李倩倩 李胜开

[摘要] 目的 探讨合并肾损害的抗中性粒细胞胞浆抗体（ANCA）相关性血管炎（AAV）患者的临床特征和预后相关因素。 方法 回顾性分析2013年8月～2017年8月在徐州医科大学附属医院肾内科确诊的65例合并肾损害的AAV患者的临床资料，包括一般特点、系统受累、辅助检查及治疗转归情况。对有完整随访结局的患者（共54例）按不同預后分为出现不良预后组（24例）和未出现不良预后组（30例），比较两组临床特点及辅助检查情况。 结果 患者平均年龄（65±12）岁，中位确诊时间1（0.5～4.0）个月。累及多系统，泌尿系统（65例）和呼吸系统（45例）最常见。肺部受累中间质病变42例（42/65，64.6%）。血清学检查示贫血57例（57/65，87.7%），C反应蛋白升高57例（87.7%），血沉增快58例（89.2%）。ANCA以MPO阳性为主（60例，92.3%）。7例行肾活检，4例为新月体性肾小球肾炎，3例免疫荧光阴性。55例行糖皮质激素治疗，47例联合环磷酰胺治疗。有完整随访资料者54例，30例缓解，16例长期血液透析，8例死亡。出现不良预后组确诊时的伯明翰血管炎活动性评分（BVAS）、血肌酐值高于未出现不良预后组，血红蛋白低于未出现不良预后组，系统受累发生率、肺间质病变发生率均高于未出现不良预后组，差异均有统计学意义（P < 0.05）。 结论 AAV好发于老年人，临床表现缺乏特异性且可累及多器官系统，以呼吸系统和泌尿系统常见。确诊时BVAS评分高、血肌酐高、血红蛋白低、重要脏器受累及肺间质病变是AAV患者预后不良的危险因素。

[关键词] 血管炎；抗中性粒细胞胞浆抗体；肾脏损害；预后

[中图分类号] R692.3 [文献标识码] A [文章编号] 1673-7210（2018）04（c）-0048-05

Clinical analysis of anti-neutrophil cytoplasmic antibodies associated with vasculitis in patients of kidney damage

LI Qianqian LI Shengkai

Department of Nephrology， the Affiliated Hospital of Xuzhou Medical University， Jiangsu Province， Xuzhou 221000， China

[Abstract] Objective To investigate the clinical characteristics and relevant factors of prognosis in patients with anti-neutrophil cytoplasmic antibodies （ANCA） associated with vasculitis （AAV）. Methods The clinical data of 65 patients of kidney damage with AAV in Department of Nephrology， the Affiliated Hospital of Xuzhou Medical University from August 2013 to August 2017 were retrospectively analyzed， including the general characteristics， systems involvement， auxiliary examinations and treatment outcomes. For patients with complete follow-up outcomes （54 cases）， they were divided into the poor prognosis group （24 cases） and the good prognosis group （30 cases） according to the different prognosis. The clinical characteristics and the auxiliary examinations were compared between the two groups. Results The average age of the patients was （65±12） years， the medium time of diagnosis was 1 （0.5-4.0） months. Multiple systems were damaged， especially in urinary system （65 cases） and respiratory system （45 cases）. CT scans showed that interstitial change was common， with 42 cases （42/65， 64.6%）. The blood test showed that anemia was 57 cases （57/65， 87.7%）. The C-reactive protein and the blood sedimentation were higher， with 57 cases （87.7%） and 58 cases （89.2%） respectively. ANCA was mainly MPO positive （60 cases， 92.3%）. Seven routine renal biopsies were performed， 4 were crescent glomerulonephritis and 3 were immunofluorescence negative. Fifity-five cases were treated with glucocorticoids and 47 cases treated with glucocorticoids combined with cyclophosphamide. There were 54 patients with complete follow-up data and showed that 30 cases were remission， 16 were treated with maintenance hemo？鄄dialysis and 8 were died. The scores of birmingham vasculitis activity scores （BVAS） and the serum creatinine in the poor prognosis group were all higher than those of good prognosis group， the hemoglobin was lower than that of good prognosis group， the incidences of systems damage and the lung interstitial change were all higher than those of good prognosis group， with statistically significant differences （P < 0.05）. Conclusion The AAV occurs predominantly in the elderly. Multi-system are involved with a variety of clinical manifestations， particularly in urinary system and respiratory system. It seems that higher BVAS score， lower hemoglobin level， heavier damage of the major organs and seriously lung interstitial change are associated with higher risk of the poor prognosis.

[Key words] Vasculitis； Anti-neutrophil cytoplasmic antibodies； Kidney damage； Prognosis

抗中性粒细胞胞浆抗体（ANCA）相关性血管炎（AAV）是一类多系统受累的自身免疫性小血管炎，中老年人多见，未经治疗1年内病死率达80%[1]。因ANCA被诱导激活后进一步活化补体引起炎性反应，导致小血管壁的炎症和纤维素样坏死而致病[2-3]，其主要靶抗原为蛋白酶3（PR3）和髓过氧化物酶（MPO）[4-5]。……