dose of 1 g daily for 3 days followed by oral prednisolone at 20 mg daily reduced his lymphadenopathy and improved the renal function. However, his renal function deteriorated again, from Cr 0.8 mg/dL to 1.8 mg/dL over 6 years in line with gradual prednisolone tapering to 6 mg daily. At age 47 years, he was referred to our nephrology department and underwent a renal biopsy. The microscopic examination showed IgA nephropathy with crescent formation, accompanied by mild lymphoplasmacytic tubulointerstitial nephritis. Treatment with the same dose of intravenous methylprednisolone therapy followed by oral prednisolone at 40 mg daily, improved his proteinuria, hematuria and renal dysfunction. The coexistence of MCD and IgA nephropathy is a rare phenomenon. In addition, IL-6, overproduced by MCD might have influenced the mesangial cell proliferation and the activity of IgA nephropathy in the present case.

摘要

我们报告一例47岁男性,患有多中心Castleman病(MCD),并因系膜增生性肾小球肾炎出现进行性肾功能不全,可能源自IgA肾病。36岁时,他因高球蛋白血症被转诊至血液科医生处。由于全身淋巴结肿大,他在41岁时接受了右颈淋巴结活检,被诊断为MCD(浆细胞型)。在此期间,出现了镜下血尿和持续性蛋白尿,肾功能恶化(血清肌酐(Cr)从0.7mg/dL升至1.4mg/dL)。给予每日1g静脉注射甲泼尼龙治疗3天,随后每日口服20mg泼尼松龙,减轻了他的淋巴结病并改善了肾功能。然而,随着泼尼松龙逐渐减量至每日6mg,他的肾功能在6年内再次恶化,Cr从0.8mg/dL升至1.8mg/dL。47岁时,他被转诊至我们的肾内科,并接受了肾活检。显微镜检查显示为伴有新月体形成的IgA肾病,伴有轻度淋巴浆细胞性肾小管间质性肾炎。给予相同剂量的静脉注射甲泼尼龙治疗,随后每日口服40mg泼尼松龙,改善了他的蛋白尿、血尿和肾功能不全。MCD和IgA肾病并存是一种罕见现象。此外,MCD过度产生的IL-6可能在本病例中影响了系膜细胞增殖和IgA肾病的活动。

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