title: Successful therapy of bosentan-refractory pulmonary arterial hypertension (PAH) with inhalative iloprost.
abstract: Pulmonary arterial hypertension (PAH) is a severe complication of systemic sclerosis. High vascular resistance in PAH arises from an imbalance between vasodilatory mediators (prostacyclin, NO) and vasoconstrictive mediators (endothelin, thromboxane A-2). Inhaled iloprost and the dual endothelin receptor antagonist bosentan have recently been shown to be effective in controlled clinical trials. Our case report demonstrates that patients with bosentan-refractory PAH can be successfully treated with iloprost inhalation.
authors.affiliation: Department of Rheumatology, University Hospital of Schleswig-Holstein, Campus Luebeck, and Rheumaklinik Bad Bramstedt, Luebeck, Germany. ahmadi@rheuma-zentrum.de
authors.forename: K
authors.initials: K
authors.lastname: Ahmadi-Simab
authors.forename: P
authors.initials: P
authors.lastname: Lamprecht
authors.forename: W L
authors.initials: WL
authors.lastname: Gross
mesh_terms: D000280:Administration, Inhalation; D000959:Antihypertensive Agents / Q000627:therapeutic use*; D000077300:Bosentan; D005080:Exercise Test / Q000187:drug effects; D005260:Female; D006439:Hemodynamics / Q000187:drug effects / Q000502:physiology; D006801:Humans; D006976:Hypertension, Pulmonary / Q000188:drug therapy* / Q000503:physiopathology; D016285:Iloprost / Q000008:administration & dosage*; D008875:Middle Aged; D011651:Pulmonary Artery / Q000473:pathology; D013449:Sulfonamides / Q000627:therapeutic use*; D016896:Treatment Outcome; D014665:Vasodilator Agents / Q000008:administration & dosage*; D016138:Walking
chemical_list: D000959:Antihypertensive Agents; D013449:Sulfonamides; D014665:Vasodilator Agents; D016285:Iloprost; D000077300:Bosentan