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.
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Galie N, Manes A, Branzi A (2004) The endothelin system in pulmonary arterial hypertension. Cardiovasc Res 61:227–237PubMedCrossRefGoogle Scholar
Launay D, Sitbon O, Le Pavec J et al (2010) Long-term outcome of systemic sclerosis-associated pulmonary arterial hypertension treated with bosentan as first-line monotherapy followed or not by the addition of prostanoids or sildenafil. Rheumatology 49:490–500PubMedCrossRefGoogle Scholar
Maiya S, Hislop AA, Flynn Y et al (2006) Response to bosentan in children with pulmonary hypertension. Heart 92:664–670PubMedCrossRefGoogle Scholar
Martini G, Foeldvari I, Russo R et al (2006) Systemic sclerosis in childhood: clinical and immunologic features of 153 patients in an international database. Arthritis Rheum 54:3971–3978PubMedCrossRefGoogle Scholar
Matucci-Cerinic M, Denton CP, Furst DE et al (2011) Bosentan treatment of digital ulcers related to systemic sclerosis: results from the RAPIDS-2 randomised, double-blind, placebo-controlled trial. Ann Rheum Dis 70:32–38PubMedCrossRefGoogle Scholar
Rosato E, Molinaro I, Borghese F et al (2010) Bosentan improves skin perfusion of hands in patients with systemic sclerosis with pulmonary arterial hypertension. J Rheumatol 37:2531–2539PubMedCrossRefGoogle Scholar
Rubin LJ, Badesch DB, Barst RJ et al (2002) Bosentan therapy for pulmonary arterial hypertension. N Engl J Med 346:896–903PubMedCrossRefGoogle Scholar
Russo RA, Katsicas MM (2007) Clinical characteristics of children with juvenile systemic sclerosis: follow-up of 23 patients in a single tertiary center. Pediatr Rheumatol Online J 5:6PubMedCrossRefGoogle Scholar
Yamane K, Miyauchi T, Suzuki N et al (1992) Significance of plasma endothelin-1 levels in patients with systemic sclerosis. J Rheumatol 19:1566–1571PubMedGoogle Scholar