伴抗中性粒细胞胞质抗体阳性的抗肾小球基膜肾炎患者临床病理特征及预后

Clinicopathological characteristics and prognosis of anti-glomerular basement membrane nephritis with antineutrophil cytoplasmic antibody

目的:探讨伴抗中性粒细胞胞质抗体(ANCA)阳性的抗肾小球基膜(GBM)肾炎患者临床、病理特征及预后。方法:选取2004年1月至2020年12月在国家肾脏疾病临床医学研究中心住院治疗的抗GBM肾炎患者,分析伴ANCA阳性患者的临床病理特征及预后,并与ANCA阴性患者进行比较。结果:192例抗GBM肾炎患者中,33例(17%)合并血清ANCA阳性,均为髓过氧化物酶(MPO)ANCA,其中2例同时合并蛋白酶3(PR3)ANCA阳性。与ANCA阴性组相比,ANCA阳性组患者发病年龄偏高、女性占比多、血清白蛋白水平高(P<0.05),而24 h尿蛋白定量、抗GBM抗体滴度低于对照组(P<0.05),病理上除肾小管间质急性病变较轻(P=0.027),两组无明显差异。ANCA阳性组患者总体预后较差,随访1年两组的人、肾存活率均无统计学意义。结论:伴ANCA阳性的抗GBM肾炎患者临床和病理特征与不伴ANCA的抗GBM肾炎相似,预后较差。

Objective:To observe the clinical features, pathological presentation, and prognosis in anti-glomerular basement membrane(GBM) nephritis patients with antineutrophil cytoplasmic antibody(ANCA) positive.Methodology:The patients diagnosed with anti-GBM nephritis between January 2004 and December 2020 at the National Clinical Research Center of Kidney Diseases were selected to analyze the clinical and pathological characteristics and prognosis of patients with ANCA positive, and compared with the cohort of anti-GBM without ANCA. Results:Of the 192 anti-GBM nephritis, 33 patients coexisted MPO-ANCA with 2 patients simultaneous with PR3-ANCA. The elder onset age, female patient ratio, and serum albumin in the ANCA group were higher than those in the control group(P<0.05), but 24 hr proteinuria and titer of anti-GBM antibody were lower(P<0.05). There was no significant difference in the pathological features except for acute lesions of tubulointerstitial(P=0.027). There was no significant difference in survival rate and renal survival at one-year follow-up between the two groups. Conclusion:Anti-GBM nephritis with ANCA patients were similar to those without ANCA patients in the clinical and pathological characteristics, with poor prognosis.