摘要：
Objective To investigate the clinical manifestations,renal pathology and prognosis of antineutrophil cytoplasmic antibody-associated small-vessel vasculitis (AAV) accompanied with renal glomerular IgA deposition.Methods A retrospective analysis was performed at the First Affiliated Hospital of Zhejiang University College of Medicine.Patients diagnosed with AAV associated renal injury by renal biopsy from February 2004 to February 2017 were enrolled.Patients with antiglomerular basement membrane antibody-mediated nephritis,systemic lupus erythematosus nephritis,Henoch Schonlein purpura nephritis,hepatitis B virus associated nephritis and other known etiology were excluded.According to immunofluorescence examination,the patients were divided into IgA deposition group and pauci-immune complex deposition group.The differences in clinical manifestation,pathological features and prognosis were compared between groups.Results A total of 150 AAV cases were included,among which 25 cases were with IgA deposition and 125 cases with pauci-immune complex deposition.The level of serum albumin in IgA deposition group was higher than that in pauci-immune complex deposition group [(35.0±6.2) g/L vs (32.6±5.3) g/L,P=0.049],but the titer of MPO-ANCA was lower [24.8(10.4,71.8) U/ml vs 63.0(21.9,100.0) U/ml,P=0.044] in IgA deposition group.There was no significant difference between two groups in other laboratory indexes and renal pathological findings.The median follow-up time was 15.2 months in IgA deposition group and 8.9 months in pauci immune complex deposition group.During the follow-up there were 8 patients (32.0％) in IgA deposition group and 29 patients (23.2％) in pauci immune complex deposition group on maintaining dialysis;2 patients (8.0％) in IgA deposition group and 7 patients (5.6％) in pauci immune complex deposition group died.There was no significant difference between two groups in patients' outcomes.Conclusions AAV patients with glomerular IgA deposition and AAV patients with typical glomerular immunoglobulin complex deposition are similar as regards clinical appearance and prognosis.%目的 探讨伴有肾小球IgA沉积的抗中性粒细胞胞浆抗体(ANCA)相关性小血管炎(AAV)肾损害患者的临床表现、肾脏病理及预后.方法 回顾性分析2004年2月至2017年2月在浙江大学医学院附属第一医院行原位肾穿刺活检明确诊断为AAV肾损害的患者,排除合并有抗肾小球基底膜抗体肾炎、系统性红斑狼疮肾炎、过敏性紫癜性肾炎、乙肝相关性肾炎及其他具有明确原发病因者.根据免疫荧光检查分为肾小球IgA沉积组和寡免疫复合物沉积组.结果 研究纳入150例患者,其中IgA沉积组25例,寡免疫复合物沉积组125例.IgA沉积组血白蛋白水平高于寡免疫复合物沉积组[(35.0±6.2) g/L比(32.6±5.3) g/L,P=0.049],但MPO-ANCA滴度[24.8(10.4,71.8) U/ml比63.0(21.9,100.0) U/ml,P=0.044]较低.两组的其他实验室指标及肾组织病理表现无显著差异.IgA沉积组中位随访15.2个月,寡免疫复合物沉积组中位随访8.9个月,期间IgA沉积组8例(32.0％)患者、寡免疫复合物沉积组29例(23.2％)患者进入维持性透析;IgA沉积组2例(8.0％)、寡免疫复合物沉积组7例(5.6％)患者死亡,两组间差异均无统计学意义.结论 伴肾小球IgA沉积的AAV患者与典型肾小球寡免疫复合物沉积的AAV患者临床表现及预后相似.
