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目的:探讨抗肾小球基底膜(glomerularbasementmembrane,GBM)抗体相关疾病的临床、病理特点。方法:回顾性分析6例抗GBM抗体相关疾病的临床、病理资料。结果:全组6例有血尿及蛋白尿,3例少尿或无尿,5例血清肌酐升高[(683～1251)μmol/L],1例肺出血,4例贫血;6例抗GBM抗体阳性,其中2例合并抗中性粒细胞胞浆抗体(antineutrophilcytoplasmantibody,ANCA)阳性;6例行肾活组织检查(肾活检),5例有细胞新月体形成,另1例无新月体仅为系膜增生;5例免疫荧光表现为IgG或IgA和补体(C3)沿肾小球毛细血管基底膜呈颗粒沉积;治疗后4例临床缓解。结论:并非所有抗GBM抗体相关疾病患者病理表现为典型的沿肾小球毛细血管基底膜呈线形沉积,一些患者表现为颗粒沉积,这类患者预后较好,并不排除存在地区分布差异性。
Objective: To investigate the clinical and pathological features of anti-glomerular basement membrane (GBM) antibody-related diseases. Methods: The clinical and pathological data of 6 anti-GBM antibody-related diseases were retrospectively analyzed. Results: All patients had hematuria and proteinuria in 6 cases, oliguria or anuria in 3 cases, elevated serum creatinine in 5 cases [(683-1251) μmol / L], 1 case of pulmonary hemorrhage and 4 cases of anemia. Six cases of anti-GBM 2 were positive with antineutrophilcytoplasmatibody (ANCA), 6 were examined by renal biopsy (renal biopsy), 5 were with crescent of cell and 1 without crescent Only mesangial proliferation; 5 cases of immunofluorescence showed IgG or IgA and complement (C3) along the glomerular capillary basement membrane deposition; 4 cases of clinical remission. CONCLUSIONS: Not all patients with anti-GBM antibody-related disease typically present a linear deposition along the glomerular capillary basement membrane, and some patients present as pellet deposits. These patients have a better prognosis and do not preclude the presence of regional differences.