【摘要】 目的:探讨抗肾小球基底膜(GBM)肾炎合并不同肾脏疾病的临床及病理特征。方法:回顾性分析39例抗GBM肾炎合并不同肾脏疾病的临床表现、免疫抗体、病理特征和预后。结果:在39例患者中，男19例，女20例，平均(50.0±14.6)岁。其中经典抗GBM肾炎22例(56.4%),非典型抗GBM肾炎3例(7.7%),合并抗中性粒细胞胞浆抗体(ANCA)相关小血管炎6例(15.4%)、膜性肾病(MN)6例(15.4%)和免疫球蛋白A(IgA)肾病2例(5.1%)。病程(2.6±6.2)个月。临床症状以血尿(69.2%)、少尿/无尿(46.2%)和发热(43.6%)为主。首次就诊时需要透析的患者为31例(79.5%),其中经典抗GBM肾炎20例(90.9%),合并ANCA相关小血管炎和MN组均为4例(66.7%),合并IgA肾病组2例(100.0%),非典型抗GBM肾炎组1例(33.3%)。经典抗GBM肾炎和合并ANCA相关小血管炎组硬化肾小球的比例较高。经典抗GBM肾炎组细胞新月体的比例最高，而合并ANCA相关小血管炎组纤维新月体比例最高。免疫荧光染色显示所有患者IgG和C3染色阳性。结论:抗GBM肾炎合并不同肾脏疾病在病理生理学和预后方面明显不同，针对不同疾病特征的干预措施可能有助于改善肾脏结局。更多还原

【Abstract】 Objective:To explore the clinical and pathological features of anti-glomerular basement membrane(GBM) nephritis combined with different renal diseases and to provide a reference for clinicians to have a more systematic and clear understanding of this atypical disease.Methods:The clinical manifestations, immune antibodies, pathological characteristics and prognosis of 39 cases of anti-GBM nephritis combined with different renal diseases were retrospectively analyzed.Results:Among the 39 patients, there were 19 males and 20 females, with an average age of 50.0±14.6 years.Among them, 22 cases(56.4%) of classic anti-GBM nephritis, 3 cases(7.7%) of atypical anti-GBM nephritis, 6 cases(15.4%) of combined antineutrophil cytoplasmic antibody(ANCA)-associated vasculitis, 6 cases(15.4%) of membranous nephropathy(MN) and 2 cases(5.1%) of immunoglobulin A(IgA) nephropathy were diagnosed.The course of the disease was 2.6±6.2 months.The main clinical symptoms were hematuria(69.2%),oliguria/anuria(46.2%) and fever(43.6%).Thirty-one patients(79.5%) required dialysis at the first visit, including 20 cases of classic anti-GBM nephritis(90.9%),4 cases of combined ANCA-associated vasculitis and MN(66.7%),2 cases of combined IgA nephropathy(100.0%),and 1 case of atypical anti-GBM nephritis(33.3%).The proportion of sclerotic glomeruli was higher in the classic anti-GBM nephritis and combined ANCA-associated vasculitis subgroups.The proportion of cellular crescents was the highest in the classic anti-GBM nephritis group, while the proportion of fibrous crescents was the highest in the combined ANCA-associated vasculitis group.Immunofluorescence staining showed that all patients were positive for IgG and C3 staining.Conclusion:Anti-GBM nephritis combined with different renal diseases is significantly different in pathophysiology and prognosis, and intervention measures targeting different disease characteristics may help improve renal outcomes.更多还原