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Bosentan in systemic sclerosis.

Gustavo A Heresi1, Omar A Minai

  • 1Respiratory Institute, Cleveland Clinic, Cleveland, OH 44195, USA.

Drugs of Today (Barcelona, Spain : 1998)
|July 4, 2008
PubMed
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Systemic sclerosis treatment with bosentan can improve survival in patients with pulmonary hypertension and reduce new digital ulcers. However, it is contraindicated in pregnancy and requires monitoring for liver transaminases.

Area of Science:

  • Rheumatology
  • Pulmonology
  • Dermatology

Background:

  • Systemic sclerosis (SSc) is a rare connective tissue disease with significant morbidity and mortality, primarily due to pulmonary complications and digital ulcers.
  • Endothelin-1 (ET-1) plays a key role in the vascular injury and fibrosis characteristic of SSc.
  • Limited treatment options have historically been available for SSc complications.

Purpose of the Study:

  • To evaluate the efficacy and safety of bosentan, an endothelin receptor antagonist, in managing SSc complications.
  • To assess bosentan's impact on pulmonary arterial hypertension, interstitial lung disease, and digital ulcers in SSc patients.

Main Methods:

  • Review of studies investigating bosentan therapy in patients with Systemic Sclerosis.
  • Analysis of bosentan's effects on exercise capacity, survival, and the incidence and healing of digital ulcers.

Related Experiment Videos

  • Assessment of bosentan's safety profile, including contraindications and adverse events like elevated liver transaminases.

    • Main Results:

    • Bosentan therapy demonstrated improved survival and prevented exercise capacity decline in SSc patients with pulmonary hypertension.
    • Bosentan did not show a beneficial effect in SSc patients with interstitial lung disease.
    • Bosentan effectively reduced the occurrence of new digital ulcers but did not accelerate the healing of existing ones.

    Conclusions:

    • Bosentan is a valuable therapeutic option for pulmonary hypertension in SSc and for preventing new digital ulcers.
    • Careful patient selection and monitoring are essential due to potential adverse effects and contraindications, particularly pregnancy and liver function.
    • Further research may explore optimizing bosentan use or alternative therapies for specific SSc manifestations like interstitial lung disease.