A new era in the management of pulmonary arterial hypertension related to scleroderma: endothelin receptor antagonism. Issue 9 (12th August 2004)

• Record Type: Journal Article
• Authors: Hachulla, E; Coghlan, J G
• Is Part Of: Annals of the rheumatic diseases. Volume 63:Issue 9(2004)
• Page Start: 1009
• Page End: 1014
• Publication Date: 2004-08-12
• Subjects: 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; Rheumatism -- Periodicals; 616.723005
• DOI: 10.1136/ard.2003.017673
• Languages: English
• ISSNs: 0003-4967

Abstract

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.