A new era in the management of pulmonary arterial hypertension related to scleroderma: endothelin receptor antagonism.
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.
Service de Médecine Interne, Hôpital Claude Huriez, Centre Hospitalier et Universitaire, 59037 Lille, France. ehachulla@chru-lille.fr
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Hachulla
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Coghlan
D000959:Antihypertensive Agents / Q000627:therapeutic use*; D000077300:Bosentan; D065128:Endothelin Receptor Antagonists; D016232:Endothelins / Q000502:physiology; D006801:Humans; D006976:Hypertension, Pulmonary / Q000188:drug therapy* / Q000209:etiology; D012595:Scleroderma, Systemic / Q000150:complications*; D013449:Sulfonamides / Q000627:therapeutic use
D000959:Antihypertensive Agents; D065128:Endothelin Receptor Antagonists; D016232:Endothelins; D013449:Sulfonamides; D000077300:Bosentan