Summary
A case of pulmonary hypertension due to veno-occlusive disease is described. The patient was a 33 year old woman who died 11 months after first complaining of breathlessness. Veno-occlusive disease was diagnosed on lung biopsy and confirmed post-mortem. Electron microscopy of the lung biopsy showed electron-dense deposits in the capillary basement membranes, and immunoglobulin and complement were demonstrated in a corresponding position by immunofluorescent microscopy. It is suggested that immune complexes may have initiated thrombotic occlusion of the small pulmonary veins.
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Braun, A., Greenberg, S. D., Malik, S., Jenkins, D. E.: Pulmonary veno-occlusive disease associated with pulmonary phlebitis. Arch. Path.95, 67–70 (1973)
Brewer, D. B., Humphreys, D. R.: Primary pulmonary hypertension with obstructive venous lesions. Brit. Heart J.22, 445–448 (1960)
British Medical Journal: Pulmonary veno-occlusive disease (editorial)3, 369 (1972)
Carrington, B. C., Liebow, A. A.: Pulmonary veno-occlusive disease. Human Path.1, 322–324 (1970)
Crane, J. T., Grimes, O. F.: Isolated pulmonary venous sclerosis: a cause of cor pulmonale. J. thorac. cardiovasc. Surg.40, 410–416 (1960)
Davies, J. D.: Behçet's syndrome with haemoptysis and pulmonary lesions. J. Path.109, 351–356 (1973)
Fulton, R. M., Hutchinson, E. C., Jones, A. M.: Ventricular weight in cardiac hypertrophy. Brit. Heart J.14, 413–420 (1952)
Horatius, R. J. de, Abruzzo, J. L., Williams, R. C.: Immunofluorescent and immunologie studies of rheumatoid lung. Arch, intern. Med.129, 441–446 (1972)
Koffler, D., Sandson, J., Carr, R., Kunkel, H. G.: Immunologic studies concerning the pulmonary lesions in Goodpasture's syndrome. Amer. J. Path.54, 293–305 (1969)
Kuhn, C.: Systemic lupus erythematosus in a patient with ultrastructural lesions of the pulmonary capillaries previously reported in the Review as due to idiopathic pulmonary haemosiderosis. Amer. Rev. resp. Dis.106, 931–932 (1972)
Liebow, A. A.: Personal communication (1967)
Liebow, A. A., McAdams, A. J., Carrington, C. B., Viamonte, M.: Intrapulmonary veno-ob-structive disease. Circulation35–36, (Suppl. II) 172 (1967)
Liebow, A. A., Moser, K. M., Southgate, M. T.: Rapidly progressive dyspnoea in a teenage boy. J. Amer. med. Ass.233, 1243–1253 (1973)
Nagaya, H., Elmore, M., Ford, C. D.: Idiopathic interstitial pulmonary fibrosis. An immune complex disease? Amer. Rev. resp. Dis.107, 826–830 (1973)
Rosenthal, A., Vawter, G., Wagenvoort, C. A.: Intrapulmonary veno-occlusive disease. Amer. J. Cardiol.31, 78–83 (1973)
Stovin, P. G. I., Mitchinson, M. J.: Pulmonary hypertension due to obstruction of the intrapulmonary veins. Thorax20, 106–113 (1965)
Wagenvoort, C. A.: Vasconstrictive primary pulmonary hypertension and pulmonary venoocclusive disease. Cardiovasc. Clin.4, 97–113 (1972)
Wagenvoort, C. A., Losekoot, G., Mulder, E.: Pulmonary veno-occlusive disease of presumably intrauterine origin. Thorax26, 429–434 (1971)
Walford, R. L., Kaplan, L.: Pulmonary fibrosis and giant cell reaction with altered elastic tissue. Endogenous “pneumoeoniosis”. Arch. Path.63, 75–90 (1957)
Walton, K. W., Heath, D.: Iron incrustation of the pulmonary vessels in patent ductus arteriosus with congential mitral disease. Brit. Heart J.22, 440–444 (1960)
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Corrin, B., Spencer, H., Turner-Warwick, M. et al. Pulmonary veno-occlusion—an complex disease?. Virchows Arch. A Path. Anat. and Histol. 364, 81–91 (1974). https://doi.org/10.1007/BF01230859
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DOI: https://doi.org/10.1007/BF01230859