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.
Abstract
Screening for PAH is significant in scleroderma as it may lead to early diagnosis and initiation of therapy http://ow.ly/TR6230aqRgq
Footnotes
Conflict of interest: None declared.
- Received March 13, 2017.
- Accepted March 14, 2017.
- Copyright ©ERS 2017