肾功能正常的抗肾小球基底膜病临床分析

目的 了解肾功能正常的抗肾小球基底膜(GBM)病的临床和病理特点.方法 回顾性分析6例长期保持肾功能正常的抗GBM病患者的临床病理特点,并与同期伴肾功能损害的29例抗GBM病患者进行比较,分析影响预后的因素.结果 35例患者中6例(17.1％)肾功能一直保持正常.5例患者有不同程度肾脏受累,1例仅有肺出血,3例表现为肺肾出血综合征.6例均行肾活检病理检查,免疫荧光:4例表现为IgG2+ ～3+沿肾小球毛细血管襻呈线样沉积;光镜差异较大,从肾小球轻微病变到新月体肾炎伴大量毛细血管襻坏死,但大多数患者病变轻微.5例患者给予免疫抑制和(或)血浆置换治疗.6例肾功能正常者与同期肾功能异常的29例抗GBM病相比,贫血轻[血红蛋白(99.67±19.80)比(77.97±20.62) g/L,P＝0.024]、抗GBM抗体滴度低[(80.23±85.73)比(224.34±145.79)EU/ml,P＝0.027]、肾小球新月体比例低(0.17±0.27比0.58±0.29,p＝0.005).平均随访12～133个月,6例肾功能正常者4例完全治愈,2例遗留少量蛋白尿、镜下血尿.结论 肾功能正常的抗GBM病并不少见,多数患者肾脏临床病理轻,预后良好.

Clinical analysis of anti glomerular basement membrane disease with normal renal function

Objective To investigate the clinical and pathologic characteristics of anti-glomerular basement membrane(GBM) disease with normal renal function.Methods The clinical and pathologic data of 6 patients with anti-GBM disease and normal renal function in Peking Union Medical College Hospital were reviewed retrospectively.Furthermore,29 patients with anti-GBM disease and impaired renal function in the same period in the same hospital were enrolled as the control group.Factors that may influence the prognosis we...