Safety and Efficacy Study of Bosentan in Progressive Pulmonary Sarcoidosis
Status:
Terminated
Trial end date:
2010-03-01
Target enrollment:
Participant gender:
Summary
Progressive pulmonary sarcoidosis occurs in up to twenty percent of patients who require
persistent treatment, but available treatment options have shown considerable long-term
toxicity and uncertain or unproven efficacy. In these patients, pulmonary fibrosis and
pulmonary hypertension are common complications which have major prognostic impact.
Endothelin-1 (ET-1) has been demonstrated to play a key role in pulmonary fibrosis and
pulmonary hypertension, and a potential role in pulmonary sarcoidosis. ET-1 is a potent
vasoconstrictor and can promote fibrosis, cell proliferation, and remodeling, and is
pro-inflammatory. Preliminary data have shown the therapeutic potential of the endothelin
receptor antagonist (ERA) bosentan in sarcoidosis associated pulmonary hypertension.
In this light, the therapeutic potential of bosentan as an add-on treatment in progressive
pulmonary sarcoidosis needs to be evaluated.