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Advances in Vascular Medicine pp 289–303Cite as

Disease Progression in Systemic Sclerosis Associated Pulmonary Arterial Hypertension

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Abstract

Systemic sclerosis associated pulmonary arterial hypertension (SSc-PAH) is an important cause of morbidity and mortality in patients with systemic sclerosis (SSc). Although studies based on echocardiography-derived pulmonary artery pressures have resulted in estimates of the prevalence of pulmonary arterial hypertension (PAH) in patients with SSc as high as 35%, more recent studies, using catheter-based diagnoses, have produced estimates of between 7.8 and 12%.1–3 Historically, SSc-PAH has had a poor outlook with rapid clinical deterioration and a 3-year survival of 30%.4 This was worse than in idiopathic pulmonary arterial hypertension (iPAH) wherein median survival prior to disease-modifying therapy was 2.8 years.5

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Author information

Authors and Affiliations

  1. Pulmonary Vascular Disease Unit, Royal Hallamshire Hospital, Sheffield, UK

    Robin Condliffe

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  1. Robin Condliffe
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Correspondence to Robin Condliffe .

Editor information

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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© 2009 Springer-Verlag London

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Condliffe, R. (2009). Disease Progression in Systemic Sclerosis Associated Pulmonary Arterial Hypertension. 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_17

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  • DOI: https://doi.org/10.1007/978-1-84882-637-3_17

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  • Print ISBN: 978-1-84882-636-6

  • Online ISBN: 978-1-84882-637-3

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