PT - JOURNAL ARTICLE AU - E Hachulla AU - J G Coghlan TI - A new era in the management of pulmonary arterial hypertension related to scleroderma: endothelin receptor antagonism AID - 10.1136/ard.2003.017673 DP - 2004 Sep 01 TA - Annals of the Rheumatic Diseases PG - 1009--1014 VI - 63 IP - 9 4099 - http://ard.bmj.com/content/63/9/1009.short 4100 - http://ard.bmj.com/content/63/9/1009.full SO - Ann Rheum Dis2004 Sep 01; 63 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.