@article {du Bois24, author = {R. M. du Bois}, title = {Blocking endothelin: breaking new ground}, volume = {16}, number = {102}, pages = {24--29}, year = {2007}, doi = {10.1183/09059180.00010206}, publisher = {European Respiratory Society}, abstract = {Endothelin is one of a number of profibrotic cytokines and growth factors, along with transforming growth factor-β, connective tissue growth factor and tumour necrosis factor-α, thought to be involved in the pathogenesis of idiopathic pulmonary fibrosis (IPF) and other interstitial lung diseases characterised by fibrosis, including IPF-related pulmonary hypertension. A growing body of evidence has supported a mitogenic effect of endothelin on fibroblasts and demonstrated that endothelin can reduce collagen breakdown and induce the synthesis of extracellular matrix components, all contributing to fibrosis. These findings, together with the detection of elevated levels of endothelin in the plasma and bronchoalveolar lavage fluid of patients, provide a sound rationale for dual endothelin receptor antagonism as a potential therapy for IPF and other fibrotic lung diseases. The randomised controlled trial of Bosentan Use in Interstitial Lung Disease (BUILD)-1 investigated the potential of bosentan for the treatment of idiopathic pulmonary fibrosis. Although bosentan did not improve exercise capacity, encouraging trends were seen in the pre-defined clinically relevant end-point of disease progression or death, as well as dyspnoea and quality of life measures. The potential efficacy of bosentan, a well-established pathogenic mediator in pulmonary arterial hypertension, will continue to be evaluated in the treatment of idiopathic pulmonary fibrosis.}, issn = {0905-9180}, URL = {https://err.ersjournals.com/content/16/102/24}, eprint = {https://err.ersjournals.com/content/16/102/24.full.pdf}, journal = {European Respiratory Review} }