Pulmonary aneurysms associated with Behçet’s syndrome typically affect young men and carry a high mortality of 30% within 2 years of diagnosis1,2. A 27-year-old man with a history of Behçet’s syndrome was admitted to our hospital with an episode of large-volume hemoptysis.
The syndrome had originally manifested with severe ophthalmic involvement leading to visual loss. He had initially been managed with high-dose steroids and mycophenolate mofetil, but had stopped attending clinic 18 months prior to this emergency admission and had not continued taking any immunosuppression.
The plain chest radiograph from his admission showed a rounded opacity in the right lower zone (Figure 1, arrow). The corresponding computed tomography (CT) image of the thorax confirmed a partially thrombosed pulmonary artery aneurysm in this region (Figure 2, arrow); a smaller aneurysm is also visible in the left lower lobe (Figure 2, arrow). A coronal image shows the relationship between the large right-side aneurysm and its accompanying arterial supply (Figure 3).
Plain chest radiograph shows rounded opacity in the right lower zone (arrow).
CT angiography of the thorax shows a large right-side, partially thrombosed pulmonary artery aneurysm (arrow); a smaller partially thrombosed aneurysm in the left lower is also visible (arrow).
CT angiography of the thorax shows the relationship between the large right-side aneurysm and its accompanying arterial supply.
Our patient was treated with high-dose intravenous steroids and cyclophosphamide, as well as radiologically guided embolization of the aneurysm. To date, he has made a good recovery.
The combination of embolization and immunosuppression is reported as giving the best chance of recovery of current treatment strategies for pulmonary aneurysms associated with Behçet’s syndrome3. Surgical intervention can be necessary for urgent treatment, but is associated with recurrence at the operative site. Anti-tumor necrosis factor is recommended for other complications of Behçet’s, especially for eye involvement4. Evidence of usage for pulmonary artery aneurysms is limited at present5, but may represent a potential for future management of this severe life-threatening complication.