非 Jo-1 抗 tRNA 合成酶自身抗体阳性的患者比 Jo-1 阳性患者的生存率更差。
Patients with non-Jo-1 anti-tRNA-synthetase autoantibodies have worse survival than Jo-1 positive patients.
机构信息
Department of Medicine, Division of Rheumatology, University of Pittsburgh School of Medicine, , Pittsburgh, Pennsylvania, USA.
出版信息
Ann Rheum Dis. 2014 Jan;73(1):227-32. doi: 10.1136/annrheumdis-2012-201800. Epub 2013 Feb 19.
OBJECTIVE
To compare the cumulative survival and event free survival in patients with Jo-1 versus non-Jo-1 anti-tRNA synthetase autoantibodies (anti-synAb).
METHODS
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.
RESULTS
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.
CONCLUSIONS
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.
目的
比较抗 tRNA 合成酶自身抗体（抗-synAb）阳性的 Jo-1 患者与非 Jo-1 患者的累积生存率和无事件生存率。
方法
纳入了 1985 年至 2009 年期间最初评估的抗-synAb 阳性患者，无论结缔组织病（CTD）诊断如何。从前瞻性收集的数据库和病历回顾中提取临床数据。通过对数秩和 Cox 比例风险方法比较 Jo-1 和非 Jo-1 之间的生存情况。
结果
202 例患者具有抗-synAb：122 例 Jo-1 和 80 例非 Jo-1（35 例 PL-12；25 例 PL-7；9 例 EJ；6 例 KS；5 例 OJ）。Jo-1 和非 Jo-1 患者初次就诊时的诊断分别为肌炎 83%和 40.0%，重叠或未分化 CTD 17%和 47.5%，系统性硬化症 0%和 12.5%（p<0.001）。Jo-1 患者的中位诊断延迟为 0.4 年，而非 Jo-1 患者为 1.0 年（p<0.001）。总体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 患者的累积和无事件生存率的风险比（HR）分别为 1.9（p=0.01）和 1.9（p=0.008）。首次诊断时的年龄和诊断延迟，但不是性别、种族和 CTD 诊断，影响了生存率。
结论
与 Jo-1 患者相比，非 Jo-1 抗-synAb 阳性患者的生存率降低。生存差异部分可能归因于非 Jo-1 患者的诊断延迟。
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