Session Information
Session Type: Abstract Submissions (ACR)
Background/Purpose: To evaluate survival in rheumatoid arthritis associated pulmonary arterial hypertension (RA-PAH) compared to idiopathic PAH (IPAH) patients. Secondary outcomes included evaluation of differences in sex predisposition, age of diagnosis, disease severity, co-morbid diseases and pulmonary hypertension treatment.
Methods: A retrospective cohort study of RA-PAH and IPAH seen at the University Health Network Pulmonary Hypertension Programme, Toronto, Canada was conducted. The primary outcome was time to all cause mortality. Unadjusted survival was evaluated using Kaplan-Meier survival curves. Propensity score models were used to adjust for differences in baseline covariates, and to assemble a matched cohort. Cox proportional hazards models were used to estimate survival in the matched cohort.
Results: Screening of 1385 patients identified 18 RA-PAH and 155 IPAH patients. RA-PAH patients had a lower proportion of males (17% versus 30%), and had an older median age of onset (64.0 years versus 53.7 years). RA-PAH patients more frequently had coronary artery disease (33% versus 16%), lower baseline mPAP (43 mmHg versus 51 mmHg), lower proportion of WHO functional class III or IV (39% versus 52%), lower median baseline BNP (58.4 pg/mL versus 95.0 pg/mL), longer 6-minute walk distance (478 m versus 381 m), less calcium channel blocker (22% versus 29%), phophodiesterase-5 inhibitor (11% versus 19%) and prostaglandin analogues (6% versus 15%) use. There were 35 deaths, 2/18 (11%) RA-PAH patients and 33/155 (21%) IPAH patients. The unadjusted 1-year survival for RA-PAH patients was 93% and for IPAH was 94%. The unadjusted hazard ratio comparing RA-PAH to IPAH survival was 1.52 (95% CI 0.16, 2.76). In the matched cohort there were 7 deaths, 2/18 (11%) RA-PAH patients and 5/18 (28%) IPAH patients, with a hazard ratio of 1.53 (95% CI 0.15, 2.84).
Conclusion: PAH is a serious manifestation of RA. Despite milder disease at presentation, survival is comparable to IPAH.
Disclosure:
S. Sadeghi,
Canadian Rheumatology Association (CRA) Summer Studentship,
2;
J. T. Granton,
Support respirology program at the hospital foundation.,
9,
Pfizer support of research study via CIHR grant.,
9;
P. Akhavan,
None;
S. R. Johnson,
None.
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ACR Meeting Abstracts - https://acrabstracts.org/abstract/survival-in-rheumatoid-arthritis-associated-pulmonary-arterial-hypertension-is-comparable-to-idiopathic-pulmonary-arterial-hypertension/