© 2003 by European Society of Cardiology
Copyright © 2003, European Society of Cardiology
The tantalizing triplet of pulmonary hypertension—BMP receptors, serotonin receptors, and angiopoietins
Department of Medicine, University of Giessen School of Medicine, Aulweg 123, 35392 Giessen, Germany
*Corresponding author. Tel.: +49-641-9942300; fax: +49-641-9942309. Email address: oliver.eickelberg@innere.med.uni-giessen.de
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See article by Blanpain et al. (pages 518–528) in this issue.
Not many diseases have attracted as much attention by the basic and clinical research community during the last several years as has idiopathic pulmonary arterial hypertension (IPAH, formerly primary pulmonary hypertension). IPAH is a severe and progressive disease localized exclusively to the lung, which exhibits an abnormally elevated precapillary pulmonary vascular resistance due to vasoconstriction and remodelling of the pulmonary vascular system. The underlying histological abnormalities consistently found in severe disease are obstructive and concentric lesions of small pulmonary resistance arterioles, which lead to an elevated pulmonary arterial pressure of >25 mm Hg at rest or >30 mm Hg during exercise. These lesions are thought to be due to enhanced proliferation of endothelial and/or smooth muscle cells or to the local transformation of quiescent fibroblasts into smooth muscle cells. The
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