Rheumatology International

, Volume 29, Issue 7, pp 769–775 | Cite as

Effects of bosentan on the skin lesions: an observational study from a single center in Japan

  • Masanori Funauchi
  • K. Kishimoto
  • H. Shimazu
  • Y. Nagare
  • S. Hino
  • T. Yano
  • K. Kinoshita
Original Article
First Online:
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Accepted:

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.

Keywords

Bosentan Connective tissue Sclerosis Modified Rodnan total skin score Raynaud’s phenomenon Digital ulcer Pulmonary arterial hypertension 
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Notes

Acknowledgments

A part of this study was supported by a grant from The Osaka Medical Research Foundation for Incurable Diseases.

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Copyright information

© Springer-Verlag 2008

Authors and Affiliations

  • Masanori Funauchi
    • 1
  • K. Kishimoto
    • 1
  • H. Shimazu
    • 1
  • Y. Nagare
    • 1
  • S. Hino
    • 1
  • T. Yano
    • 1
  • K. Kinoshita
    • 1
  1. 1.Department of Nephrology and RheumatologyKinki University School of MedicineOsakaJapan

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