目的:了解系统性血管炎合并肾小球膜性病变患者的临床病理特征. 方法:收集符合上述要求的4例住院患者,分析临床及肾活检病理资料.结果:4例患者均符合系统性血管炎的诊断标准,其中2例抗中性粒细胞胞质抗体(ANCA)阳性[MPO-ANCA(ELISA法)和P-ANCA(荧光法)];2例临床符合急进性肾炎综合征,4例患者除血尿外,均存在肾病性蛋白尿(其中3例为超大量蛋白尿)、低蛋白血症,3例血清肌酐升高;肾活检组织学除见肾小球新月体、袢坏死等血管炎性病变外,还存在肾小球膜性病变;IgG亚型分析提示继发性膜性病变.2例患者经积极免疫抑制剂治疗后,血尿减少,ANCA滴度下降,2例未积极治疗者,1例肾功能缓慢进展,1例进入维持性血液透析. 结论:系统性血管炎合并肾小球膜性病变的诊断需要依赖肾活检,预后与是否积极治疗相关.其发病机制有待于进一步深入研究.%Objective:To investigate the clinical and histological features in systemic vasculitis patients combined with membranous nephropathy. Methodology:The clinical data and histological features of four patients in hospital were analyzed. Results :They were three males and one female with the age of 39 to 73 years old. All of them were in accord with the diagnosis of systemic vasculitis. Among them, 2 cases were serum MPO-ANCA and P-ANCA positive, and 2 cases were as rapidly progressive glomerulonephritis. All patients had nephrotic syndrome (3 had massive proteinuria) except for large micro-hematuria. 3 had elevated serum creatinine (SCr). Renal biopsy specimen revealed necrotizing glomerulonephritis with crescent formation,and subepithelial electron-dense deposits were detected by electron microscopy.Subtype IgG analysis prefered the secondary membranous glomerulonephropathy. 2 patients had significantly improvement of hematuria and ANCA titer with immunosuppressive therapy. But those who didn't receive active treatment, 1 had increased SCr and 1 entered ESRD. Conclusion: Systemic vasculitis with membranous nephropathy was rare coincidence. The diagnosis depended on renal biopsy.
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