Pulmonary artery aneurysms - Behçet disease

Case contributed by Dr Ahmed Abdrabou

Presentation

Known case of "a systemic disease", develops chest pain and dyspnea.

Patient Data

Age: 35 years
Gender: Male

A saccular aneurysm arises from the descending branch of each pulmonary. Another large aneurysmal sac with large mural thrombus is seen at the left lingular region likely arising from segmental branch. Nevertheless, there is thrombosed SVC with development of collateral pathways through the prominent azygos/hemiazygos as well as IVC-hepatic veins-internal mammary veins pathoway.

Case Discussion

Behçet disease is a sort of systemic vasculitis characterized by oral and genital ulcers as well as uveitis of the eye. Thoracic manifestations of Behçet disease include multiple bilateral pulmonary artery aneurysms that are of fusiform or saccular type and usually involves the main pulmonary artery or its descending branch. SVC obstruction developed secondary to inflammation of the vessel wall that led to thrombosis. Collateral pathways developed to bypass the thrombosed SVC 1.

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Case information

rID: 32749
Published: 11th Dec 2014
Last edited: 14th Aug 2019
System: Vascular, Chest
Inclusion in quiz mode: Included

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