PMID: 16634366Apr 26, 2006Paper

Connective tissue associated pulmonary arterial hypertension

Lupus
J G Coghlan, C Handler

Abstract

The number of publications in the field of pulmonary hypertension has increased exponentially over the past 10 years. As a consequence our understanding of the condition has advanced enormously. Pulmonary hypertension is now recognised as an uncommon and treatable disease, rather than a rare lethal disease. Progress in the field of connective tissue disease associated pulmonary hypertension has been less consistent. This paper covers the most influential published data on connective tissue associated pulmonary hypertension, and adds some insights from data available on file with pharmaceutical companies and from our database at the Royal Free Hospital. The following conclusions may be drawn. First, the prevalence of pulmonary hypertension associated with connective tissue diseases is lower than generally accepted. Second, the natural history and prognosis, at least in the setting of systemic sclerosis, is worse than seen in patients with idiopathic pulmonary hypertension. Finally, widespread and timely availability of disease modifying therapies substantially improves the prognosis for patients with systemic sclerosis associated pulmonary hypertension, in the treatment population and consequentially in the population as a whole...Continue Reading

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Citations

Jan 22, 2013·Rheumatology International·Anupama Shahane
Aug 13, 2010·Nature Reviews. Rheumatology·Maurizio CutoloVanessa Smith
Apr 9, 2010·International Journal of Rheumatic Diseases·Geoff Strange, Peter Nash
Dec 7, 2007·Annals of the Rheumatic Diseases·C P DentonUNKNOWN TRacleer Use in PAH associated with Scleroderma and Connective Tissue Diseases (TRUST) Investigators
Nov 5, 2010·Seminars in Arthritis and Rheumatism·Soumya Chatterjee
Jul 11, 2009·Clinical Radiology·A Devaraj, D M Hansell
Nov 15, 2011·Arthritis Care & Research·Aryeh FischerVirginia D Steen
Sep 10, 2010·International Journal of Rheumatology·Maurizio CutoloVanessa Smith
Aug 5, 2010·International Journal of Occupational Medicine and Environmental Health·Dorota KaletaWojciech Drygas
Dec 3, 2016·European Respiratory Review : an Official Journal of the European Respiratory Society·Gérald SimonneauNazzareno Galiè
Jul 16, 2008·Jornal brasileiro de pneumologia : publicaça̋o oficial da Sociedade Brasileira de Pneumologia e Tisilogia·Alfredo Nicodemos Cruz SantanaCarmen Silvia Valente Barbas

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