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目的 通过对抗中性粒细胞胞质抗体(ANCA)相关性血管炎(AAV)死亡患者的临床特点进行分析,探索其死亡危险因素,为今后早期诊治、降低病死率及改善预后提供依据.方法 回顾性分析2003年11月至2017年2月收住解放军总医院第一附属医院确诊的AAV患者,对比死亡组和对照组患者在各项临床特点、实验室检查指标的异同,寻找影响其死亡的危险因素.结果 (1)66例AAV患者中死亡组20例(男:女为12∶8),总病死率30.3％;(2)死亡组平均发病年龄67岁,显著高于对照组的55岁(P =0.009);(3)死亡组血管炎损害指数(VDI)均值6.4,显著高于对照组的4.4(P =0.006);(4)从心、肺、肾、胃肠道、中枢神经系统等重要脏器受累的表现分析,3个及以上器官受累者,死亡组所占比例为85％,显著高于对照组的47.8％ (P =0.004 8).其中,心脏瓣膜杂音、新近发生早搏、主动脉瓣关闭不全和慢性心衰/心肌病以及大咯血等的发生率均显著高于对照组(P＜0.05);(5)死亡组感染发生率显著高于对照组(55％ vs 28.3％,P=0.038).结论 发病年龄偏晚、多脏器受累,发生大咯血、心瓣膜病变及心衰等表现,VDI增高及合并感染均是AAV患者死亡的危险因素.

Objectives Death risk factors of patients with antineutrophil cytoplasmic antibody (ANCA) associated vasculitis (AAV) were explored by the analysis of clinical characteristics of AAV patients,as to provide the basis for early diagnosis and treatment,and reduction of mortality and also improvement of prognosis.Methods A retrospective study was conducted in patients with AAV which were admitted to this hospital from November 2003 to February 2017,by the contrasts of the similarities and differences of clinical characteristics between the death group and non-death group,for explore the risk factors of death.Results (1) A total of 66 patients with AAV were included in this study,in which 20 were died (male/female was 12/8),and 46 were still alive,with a total mortality rate of 30.3％.(2) The average age of disease onset in the death group was (67 ± 13) years,which was significantly higher than that of the control group (55 ± 18,P =0.009).(3)The mean value of vasculitis damage index (VDI) in the death group was (6.4 ± 2.5),which was significantly higher than that in the non-death group (4.4 ± 2.5,P =0.006).(4)As to multiple organs involvements among the heart,lung,kidney,gastrointestinal tract,central nervous system and other organs,the proportion of three or more organs involvement in the death group was 85％ (17/20),which was significantly higher than that in the control group 47.8％ (22/46),P =0.004 8.The incidence of heart murmurs,recent premature beats,aortic insufficiency,chronic heart failure/cardiomyopathy,and massive hemoptysis were significantly higher than those in the control group (P ＜0.05).(5) The incidence of infection in the death group (55％) was significantly higher than that in the control group (28.3％,P =0.038).Conclusion An onset age of more than 65,multiple organs involvement,especially the occurrence of massive hemoptysis,heart valve diseases,heart failure and other cardiovascular involvements,increased VDI and combination of infections are the risk factors of death in AAV patients.