title: Effects of bosentan on the skin lesions: an observational study from a single center in Japan.
abstract: Effects of a dual endothelin receptor antagonist, bosentan on peripheral circulatioin and skin lesions as well as pulmonary arterial hypertension (PAH) were investigated in Japanese patients with connective tissue diseases (CTD). Fifteen patients with PAH associated with CTD [systemic sclerosis (SSc) 13, mixed connective tissue disease (MCTD) 2] were treated with bosentan for 40-96 weeks, and changes of exercise capacity (6-min walk distance and Borg's dyspnea scale), cardio-pulmonary hemodynamics (right ventricular pressure, specific activity scale and cardiac index), Raynaud's phenomenon, digital ulcers and dermal sclerosis were observed. Bosentan improved exercise capacity, had a positive effect on hemodynamic parameters, and was well tolerated as previously reported. After a median 8 weeks of treatment, 13 out of 15 patients had improved Raynaud's phenomenon. Digital ulcers also improved after a median 12 weeks' treatment in all of 8 patients. Modified Rodnan total skin score decreased from 21.0 +/- 5.9 to 11.5 +/- 3.9 in diffuse cutaneous SSc and from 17.0 +/- 6.5 to 9.5 +/- 4.5 in limited cutaneous SSc after 24 months' treatment, reaching significance after 6 months in both groups. These data suggest that bosentan is effective for both PAH and peripheral vascular diseases in Japanese patients with CTD. The pathological background to the improvement in dermal sclerosis observed in this study should be further investigated.
authors.affiliation: Department of Nephrology and Rheumatology, Kinki University School of Medicine, 377-2 Ohnohigashi, Osaka-Sayama, Osaka, 589-8511, Japan. mn-funa@med.kindai.ac.jp
authors.forename: Masanori
authors.initials: M
authors.lastname: Funauchi
authors.forename: K
authors.initials: K
authors.lastname: Kishimoto
authors.forename: H
authors.initials: H
authors.lastname: Shimazu
authors.forename: Y
authors.initials: Y
authors.lastname: Nagare
authors.forename: S
authors.initials: S
authors.lastname: Hino
authors.forename: T
authors.initials: T
authors.lastname: Yano
authors.forename: K
authors.initials: K
authors.lastname: Kinoshita
mesh_terms: D000328:Adult; D000368:Aged; D000959:Antihypertensive Agents / Q000008:administration & dosage* / Q000009:adverse effects; D001158:Arteries / Q000187:drug effects / Q000276:immunology / Q000503:physiopathology; D000077300:Bosentan; D003240:Connective Tissue Diseases / Q000150:complications / Q000188:drug therapy* / Q000503:physiopathology; D017079:Exercise Tolerance / Q000187:drug effects / Q000502:physiology; D005260:Female; D006801:Humans; D006976:Hypertension, Pulmonary / Q000188:drug therapy* / Q000276:immunology / Q000503:physiopathology; D007564:Japan; D008297:Male; D008875:Middle Aged; D008947:Mixed Connective Tissue Disease / Q000150:complications / Q000188:drug therapy / Q000503:physiopathology; D011446:Prospective Studies; D011651:Pulmonary Artery / Q000187:drug effects / Q000276:immunology / Q000503:physiopathology; D011928:Raynaud Disease / Q000188:drug therapy / Q000276:immunology / Q000503:physiopathology; D012595:Scleroderma, Systemic / Q000150:complications / Q000188:drug therapy / Q000503:physiopathology; D012871:Skin Diseases / Q000188:drug therapy* / Q000276:immunology / Q000503:physiopathology; D013449:Sulfonamides / Q000008:administration & dosage* / Q000009:adverse effects; D014456:Ulcer / Q000188:drug therapy / Q000276:immunology / Q000503:physiopathology; D014657:Vasculitis / Q000188:drug therapy* / Q000276:immunology / Q000503:physiopathology
chemical_list: D000959:Antihypertensive Agents; D013449:Sulfonamides; D000077300:Bosentan