269例抗中性粒细胞胞浆抗体相关性血管炎患者的临床病理特征及预后  

Clinical characteristics and prognosis in 269 patients with antineutrophil cytoplasimc antibody associated vasculitis

作　　者：伍婷 钟永[1] 周亚欧[2] 陈金彪[3] 杨映强 唐荣[1] 孟婷[1] 肖舟[1] 肖湘成[1] 周巧玲[1] 肖平[1] WU Ting;ZHONG Yong;ZHOU Ya’ou;CHEN Jinbiao;YANG Yingqiang;TANG Rong;MENG Ting;XIAO Zhou;XIAO Xiangcheng;ZHOU Qiaoling;XIAO Ping(Department of Nephrology,Xiangya Hospital,Central South University,Changsha 410008;Department of Rheumatology and Immunology,Xiangya Hospital,Central South University,Changsha 410008;Department of Medical Records&Information,Xiangya Hospital,Central South University,Changsha 410008,China)

机构地区：[1]中南大学湘雅医院肾内科,长沙410008 [2]中南大学湘雅医院风湿免疫科,长沙410008 [3]中南大学湘雅医院病案信息科,长沙410008

出　　处：《中南大学学报（医学版）》2020年第8期916-922,共7页Journal of Central South University ：Medical Science

基　　金：国家自然科学基金(81800641);湖南省自然科学基金(2018JJ3853)。

摘　　要：目的:了解抗中性粒细胞胞浆抗体(antineutrophil cytoplasimc antibody,ANCA)相关性血管炎(ANCAassociated vasculitis,AAV)的临床病理特征、预后及其影响因素。方法:回顾性分析中南大学湘雅医院2010年1月至2018年1月新诊断为AAV的269例住院患者的基本资料、临床病理数据,定期随访肾存活及患者存活情况,并对其临床病理及预后数据进行统计学分析及预后因素分析。结果:在269例AAV患者中,显微镜下多血管炎(microscopic polyangiitis,MPA)225例,占83.64%;肉芽肿性多血管炎(granulomatosis with polyangiitis,GPA)33例,占12.27%;嗜酸性肉芽肿性多血管炎(eosinophilic granulomatosis with polyangiitis,EGPA)11例,占4.09%。在MPA患者中,男女比例约为1:1,而GPA和EGPA患者中男性明显多于女性。从发病到确诊的中位时间为64.0 d,30 d内确诊65例,占24.16%。94.67%的MPA患者肾受累,显著高于GPA患者(63.63%),GPA累及耳鼻喉系统更常见(42.42%)。肾1年生存率在GPA和MPA患者中分别为75%和59.3%,1年病死率分别为16.7%和16.9%。多因素logistic回归分析显示:低血小板、低球蛋白血症、低免疫球蛋白G及血清肌酐升高是AAV患者1年肾生存率的危险因素,而血清肌酐升高是AAV患者全因死亡的独立危险因素。结论:AAV患者以MPA为主,患者进展为终末期肾病比例和病死率仍较高。早期诊断和及时治疗对改善AAV患者预后至关重要。Objective:To investigate the clinic-pathological characteristics,prognosis and its risk factors for antineutrophil cytoplasimc antibody(ANCA)-associated vasculitis(AAV).Methods:The basic information and clinic-pathological characteristics of AAV patients,who was diagnosed from January 2010 to January 2018 in Xiangya Hospital,Central South University,were retrospectively collected.The renal survival and patient survival were regular followed up,and their clinical pathological,prognosis data and risk factors for renal were analyzed.Results:Among 269 AAV patients,225 patients(83.64%)were microscopic polyangiitis(MPA),33 patients(12.27%)were granulomatosis with polyangiitis(GPA),and 11 patients(4.09%)were eosinophilic granulomatosis with polyangiitis(EGPA).Male-to-female ratio was almost 1∶1 for MPA(147/122),but GPA and EGPA was more frequent among man.The medium time from disease onset to diagnosis was 64.0(32.5,148.5)days,65 patients(24.16%)were diagnosed within 30 days.A total of 94.67%patients had kidney involvement in MPA patients,which was significantly higher than that in patients with GPA(63.63%),but ear,nose,and throat manifestations were more frequent in GPA patients.The 1-year kidney survival rates of GPA and MPA patients in this study were 75%and 59.3%,respectively.The 1-year death rates were 16.7%and 16.9%,respectively.The multivariable logistic regression analysis revealed that the low platelet level,low globulin level,low immunoglobin G,and high serum creatinine level were independent risk factors for the 1-year renal survival rate in AAV patients.Multiple factor logistic regression analysis showed that the serum creatinine level was an independent risk factor for all-cause mortality.Conclusion:MPA is the priority in the AAV patients.The proportion of patients with advanced end-stage renal disease and mortality is still high,and the early diagnosis and treatment in time is crucial for the improvement of prognosis for AAV.

关 键 词：血管炎 抗中性粒细胞胞浆抗体 临床特征 预后 

分 类 号：R593.2[医药卫生—内科学]