Pulmonary arterial hypertension: screening challenges in systemic sclerosis and future directions

Extract

Despite the advent of multiple medical therapies for pulmonary arterial hypertension (PAH), there is currently no cure available for this disease which continues to impose a very heavy burden of morbidity and mortality on afflicted patients. Survival on medical therapy is at best 7 years in patients with idiopathic PAH [1] and significantly less in patients with associated PAH (APAH), such as those with connective tissue disease (CTD) and particularly with scleroderma-associated PAH (SSc-PAH), where median survival is about 4 years.