title: [Long-term therapy with endothelin-1 antagonist for pulmonary hypertension secondary to systemic sclerosis].
abstract: Pulmonary hypertension in systemic sclerosis appears in up to 35% of patients, is characterized by increased pulmonary artery resistance, and carries a poorer prognosis than in patients with other causes of pulmonary hypertension. The recommended therapy for stage III disease, based on clinical trials and by the Israeli Ministry of Health for 2006, includes bosentan (Tracleer), an endothelin-1 antagonist. This is a case report of a patient with severe pulmonary hypertension secondary to systemic sclerosis where therapy with prostacyclins was contraindicated due to the development of congestive heart failure and severe atherosclerosis. The patient responded well to bosentan monotherapy for 4 years until his death.
authors.affiliation: Department of Medicine C, Wolfson Medical Center, Tel Aviv University, Israel.
authors.forename: Lilach
authors.initials: L
authors.lastname: Shalev
authors.forename: Pnina
authors.initials: P
authors.lastname: Langevitz
authors.forename: Gisele
authors.initials: G
authors.lastname: Zandman-Goddard
mesh_terms: D000959:Antihypertensive Agents / Q000627:therapeutic use*; D000077300:Bosentan; D019332:Endothelin-1 / Q000037:antagonists & inhibitors*; D017809:Fatal Outcome; D006801:Humans; D006976:Hypertension, Pulmonary / Q000188:drug therapy* / Q000209:etiology; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D012595:Scleroderma, Systemic / Q000150:complications* / Q000503:physiopathology; D013449:Sulfonamides / Q000627:therapeutic use*
chemical_list: D000959:Antihypertensive Agents; D019332:Endothelin-1; D013449:Sulfonamides; D000077300:Bosentan