The Pathophysiology of Scleroderma Involving the Heart and Respiratory System

Article1 April 1964The Pathophysiology of Scleroderma Involving the Heart and Respiratory SystemMARVIN A. SACKNER, M.D., NECATI AKGUN, M.D., PHILIP KIMBEL, M.D., DAVID H. LEWIS, M.D.MARVIN A. SACKNER, M.D., NECATI AKGUN, M.D., PHILIP KIMBEL, M.D., DAVID H. LEWIS, M.D.Author, Article, and Disclosure Informationhttps://doi.org/10.7326/0003-4819-60-4-611 SectionsAboutPDF ToolsAdd to favoritesDownload CitationsTrack CitationsPermissions ShareFacebookTwitterLinkedInRedditEmail ExcerptThe lungs from patients with scleroderma show varying degrees of pulmonary fibrosis and medial hypertrophy of the pulmonary arterioles (1). However, little attention has been directed toward the hemodynamics of the pulmonary circulation particularly as related to the parenchymal alterations despite the morphologic observations consistent with pulmonary hypertension. Studies of pulmonary function show restrictive lung disease and impairment of gaseous diffusion, but tightness of the skin over the chest wall and myocardial fibrosis, which have been thought to produce physiologic disturbances, have not been critically evaluated (2-6). The purpose of the following report is to systematically investigate the physiologic nature...References1. MATSUI S: Über die Pathologie und Pathogenese von Sclerodermia universalis. Mitt. Med. Fak. Tokyo 31: 55, 1924. Google Scholar2. BALDWINCOURNANDRICHARDS EDADW: Pulmonary insufficiency. II. A study of thirty-nine cases of pulmonary fibrosis. Medicine 1: 25, 1949. Google Scholar3. MILLERFOWLERHELMHOLZ RDWSFH: Scleroderma of the lungs. Proc. Mayo Clin. 34: 66, 1954. Google Scholar4. ADHIKARIBIANCHIBOUSHYSAKAMOTOLEWIS PKFASFABM: Pulmonary function in scleroderma: its relation to changes in the chest roentgenogram and in the skin of the thorax. Amer. Rev. Resp. Dis. 86: 823, 1962. MedlineGoogle Scholar5. 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NAIMARKCHERNIACK ARM: Compliance of the respiratory system and its components in health and disease. J. Appl. Physiol. 15: 377, 1960. CrossrefMedlineGoogle Scholar This content is PDF only. To continue reading please click on the PDF icon. Author, Article, and Disclosure InformationAffiliations: Philadelphia, PennsylvaniaFrom the Division of Cardiology and Department of Medicine, Philadelphia General Hospital, and the Department of Physiology, Graduate School of Medicine, University of Pennsylvania, Philadelphia, Pennsylvania.This work was supported in part by U. S. Navy contract NOWR2744, No. 102-470, grant 62-G-123 from the American Heart Association, and grants H 4797 and HE-07415-01 from the United States Public Health Service, Bethesda, Maryland.Presented in part at the Forty-third Annual Session of the American College of Physicians, Philadelphia, Pennsylvania, April, 1962, and at the Nineteenth Annual Meeting of the American Federation for Clinical Research, Atlantic City, New Jersey, April, 1962.Requests for reprints should be addressed to Marvin A. Sackner, M.D., Department of Physiology, Graduate School of Medicine, University of Pennsylvania, Philadelphia, Pennsylvania 19104. 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