Long-term patient survival in ANCA-associated vasculitis

Long-term patient survival in ANCA-associated vasculitis
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DOI:
10.1136/ard.2010.137778
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发表时间:
2011-03-01
影响因子:
27.4
通讯作者:
Westman, Kerstin
Westman, Kerstin
中科院分区:
医学1区
文献类型:
--
作者:
Flossmann, Oliver;Berden, Annelies;Westman, Kerstin

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背景韦格纳肉芽肿和显微镜下的多血管炎是抗中性粒细胞胞浆抗体(ANCA)相关的血管炎,发病率和死亡率都很高。目前治疗ANCA相关性脉管炎的患者的长期存活率尚不确定。目的在一个国际性、多中心、前瞻性招募的具有代表性的患者队列中描述患者的长期存活率和可能的预后因素,这些患者在呈现时按照严格定义的方案进行治疗,包括ANCA相关性血管炎的全部病程。方法收集1995-2002年间4个随机对照试验在诊断时招募的患者的结果数据。试验资格根据疾病的严重程度和范围进行定义,涵盖ANCA相关性血管炎的严重程度,并使用一致的诊断标准。结果中位随访时间为5.2年,死亡133例(25%)。与年龄和性别匹配的普通人群相比,死亡率为2.6(95%可信区间为2.2比3.1)。第一年内死亡的主要原因是感染(48%)和活动性脉管炎(19%)。第一年后的主要死因是心血管疾病(26%)、恶性肿瘤(22%)和感染(20%)。多变量分析显示估计的肾小球滤过率
Background Wegener's granulomatosis and microscopic polyangiitis are antineutrophil cytoplasm antibodies (ANCA)-associated vasculitides with significant morbidity and mortality. The long-term survival of patients with ANCA associated vasculitis treated with current regimens is uncertain.Objective To describe the long-term patient survival and possible prognostic factors at presentation in an international, multicentre, prospectively recruited representative patient cohort who were treated according to strictly defined protocols at presentation and included the full spectrum of ANCA-associated vasculitis disease.Methods Outcome data were collected for 535 patients who had been recruited at the time of diagnosis to four randomised controlled trials between 1995 and 2002. Trial eligibility was defined by disease severity and extent, covered the spectrum of severity of ANCA-associated vasculitis and used consistent diagnostic criteria. Demographic, clinical and laboratory parameters at trial entry were tested as potential prognostic factors in multivariable models.Results The median duration of follow-up was 5.2 years and 133 (25%) deaths were recorded. Compared with an age-and sex-matched general population there was a mortality ratio of 2.6 (95% CI 2.2 to 3.1). Main causes of death within the first year were infection (48%) and active vasculitis (19%). After the first year the major causes of death were cardiovascular disease (26%), malignancy (22%) and infection (20%). Multivariable analysis showed an estimated glomerular filtration rate