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伴有类固醇反应性肾病综合征的系膜IgA肾病:类固醇诱导缓解后系膜IgA沉积物消失

Mesangial IgA nephropathy with steroid-responsive nephrotic syndrome: disappearance of mesangial IgA deposits following steroid-induced remission.

作者信息

Cheng I K, Chan K W, Chan M K

机构信息

Department of Medicine, University of Hong Kong, Queen Mary Hospital, Pokfulam.

出版信息

Am J Kidney Dis. 1989 Nov;14(5):361-4. doi: 10.1016/s0272-6386(89)80168-4.

Abstract

This report describes the clinical features and renal biopsy pathology in two patients with immunoglobulin A (IgA) nephropathy and nephrotic syndrome before and after steroid-induced remission. Apart from confirming the frequently relapsing course and mild glomerular changes observed in patients with IgA nephropathy and steroid-responsive nephrotic syndrome (SRNS), we were able to show that mesangial expansion and mesangial IgA deposits disappeared or were greatly reduced in repeat renal biopsies following steroid-induced remission. Because mesangial IgA deposits usually persist in repeat biopsies obtained from patients with typical IgA nephropathy, their resolution in our patients following steroid remission would support the proposal that the association of IgA nephropathy and SRNS may represent a distinct clinical syndrome. It is postulated that the presence of mesangial IgA deposits during nephrotic presentation and their disappearance following steroid-induced remission may result from increased mesangial sequestration of IgA circulating immune complexes (CIC) during the period of enhanced glomerular permeability and that the increased load of IgA CICs may reflect a common defect in mucosal immunity or immunoregulation in these patients.

摘要

本报告描述了两名免疫球蛋白A(IgA)肾病合并肾病综合征患者在激素诱导缓解前后的临床特征及肾活检病理情况。除了证实IgA肾病合并激素敏感性肾病综合征(SRNS)患者常出现复发病程及轻微肾小球改变外,我们还发现,在激素诱导缓解后的重复肾活检中,系膜扩张和系膜IgA沉积消失或显著减少。由于系膜IgA沉积在典型IgA肾病患者的重复活检中通常持续存在,因此我们患者在激素缓解后系膜IgA沉积的消退支持了以下观点,即IgA肾病与SRNS的关联可能代表一种独特的临床综合征。据推测,肾病表现期系膜IgA沉积的存在及其在激素诱导缓解后的消失,可能是由于肾小球通透性增强期间系膜对循环免疫复合物(CIC)中IgA的隔离增加所致,而IgA CIC负荷的增加可能反映了这些患者黏膜免疫或免疫调节的共同缺陷。

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