Patients with non-Jo-1 anti-tRNA-synthetase autoantibodies have worse survival than Jo-1 positive patients.

Patients with non-Jo-1 anti-tRNA-synthetase autoantibodies have worse survival than Jo-1 positive patients.
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DOI:
10.1136/annrheumdis-2012-201800
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发表时间:
2014-01
影响因子:
27.4
通讯作者:
Oddis CV
Oddis CV
中科院分区:
医学1区
文献类型:
--
作者:
Aggarwal R;Cassidy E;Fertig N;Koontz DC;Lucas M;Ascherman DP;Oddis CV

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比较Jo-1和非Jo-1抗tRNA合成酶自身抗体(抗SynAb)患者的累积生存期和无事件生存期。从1985年到2009年初步评估的抗联体抗体阳性患者被纳入,而不考虑结缔组织病(CTD)的诊断。临床数据提取自前瞻性收集的数据库和图表回顾。采用对数秩法和COX比例风险法比较Jo-1和非Jo-1患者的生存率。抗突触抗体阳性202例,其中Jo-1 122例,非Jo-1 80例(PL-12 35例,PL-7 25例,EJ 9例,KS 6例,OJ 5例)。Jo-1和非Jo-1患者初诊诊断分别为肌炎83%和40.0%,重叠或未分化CTD分别为17%和47.5%,系统性硬化症分别为0%和12.5%(p<0.001)。Jo-1患者的诊断延迟中位数为0.4年,而非Jo-1患者的中位延迟时间为1.0年(p<0.001)。在整个队列中最常见的死亡原因是肺纤维化(49%)和肺动脉高压(11%)。Jo-1患者的5年和10年未调整累积生存率分别为90%和70%,而非Jo-1患者分别为75%和47%(p<0.005)。与Jo-1患者相比,非Jo-1患者的累积风险比(HR)为1.9(p=0.01),诊断后无事件生存的风险比(HR)为1.9(p=0.008)。首次诊断年龄和延迟诊断影响生存率,但与性别、种族和CTD诊断无关。与Jo-1患者相比,非Jo-1抗SynAb阳性患者的存活率降低。存活率的差异可能部分归因于非Jo-1患者的诊断延迟。
To compare the cumulative survival and event free survival in patients with Jo-1 versus non-Jo-1 anti-tRNA synthetase autoantibodies (anti-synAb). Anti-synAb positive patients initially evaluated from 1985 to 2009 were included regardless of the connective tissue disease (CTD) diagnosis. Clinical data were extracted from a prospectively collected database and chart review. Survival between Jo-1 and non-Jo-1 was compared by log rank and Cox proportional hazards methods. 202 patients possessed anti-synAb: 122 Jo-1 and 80 non-Jo-1 (35 PL-12; 25 PL-7; 9 EJ; 6 KS; 5 OJ). The diagnoses at first visit for Jo-1 and non-Jo-1 patients were myositis in 83% and 40.0%, overlap or undifferentiated CTD in 17% and 47.5%, and systemic sclerosis in 0% and 12.5%, respectively (p<0.001). The median delay in diagnosis was 0.4 years in Jo-1 patients versus 1.0 year in non-Jo-1 patients (p<0.001). The most common causes of death in the overall cohort were pulmonary fibrosis in 49% and pulmonary hypertension in 11%. The 5- and 10-year unadjusted cumulative survival was 90% and 70% for Jo-1 patients, and 75% and 47% for non-Jo-1 patients ( p<0.005). The hazard ratio (HR) of non-Jo-1 patients compared with Jo-1 patients was 1.9 (p=0.01) for cumulative and 1.9 (p=0.008) for event free survival from diagnosis. Age at first diagnosis and diagnosis delay but not gender, ethnicity and CTD diagnosis influenced survival. Non-Jo-1 anti-synAb positive patients have decreased survival compared with Jo-1 patients. The difference in survival may be partly attributable to a delay in diagnosis in the non-Jo-1 patients.
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