Pulmonary arterial hypertension (PAH), a common complication of systemic sclerosis, carries a very severe prognosis and is one of the leading causes of death in patients who suffer from it. Indeed, response to modern medical therapy has been disappointing in scleroderma-related PAH compared with other forms of PAH from the WHO group 1 classification of diseases, despite similar histological changes involving the pulmonary vasculature. This review discusses specific features of scleroderma-related PAH, currently available and US FDA-approved therapy for this syndrome, as well as potential future therapeutic developments based on newly acquired knowledge of this disorder.
- Endothelin receptor antagonist
- Phosphodiesterase inhibitor
- Pulmonary arterial hypertension
ASJC Scopus subject areas
- Immunology and Allergy
- Pulmonary and Respiratory Medicine
- Public Health, Environmental and Occupational Health