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合并抗肾小球基底膜病和免疫复合物性肾小球肾炎。

Concurrent antiglomerular basement membrane disease and immune complex glomerulonephritis.

作者信息

Cui Zhao, Zhao Ming-Hui, Wang Su-Xia, Liu Gang, Zou Wan-Zhong, Wang Hai-Yan

机构信息

Renal Division and Institute of Nephrology, Peking University First Hospital, Beijing 100034, P.R. China.

出版信息

Ren Fail. 2006;28(1):7-14. doi: 10.1080/08860220500461195.

DOI:10.1080/08860220500461195
PMID:16526313
Abstract

Antiglomerular basement membrane (GBM) disease is characteristically described with linear deposition of IgG along GBM. However, the concurrent glomerular immune complex deposition was not rare and might be contributed to the development of anti-GBM disease. In the current series, glomerular immune complexes were identified in 10 of 47 patients who presented with renal-biopsy-proven anti-GBM disease. Six of the 10 patients complicated with a well-documented glomerulonephritis, including two patients with membranous nephropathy, one patient with IgA nephropathy, one patient with membranoproliferative glomerulonephritis, one patient with Schonlein-Henoch nephritis, and one patient with hepatitis B virus associated membranous nephritis. The other four patients had immune complexes with IgG or IgM predominance deposited in glomerular mesangium without a well-documented glomerulonephritis. Clinical and pathological data of patients with immune complex deposition (n = 10) were compared with those of patients with anti-GBM disease alone (n = 37). There was no significant difference in age, gender, clinical and pathological manifestations, and renal outcome between the two groups. In general, the association of glomerular immune complexes did not lead to a benign prognosis. Plasma exchange and extensive immunosuppressive therapy should be carried out as soon as possible. The immune complexes deposited in glomeruli might participate in the initiation of anti-GBM disease.

摘要

抗肾小球基底膜(GBM)病的特征性表现为IgG沿GBM呈线性沉积。然而,并发的肾小球免疫复合物沉积并不罕见,可能促成了抗GBM病的发生。在本系列研究中,47例经肾活检证实为抗GBM病的患者中有10例发现了肾小球免疫复合物。这10例患者中有6例并发了有充分记录的肾小球肾炎,包括2例膜性肾病患者、1例IgA肾病患者、1例膜增生性肾小球肾炎患者、1例紫癜性肾炎患者和1例乙型肝炎病毒相关性膜性肾病患者。另外4例患者的免疫复合物以IgG或IgM为主,沉积于肾小球系膜,未并发有充分记录的肾小球肾炎。将有免疫复合物沉积的患者(n = 10)与单纯抗GBM病患者(n = 37)的临床和病理数据进行了比较。两组在年龄、性别、临床和病理表现及肾脏转归方面无显著差异。总体而言,肾小球免疫复合物的存在并未导致预后良好。应尽快进行血浆置换和广泛的免疫抑制治疗。沉积于肾小球的免疫复合物可能参与了抗GBM病的发病过程。

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