Article Text
PDFReview
A new era in the management of pulmonary arterial hypertension related to scleroderma: endothelin receptor antagonism
Abstract
Evidence suggests that endothelin may have a fundamental role in scleroderma pathogenesis, including pulmonary arterial hypertension (PAH)—a leading cause of death in patients with scleroderma. Development of a new class of drug, endothelin receptor antagonists, heralds an improved outlook for patients with scleroderma and related diseases. Heightened vigilance towards early detection of PAH in scleroderma and a multidisciplinary approach to diagnosis and treatment may improve clinical outcomes for these patients.
- CCBs, calcium channel blockers
- ERA, endothelin receptor antagonist
- FVC, forced vital capacity
- ILD, interstitial lung disease
- mPAP, mean pulmonary arterial pressure
- PAH, pulmonary arterial hypertension
- Tlco, carbon monoxide transfer factor
- VSMC, vascular smooth muscle cells
- scleroderma
- pulmonary arterial hypertension
- diagnosis
- endothelin receptor antagonists