Sadeghi Saghar, Granton John T, Akhavan Pooneh, Pasarikovski Christopher R, Roos Adrienne M, Thenganatt John, Moric Jakov, Johnson Sindhu R
Division of Rheumatology, Department of Medicine, Toronto Western Hospital, Mount Sinai Hospital, Toronto, Ontario, Canada.
Respirology. 2015 Apr;20(3):481-7. doi: 10.1111/resp.12464. Epub 2015 Jan 13.
In this study, we evaluated survival in rheumatoid arthritis-associated pulmonary arterial hypertension (RA-PAH) compared with idiopathic pulmonary arterial hypertension (IPAH) patients, and evaluate differences in disease severity and treatment.
We conducted a retrospective cohort study of RA-PAH and IPAH at the University Health Network Pulmonary Hypertension Programme, Toronto, Canada. The primary outcome was time to all-cause mortality. We evaluated survival using Kaplan-Meier curves. Using a propensity score-matched cohort, we used Cox proportional hazards models to estimate survival.
Screening 1385 patients identified 18 RA-PAH and 155 IPAH patients. RA-PAH patients had an older median age of onset (64.0 vs 53.7 years) and lower baseline mean pulmonary arterial pressure (mPAP) (41 vs 50 mm Hg, P = 0.02). RA-PAH patients tended to have a higher proportion of females (83% vs 70%, relative risk 0.55, 95% confidence interval (CI): 0.19-1.57), lower proportion with baseline World Health Organization functional class III/IV (39% vs 52%), lower median baseline brain natriuretic peptide (58.4 vs 95.0 pg/mL) and longer baseline 6-min walk distance (440 vs 397 m). There were 35 deaths, 2/18 (11%) RA-PAH patients and 33/155 (21%) IPAH patients. The unadjusted 1-year survival was 93% for RA-PAH and 94% for IPAH. In the matched cohort, there were seven deaths: 2/18 (11%) RA-PAH and 5/18 (28%) IPAH patients, hazard ratio 1.53 (95% CI: 0.15-2.84). Separation of survival curves did not achieve statistical significance, log-rank 0.56.
Compared with IPAH patients, RA-PAH patients have an older age of onset and lower baseline mPAP. RA-PAH patients have comparable survival to IPAH patients.
在本研究中，我们评估了类风湿关节炎相关肺动脉高压（RA-PAH）患者与特发性肺动脉高压（IPAH）患者的生存率，并评估疾病严重程度和治疗方面的差异。
我们在加拿大多伦多大学健康网络肺动脉高压项目中对RA-PAH和IPAH患者进行了一项回顾性队列研究。主要结局是全因死亡时间。我们使用Kaplan-Meier曲线评估生存率。通过倾向评分匹配队列，我们使用Cox比例风险模型来估计生存率。
筛查1385例患者后，确定了18例RA-PAH患者和155例IPAH患者。RA-PAH患者的中位发病年龄较大（64.0岁对53.7岁），基线平均肺动脉压（mPAP）较低（41对50 mmHg，P = 0.02）。RA-PAH患者中女性比例往往较高（83%对70%，相对风险0.55，95%置信区间（CI）：0.19 - 1.57），基线时世界卫生组织功能分级为III/IV级的比例较低（39%对52%），基线脑钠肽中位数较低（58.4对95.0 pg/mL）以及基线6分钟步行距离较长（440对397 m）。共有35例死亡，2/18（11%）例RA-PAH患者和33/155（21%）例IPAH患者。RA-PAH患者未经调整的1年生存率为93%，IPAH患者为94%。在匹配队列中，有7例死亡：2/18（11%）例RA-PAH患者和5/18（28%）例IPAH患者，风险比为1.53（95% CI：0.15 - 2.84）。生存曲线的分离未达到统计学显著性，对数秩检验为0.56。
与IPAH患者相比，RA-PAH患者发病年龄较大且基线mPAP较低。RA-PAH患者与IPAH患者的生存率相当。
