Pulmonary arterial hypertension (PAH) is a common complication of connective tissue diseases (CTD) and a leading cause of death in this population. Despite significant advances in therapy for PAH, particularly for idiopathic PAH (IPAH), the response to treatment in patients with CTD associated with PAH, mainly in sclerodermaassociated PAH (SSc-PAH), has been quite disappointing. This chapter reviews the epidemiology, clinical manifestations, pathophysiology and currently available therapies for CTDassociated PAH with a particular focus on SSc-PAH, animal models of disease, and newly identified potential targets for therapy for this devastating syndrome.
- Connective tissue disease
- Pulmonary arterial hypertension
- Systemic sclerosis
ASJC Scopus subject areas
- Pulmonary and Respiratory Medicine