Advertisement

Pediatric Cardiology

, 32:1040 | Cite as

Successful Treatment with Bosentan for Pulmonary Hypertension and Reduced Peripheral Circulation in Juvenile Systemic Sclerosis

  • Masaki ShimizuEmail author
  • Yoko Hashida
  • Kazuyuki Ueno
  • Tadafumi Yokoyama
  • Yuko Nakayama
  • Takekatsu Saito
  • Kunio Ohta
  • Kazuhiko Takehara
  • Akihiro Yachie
Case Report

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.

Keywords

Bosentan Juvenile systemic sclerosis Pulmonary hypertension 

References

  1. 1.
    Galie N, Manes A, Branzi A (2004) The endothelin system in pulmonary arterial hypertension. Cardiovasc Res 61:227–237PubMedCrossRefGoogle Scholar
  2. 2.
    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
  3. 3.
    Maiya S, Hislop AA, Flynn Y et al (2006) Response to bosentan in children with pulmonary hypertension. Heart 92:664–670PubMedCrossRefGoogle Scholar
  4. 4.
    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
  5. 5.
    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
  6. 6.
    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
  7. 7.
    Rubin LJ, Badesch DB, Barst RJ et al (2002) Bosentan therapy for pulmonary arterial hypertension. N Engl J Med 346:896–903PubMedCrossRefGoogle Scholar
  8. 8.
    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
  9. 9.
    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

Copyright information

© Springer Science+Business Media, LLC 2011

Authors and Affiliations

  • Masaki Shimizu
    • 1
    Email author
  • Yoko Hashida
    • 1
  • Kazuyuki Ueno
    • 1
  • Tadafumi Yokoyama
    • 1
  • Yuko Nakayama
    • 1
  • Takekatsu Saito
    • 1
  • Kunio Ohta
    • 1
  • Kazuhiko Takehara
    • 2
  • Akihiro Yachie
    • 1
  1. 1.Department of Pediatrics, School of Medicine, Institute of Medical, Pharmaceutical and Health SciencesKanazawa UniversityKanazawaJapan
  2. 2.Department of Dermatology, School of Medicine, Institute of Medical, Pharmaceutical and Health SciencesKanazawa UniversityKanazawaJapan

Personalised recommendations