Impact of Comorbidities on Treatments and Outcomes of Systemic Sclerosis–Associated Pulmonary Arterial Hypertension

Abstract

Aim: Treatment recommendations for systemic sclerosis–associated pulmonary arterial hypertension (SSc-PAH) have evolved from initial monotherapy to upfront combination therapy with agents, including endothelin receptor antagonists, phosphodiesterase-5 inhibitors and prostanoids. In the presence of comorbidities, such as heart and lung disease, some clinicians have favoured monotherapy due to concerns about worsening ventilation–perfusion mismatch. We sought to evaluate whether comorbidity burden impacts prescribing practices, quality of life and survival in SSc-PAH. Methods: We analysed prospectively collected data from participants recruited to the Australian Scleroderma Cohort Study (ASCS)..