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Rheumatology 2006 45(Supplement 3):iii11-iii13; doi:10.1093/rheumatology/kel291
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© The Author 2006. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For Permissions, please email: journals.permissions@oxfordjournals.org

Pulmonary arterial hypertension (PAH) in connective tissue diseases

C. M. Kähler and D. Colleselli

Pneumology Service, Division of General Internal Medicine, Department of Internal Medicine, Medical University of Innsbruck, Austria.

Correspondence to: Ao. Univ.-Prof. Christian M. Kähler MD, Pneumology Service, Division of General Internal Medicine, Department of Internal Medicine, Innsbruck Medical University, Anichstrasse 35, A-6020 Innsbruck, Austria. E-mail: C.M.Kaehler{at}uibk.ac.at

Pulmonary arterial hypertension (PAH) is characterized by progressive obliteration of the small pulmonary vascular bed as a result of vascular proliferation and remodelling of the vessel wall leading to permanently increased pulmonary vascular resistance and elevated pulmonary artery pressures, which result in right heart failure and premature death. Pathologic processes behind the complex vascular changes associated with PAH include vasoconstrictor/vasodilator imbalance, thrombosis, misguided angiogenesis and inflammation. Besides idiopathic PAH, it can also occur in association with portal hypertension, HIV infection, congenital cardiac left-to-right shunts and connective tissue diseases (CTD). Unfortunately, despite recent major improvements in PAH treatment, no current therapy can yet cure this devastating condition. This review will briefly highlight epidemiology, pathogenesis, and diagnostic and treatment options known so far for PAH occurring in connection with CTD.


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V. Smith, M. De Pauw, G. Brusselle, J. Van Praet, and F. De Keyser
Re: Pulmonary arterial hypertension in connective tissue diseases
Rheumatology, September 1, 2007; 46(9): 1510 - 1510.
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