1. Survival in rheumatoid arthritis-associated pulmonary arterial hypertension compared with idiopathic pulmonary arterial hypertension.
- Author
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Sadeghi S, Granton JT, Akhavan P, Pasarikovski CR, Roos AM, Thenganatt J, Moric J, and Johnson SR
- Subjects
- Adult, Aged, Familial Primary Pulmonary Hypertension mortality, Familial Primary Pulmonary Hypertension physiopathology, Female, Humans, Hypertension, Pulmonary etiology, Hypertension, Pulmonary physiopathology, Male, Middle Aged, Ontario epidemiology, Proportional Hazards Models, Pulmonary Wedge Pressure, Retrospective Studies, Survival Rate trends, Arthritis, Rheumatoid complications, Hypertension, Pulmonary mortality
- Abstract
Background and Objective: 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., Methods: 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., Results: 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., Conclusions: 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., (© 2015 Asian Pacific Society of Respirology.)
- Published
- 2015
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