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PAH in CTD – Clinical Trials Criteria and Performance

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Advances in Vascular Medicine

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Abstract

Pulmonary arterial hypertension (PAH) is characterized by vascular proliferation and remodeling ultimately leading to right ventricular failure and death. PAH has emerged in recent years as the leading cause of death and late disease morbidity in systemic sclerosis and is an important complication of other connective tissue disorders. Pulmonary endothelial injury is associated with reduced production of vasodilating substances such as prostacyclin and nitric oxide and increased production of vasoconstrictive and proproliferative endothelin. Short term controlled trials have suggested benefits of endothelin receptor antagonism including improved exercise capacity and hemodynamics and increased time to clinical worsening. Improved survival is suggested from long term open label observational cohorts. As therapeutic options increase, it is important to consider the validity of outcome measures and the relative merits of endothelin receptor selectivity.

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Authors and Affiliations

  1. University of Michigan Scleroderma Program, University of Michigan Health System, Ann Arbor, MI, 48106, USA

    James R. Seibold

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  1. James R. Seibold
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Correspondence to James R. Seibold .

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Editors and Affiliations

  1. University College London, Gower Street, London, WC1E 6BT, United Kingdom

    David Abraham

  2. The Royal Free & Univ College Med School, Gower Street, London, WC1E 6BT, United Kingdom

    Handler Clive

  3. University College London, Gower Street, London, WC1E 6BT, United Kingdom

    Michael Dashwood

  4. The Royal Free & Univ College Med School, Gower Street, London, WC1E 6BT, United Kingdom

    Gerry Coghlan

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Seibold, J.R. (2009). PAH in CTD – Clinical Trials Criteria and Performance. In: Abraham, D., Clive, H., Dashwood, M., Coghlan, G. (eds) Advances in Vascular Medicine. Springer, London. https://doi.org/10.1007/978-1-84882-637-3_16

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