EDITORIAL

The endothelin system in pulmonary arterial hypertension

The endothelin system and its role in pulmonary arterial hypertension (PAH)

J Pepke-Zaba¹, N W Morrell²

¹ Papworth Hospital, Cambridge, UK
² University of Cambridge School of Clinical Medicine, Addenbrooke’s and Papworth Hospitals, Cambridge, UK

Correspondence to:
Correspondence to:
Dr J Pepke-Zaba
Pulmonary Vascular Diseases Unit, Papworth Hospital, Cambridge CB3 8RE, UK; jjm.zaba@ntlworld.com

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Endothelin receptor antagonists represent a major advance in the treatment of PAH but much remains to be learned of their effectiveness in specific forms of pulmonary hypertension

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Keywords: endothelin receptor antagonists; pulmonary arterial hypertension; bosentan

The first 150 words of the full text of this article appear below.

The endothelin system is emerging as an important mediator in pulmonary arterial hypertension (PAH) and the endothelin receptor antagonists represent a major advance in the treatment of this condition. PAH results from a massive proliferation of myofibroblast cells in the intima of small pulmonary arteries. Thickening of the media is also observed and abnormal proliferation of endothelial cells may result in plexiform lesions.

The endothelin system has been extensively studied over the 15 years since its initial discovery by Yanagisawa and co-workers in 1988. It is clear that endothelin 1 (ET-1) is a key mediator of pulmonary vascular biology and pathophysiology. It is a powerful vasoconstrictor and proliferative cytokine. In the early 1990s ET-1 was identified as an important mediator of PAH. Plasma ET-1 levels were found to be elevated in patients with pulmonary hypertension and correlated with the raised pulmonary vascular resistance.¹ . . . [Full text of this article]

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