A case of Behçet's syndrome with rupture of a pulmonary aneurysm: autopsy findings and a literature review.

A 34 year old man suffering from oral and genital ulceration with uveitis (the complete type of Behçet's syndrome) developed fluctuating radiological opacities in the right lung and showed recurrent hemoptysis. Pulmonary angiography showed multiple aneurysms and obstruction of the pulmonary arteries. After he was treated with prednisolone, the symptoms and pulmonary manifestation improved temporarily. However, he died suddenly from a massive hemoptysis. An autopsy revealed the aneurysmal rupture of right basilar branch of pulmonary artery into the right B6 bronchus. Histopathological findings in the lung showed multiple organized thrombi, inflammatory infiltration and vascular wall destruction of pulmonary arteries of various sizes. Only 47 previously reported cases of Behçet's syndrome which showed pulmonary involvements were revealed by a search of the published literature.

[1]  Slavin Re,et al.  Pathology of the lung in Behçet's disease. Case report and review of the literature. , 1981 .

[2]  B. Wechsler,et al.  Multiple pulmonary arterial aneurysms in Behcet's disease and Hughes-Stovin syndrome. , 1981, The American journal of medicine.

[3]  R. Slavin,et al.  Pathology of the lung in Behçet's disease. Case report and review of the literature. , 1981, The American journal of surgical pathology.

[4]  R. Shapiro,et al.  The immune complex pathogenesis of glomerulonephritis and pulmonary vasculitis in Behçet's disease. , 1979, The American journal of medicine.

[5]  Reza Mj,et al.  Behçet's disease: a case with hemoptysis, pseudotumor cerbri, and arteritis. , 1978 .

[6]  T. Petty,et al.  Recurrent pneumonia in Behçet's syndrome. Roentgenographic documentation during 13 years. , 1977, JAMA.

[7]  E. Cadman,et al.  Pulmonary manifestations in Behçet syndrome. Case report and review of the literature. , 1976, Archives of internal medicine.

[8]  T. Chajek BEHÇET'S DISEASE. REPORT OF 41 CASES AND A REVIEW OF THE LITERATURE , 1975, Medicine.

[9]  K. Ooe,et al.  An autopsy case of Behçet's syndrome associated with pulmonary arteritis and tuberculosis. , 1974, Japanese circulation journal.

[10]  J. Davies Behçet's syndrome with haemoptysis and pulmonary lesions , 1973, The Journal of pathology.

[11]  E. Kansu,et al.  Behçet's syndrome with obstruction of the venae cavae. A report of seven cases. , 1972, The Quarterly journal of medicine.

[12]  A. Decroix Thoracic manifestations of Behcet's syndrome. , 1969, Thorax.

[13]  E. Hills Behçet's syndrome with aortic aneurysms. , 1967, British medical journal.

[14]  R. A. Green,et al.  Pulmonary artery aneurysms with recurrent thrombophlebitis. The "Hughes-Stovin syndrome". , 1962, Annals of internal medicine.

[15]  R. Charlton,et al.  Multiple Pulmonary Artery Aneurysms , 1961, Thorax.

[16]  P. Stovin,et al.  Segmental pulmonary artery aneurysms with peripheral venous thrombosis. , 1959, British journal of diseases of the chest.

[17]  J. B. Dalgaard,et al.  Mucocutaneous-ocular syndrome with intestinal involvement; a clinical and pathological study of four fatal cases. , 1958, The American journal of medicine.

[18]  C. Pirani,et al.  Thromboendarteritis with multiple mycotic aneurysms of branches of the pulmonary artery. , 1949, American journal of diseases of children.

[19]  J. Beattie,et al.  Multiple Embolic Aneurysms of Pulmonary Arteries following Thrombosis of Veins of Leg; Death from Rupture of Aneurysm into Lung. , 1912, Proceedings of the Royal Society of Medicine.