A 24 year-old male patient presented with a recurrent oral and genital aphthous ulcers. There was a long-standing pain in his knee and elbow joints. The patient also complained of breathlessness at rest and effort. The patient had previously diagnosed Behçet's disease (BD). Chest computed tomography (CT) demonstrated multiple saccular pulmonary artery aneurism sacs especially based on bilaterally hilar-perihilar areas (Fig. 1A). Also no contrast filling was observed in the superior vena cava (SVC) owing to possible thrombus. Enlarged collateral vessels were in the left hemithorax wall and paravertebral areas (Fig. 1B). Venous circulation was reaching the inferior vena cava and right atrium via these collateral vessels, the left pherenic vein, the azygos-hemiazygos veins. There were also enlarged venous collaterals in the upper mediasten and anterior mediasten. Because of the ruptured pulmonary artery aneurysm, the patient died 10 days after presentation.
(A) Posterior view 3D volume rendering pulmonary CT angiography demonstrates multiple pulmonary artery aneurisms (arrows). (B) Anterior view 3D volume rendering CT angiography reveals enlarged collateral vessels in the left hemithorax wall and paravertebral areas (dashed arrows). Contrast media performed from left upper extremity reaches the inferior vena cava (IVC) and right atrium via these collateral vessels and the dilated left pherenic vein (dashed arrows). No contrast filling is observed in the SVC.
Vasculitis is the main cause of pathophysiology in BD. Arterial and venous vessels can involved by the disease. In vasculitis, the small, middle, and large size arteries are affected.1 Venous system involvement is more frequent than arterial system involvement. Thrombophlebitis, deep vein thrombosis, venous aneurysms are venous involvement patterns. SVC thrombosis is rar and serious venous complication of the disease.
