摘要目的 探讨2010年Berden等人提出的抗中性粒细胞胞质抗体(ANCA)相关性小血管炎的肾脏病理分型与肾脏预后的相关性.方法 收集2000年1月1日至2015年5月31日于北京协和医院接受经皮肾活检,临床资料保存完整的ANCA相关性小血管炎患者的临床及肾脏病理资料,按照Berden分型的流程分为4型(局灶型、新月体型、混合型、硬化型),比较4型患者临床特征、各随访时间点的肾功能、肾脏缓解情况.结果 共有88例患者入选,局灶型19例(21.6％),新月体型21例(23.9％),混合型32例(36.4％),硬化型16例(18.2％).22例患者进展至终末期肾病(ESRD),19例患者死亡(其中1例患者死亡前已进入ESRD).基线时各型平均肾小球滤过率估算值分别为68.04、25.45、30.04、15.16 ml· min-1·(1.73 m2)-1(P＜0.05).且随访各期局灶型的肾功能始终最佳,硬化型最差,新月体型及混合型居中且接近.6个月时局灶型、新月体型、混合型、硬化型的肾脏缓解率分别是62.5％、73.7％、57.5％、30.8％(P＞0.05),新月体型肾功能改善绝对值明显高于其他组.结论 局灶型的预后最好,硬化型最差,新月体和混合型居中.Berden分型与肾脏预后有相关性.

abstractsObjective To assess the predictive value of Berden classification in ANCA associated glomerulonephritis.Methods Patients with confirmed ANCA associated glomerulonephritis were included,by retrieving the medical database in Peking Union Medical College Hospital from January 2000 to May 2015.Their detailed information during hospitalization and follow-up was recorded.The patients were divided into four categories based on Berden classification.The differences in clinical characters,renal function and response for treatment were compared.Results Among the 88 patients with ANCA-associated glomerulonephritis,19 (21.6％),21 (23.9％),32 (36.4％)and 16 (18.2％) patients were classified as focal,mixed,crescentic and sclerotic category.22 patients developed ESRD,and 19 patients died during follow up (1 patient developed ESRD before died).The mean estimated glomerular filtration rate (eGFR) at baseline was 68.04,25.45,30.04,15.16 ml·min-1·(1.73 m2)-1 (P ＜ 0.05) in focal,crescentic,mixed and sclerotic category,respectively.During follow-up period,focal category always had the best renal function,while sclerotic category had the worst renal function.Crescentic category and mixed category were similar and in the middle.Remission rate at 6m was 62.5％,73.7％,57.5％,30.8％(P ＞ 0.05).And crescentic category had the greatest improvement in eGFR at 6m.Conclusions Focal category had relatively preserved renal function and favorable renal outcome,while the sclerotic category had the worst renal outcome.Crescentic and mixed category had an intermediate outcome.We support the use of the Berden classification in predicting the renal prognosis of patients with ANCA associated glomerulonephritis.