RT Journal Article SR Electronic T1 A new era in the management of pulmonary arterial hypertension related to scleroderma: endothelin receptor antagonism JF Annals of the Rheumatic Diseases JO Ann Rheum Dis FD BMJ Publishing Group Ltd and European League Against Rheumatism SP 1009 OP 1014 DO 10.1136/ard.2003.017673 VO 63 IS 9 A1 Hachulla, E A1 Coghlan, J G YR 2004 UL http://ard.bmj.com/content/63/9/1009.abstract AB 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.