Abstract
The plexiform lesions of severe pulmonary arterial hypertension (PAH) are similar in histologic appearance, whether the disease is idiopathic or secondary. Both forms of the disease show actively proliferating endothelial cells without evidence of apoptosis. Here, we discuss the pathobiology of the atypical, angioproliferative endothelial cells in severe PAH. The concept of the endothelial cell as a "quasi-malignant" cell provides a new framework for antiproliferative, antiangiogenic therapy in severe PAH.
Publication types
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Research Support, N.I.H., Extramural
MeSH terms
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Cell Proliferation
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Endothelial Cells / pathology
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Endothelial Cells / physiology
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Gene Expression Regulation / physiology
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Humans
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Hypertension, Pulmonary / drug therapy
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Hypertension, Pulmonary / pathology
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Hypertension, Pulmonary / physiopathology*
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Immunohistochemistry
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Neoplasms / physiopathology
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Pulmonary Artery / pathology
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Terminology as Topic
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Vasoconstriction / physiology
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Wnt Proteins / metabolism
Substances
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WNT7A protein, human
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Wnt Proteins