Melanie B. Peachell, MD; Nestor L. Muller, MD, PhD

Disclosures

Semin Respir Crit Care Med. 2004;25(5) 

In This Article

Behçet's Disease

Behçet's disease is a rare chronic, multisystem disease characterized by the presence of uveitis and oral and genital ulcers. It occurs most commonly in the Middle East and Japan and affects men more commonly than women.[29] Thoracic vascular involvement occurs in 25% of patients and pulmonary manifestations in ~5%.[29,30,31]

The most common thoracic manifestations of Behçet's disease are narrowing or thrombosis of the superior vena cava, pulmonary artery aneurysms, pulmonary hemorrhage, and pulmonary infarction.[32,33] Thrombosis of the superior vena cava is manifested radiologically by the presence of widening of the mediastinum.

Pulmonary artery aneurysms are manifested radiographically by the presence of single or multiple round perihilar opacities or rapidly progressive unilateral hilar enlargement.[29,32] The aneurysms may be single or multiple, unilateral or bilateral, and measure 1 to 7 cm in diameter.[32,34,35] Aneurysm formation is more commonly seen on the right (59%) and in the lobar arteries (54%).[35] The right lower lobe artery is the most commonly affected (35%) followed by the left lower lobe and right main pulmonary arteries[35] (Fig. 7). The presence, size, and location of pulmonary artery aneurysms can be assessed with CT and MRI.[32,35,36] Conventional angiography is not recommended in the imaging of patients with Behçet's disease because catheter insertion may lead to venous thrombosis or aneurysm formation at the site of puncture.

Behçet's disease. Computed tomographic image at the level of the superior pulmonary veins demonstrates circumferential nonocclusive thrombus within an aneurysm of the right interlobar artery (arrows). The patient was a 48-year-old man.

Pulmonary artery aneurysms can increase in size and rupture, leading to pulmonary hemorrhage and death, or regress spontaneously with treatment.[32,33,35] Pulmonary hemorrhage can result in focal, multifocal, or diffuse airspace consolidation.[29,34] Thrombotic occlusion of the aneurysmal pulmonary artery can result in localized areas of consolidation secondary to pulmonary infarction, areas of oligemia, and areas of atelectasis.[34,37]

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