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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 understand the clinicopathological features of patients with systemic vasculitis and glomerular membrane disease. Methods: Four inpatients who meet the above requirements were collected and their clinical and renal biopsy data were analyzed. Results: All the 4 patients were in line with the diagnostic criteria of systemic vasculitis, of which 2 were anti-neutrophil cytoplasmic antibody (ANCA) positive [MPO-ANCA (ELISA) and P-ANCA In addition to hematuria, nephrotic proteinuria (3 of them was hypervolemic proteinuria), hypoalbuminemia, and 3 cases of elevated serum creatinine were found in all 4 patients except for hematuria. In addition to histological examination of renal biopsies Glomerular crescent, 袢 necrosis and other vascular inflammatory lesions, there are still glomerular lesions; IgG subtype analysis suggests secondary membranous lesions. After 2 patients were treated with active immunosuppressive agents, hematuria decreased, ANCA titers decreased, 2 patients were not actively treated, 1 patient had slow progression of renal function, and 1 patient was in maintenance hemodialysis. CONCLUSIONS: The diagnosis of systemic vasculitis associated with mesangial lesions depends on renal biopsy, and prognosis is associated with aggressive treatment. Its pathogenesis needs to be further studied.