title: Successful treatment with bosentan for pulmonary hypertension and reduced peripheral circulation in juvenile systemic sclerosis.
abstract: Pulmonary arterial hypertension (PAH) when associated with systemic sclerosis (SSc) (SSc-PAH) is one of the leading causes of mortality and is found in 10-15% of adult patients with SSc. The ET receptor antagonist bosentan has been shown to be effective in the treatment of adult patients with SSc-PAH. Furthermore, it has been shown that bosentan ameliorates decreased skin perfusion and digital ulceration secondary to SSc. However, the effectiveness and safety of bosentan for treatment of juvenile SSc still remains unclear. We describe a case of juvenile SSc-PAH successfully treated with bosentan. The present case shows that bosentan ameliorated PAH and peripheral circulation as evaluated by cold stress thermography. No bosentan-related adverse events such as liver dysfunction were observed. Prospective randomized trials are required to validate the effectiveness of bosentan for patients with juvenile SSc; however, bosentan might be useful for the management of patients with juvenile SSc.
authors.affiliation: Department of Pediatrics, School of Medicine, Institute of Medical, Pharmaceutical and Health Sciences, Kanazawa University, Kanazawa, Japan. shimizum@staff.kanazawa-u.ac.jp
authors.forename: Masaki
authors.initials: M
authors.lastname: Shimizu
authors.forename: Yoko
authors.initials: Y
authors.lastname: Hashida
authors.forename: Kazuyuki
authors.initials: K
authors.lastname: Ueno
authors.forename: Tadafumi
authors.initials: T
authors.lastname: Yokoyama
authors.forename: Yuko
authors.initials: Y
authors.lastname: Nakayama
authors.forename: Takekatsu
authors.initials: T
authors.lastname: Saito
authors.forename: Kunio
authors.initials: K
authors.lastname: Ohta
authors.forename: Kazuhiko
authors.initials: K
authors.lastname: Takehara
authors.forename: Akihiro
authors.initials: A
authors.lastname: Yachie
mesh_terms: D000959:Antihypertensive Agents / Q000008:administration & dosage / Q000627:therapeutic use*; D000077300:Bosentan; D002648:Child; D004305:Dose-Response Relationship, Drug; D004452:Echocardiography; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D006976:Hypertension, Pulmonary / Q000188:drug therapy* / Q000209:etiology / Q000503:physiopathology; D011669:Pulmonary Wedge Pressure / Q000187:drug effects*; D012595:Scleroderma, Systemic / Q000150:complications* / Q000503:physiopathology; D013449:Sulfonamides / Q000008:administration & dosage / Q000627:therapeutic use*; D013817:Thermography; D014655:Vascular Resistance
chemical_list: D000959:Antihypertensive Agents; D013449:Sulfonamides; D000077300:Bosentan