New phenotype and outcomes in connective tissue disease-associated pulmonary arterial hypertension: relevance of age-related comorbidities

Contemporary CTD-PAH patients included in REHAP are older and have a lower comorbidity burden than IPAH patients, while exhibiting similar survival rates up to 5 years. CKD is an independent prognostic factor in both aetiologies. CTD-PAH patients tend to receive a more conservative treatment approach.