A retrospective study based on data from the Spanish pulmonary arterial hypertension Registry (REHAP) set out to compare the demographic and clinical profiles, comorbidity burden, and survival rates of patients with connective tissue disease-associated pulmonary arterial hypertension (CTD-PAH) against those with idiopathic pulmonary arterial hypertension (IPAH). The study, covering the period 2014–2023, included 757 patients in total.
While connective tissue disease-associated pulmonary arterial hypertension patients were older and had fewer comorbidities, their survival rates over five years were remarkably similar to idiopathic pulmonary arterial hypertension patients. The study also examined the impact of seven age-related comorbidities on survival outcomes. Of these, chronic kidney disease emerged as the only independent predictor of poor prognosis in both groups, even after adjusting for known pulmonary arterial hypertension prognostic factors.
Connective tissue disease-associated pulmonary arterial hypertension patients tended to receive more conservative treatment, with monotherapy used more frequently than in idiopathic pulmonary arterial hypertension.
The findings underline the importance of monitoring kidney function in all pulmonary arterial hypertension patients, regardless of underlying cause.
Citation
Rafael Bravo-Marqués, Francisco Rivas-Ruiz, Andrés Tenes, Víctor M Becerra-Muñoz, Eva Gutierrez-Ortiz, Irene Martin de Miguel, Joan A Barberà, Manuel López-Meseguer, Pedro Antonio Chinchurreta Capote, Isabel Blanco Vich, Pilar Escribano-Subias, the REHAP investigators , New phenotype and outcomes in connective tissue disease-associated pulmonary arterial hypertension: relevance of age-related comorbidities, Rheumatology, 2026;, keag105, https://doi.org/10.1093/rheumatology/keag105