Expression of Endothelin-1 in the Lungs of Patients with Pulmonary Hypertension
Adel Giaid, Masashi Yanagisawa, David Langleben, Rene P. Michel, Robert Levy, Hani Shennib, Sadao Kimura, Tomoh Masaki, William P. Duguid, and Duncan J. Stewart
Background Pulmonary hypertension is characterized by an increasein vascular tone or an abnormal proliferation of muscle cellsin the walls of small pulmonary arteries. Endothelin-1 is apotent endothelium-derived vasoconstrictor peptide with importantmitogenic properties. It has therefore been suggested that endothelin-1may contribute to increases in pulmonary arterial tone or smooth-muscleproliferation in patients with pulmonary hypertension. We studiedthe sites and magnitude of endothelin-1 production in the lungsof patients with various causes of pulmonary hypertension.
Methods We studied the distribution of endothelin-1-like immunoreactivity(by immunocytochemical analysis) and endothelin-1 messengerRNA (by in situ hybridization) in lung specimens from 15 controlsubjects, 11 patients with plexogenic pulmonary arteriopathy(grades 4 through 6), and 17 patients with secondary pulmonaryhypertension and pulmonary arteriopathy of grades 1 through3.
Results In the controls, endothelin-1-like immunoreactivitywas rarely seen in vascular endothelial cells. In the patientswith pulmonary hypertension, endothelin-1-like immunoreactivitywas abundant, predominantly in endothelial cells of pulmonaryarteries with medial thickening and intimal fibrosis. Likewise,endothelin-1 messenger RNA was increased in the patients withpulmonary hypertension and was expressed primarily at sitesof endothelin-1-like immunoreactivity. There was a strong correlationbetween the intensity of endothelin-1-like immunoreactivityand pulmonary vascular resistance in the patients with plexogenicpulmonary arteriopathy, but not in those with secondary pulmonaryhypertension.
Conclusions Pulmonary hypertension is associated with the increasedexpression of endothelin-1 in vascular endothelial cells, suggestingthat the local production of endothelin-1 may contribute tothe vascular abnormalities associated with this disorder.
Source Information
From the Departments of Pathology (A.G., R.P.M., W.P.D.), Medicine (D.L., R.L., D.J.S.), and Surgery (H.S.), McGill University, Montreal; the Howard Hughes Medical Institute and Department of Molecular Genetics, University of Texas Southwest Medical Center, Dallas (M.Y.); Kyoto University, Kyoto, Japan (T.M.); and the Faculty of Medicine, Chiba University, Chiba, Japan (S.K.).
Address reprint requests to Dr. Giaid at the Dept. of Pathology, Montreal General Hospital, 1650 Cedar Ave., Montreal, QC H3G 14A, Canada.
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