肾功能正常的抗肾小球基底膜病临床分析

Clinical Analysis of Anti-glomerular Basement Membrane Disease with Normal Renal Function

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IgG 2+～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。

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 were analyzed.Results Six（17.1%） of all 35 patients maintained normal renal function for 12-133 months during follow-up.Five patients had microhematuria and proteinuria,one had pulmonary hemorrhage only,and three manifested as Goodpasture syndrome.Renal biopsies from 4 patients revealed linear deposition of IgG 2＋-3＋ along the glomerular capillary walls by immunofluorescence.As shown by normal light microscopy,mild mesangial proliferation and crescentic glomerulonephritis with a large amount of fibrinoid necrosis of glomerular capillary walls were observed in different patients;however,most pathological changes were mild.Five of these six patients were treated with immunosuppressive drugs and/or plasma exchange.Compared with the control group,the 6 patients with normal renal function had significantly higher hemoglobin[（77.97±20.62）vs.（99.67±19.80）g/L,P=0.024],lower titers of anti-GBM antibody[（224.34±145.79）vs.（80.23±85.73）EU/ml,P=0.027],and lower ratio of glomeruli with crescents[（0.58±0.29）vs.（0.17±0.27）,P=0.005].These 6 patients with normal renal function were followed up for 12-133 months,among whom 4 patients achieved complete remission and 2 had mild proteinuria and microhematuria.Conclusions Anti-GBM disease with normal renal function is not uncommon.Most patients have mild pathologic changes and good prognosis.