Pulmonary Artery Aneurysm in Behcet Disease: Medical, Endovascular or Surgical Intervention

Abstract

Behçet's disease is a chronic inflammatory condition that predominantly affects the body's blood vessels, exhibiting various clinical manifestations and complications. The exact cause remains unclear, but genetic predisposition, immune responses, and vascular activation are believed to contribute to its development. This disease is more prevalent in certain geographic regions and primarily affects young adults, particularly males. Pulmonary aneurysm, a complication of Behçet's disease, is the leading cause of mortality in Behcet disease. In this review, we summarize the complications of Behcet disease with a focus on pulmonary artery aneurysms. We discussed the medical, endovascular, and surgical management of pulmonary aneurysms in Behcet disease and the indications and outcomes of the different treatment options. Corticosteroids and cyclophosphamide remain the preferred first-line therapy. However, clinical improvement with infliximab or adalimumab, tumor necrosis factor-alpha (TNFα) blocking agents, have been reported after treatment failure with recommended first-line agents. In patients who fail medical therapy or those with life-threatening hemoptysis, endovascular or surgical intervention is the next option. Endovascular interventions include pulmonary artery embolization with coils or acrylic glue and using plugs, occluders, or stents. Endovascular interventions usually have fewer adverse effects than surgery. Although the risk of surgical procedures is high in pulmonary artery aneurysms, it could be a life-saving procedure in patients with life-threatening hemoptysis. Surgical options, including pulmonary artery ligation, aneurysmorrhaphy, segmentectomy, lobectomy, or pneumonectomy are available. However, the results of surgical therapy for Behçet aneurysms are often disappointing.

Keywords: behcet’s syndrome; endovascular interventions; hemoptysis; pulmonary artery aneurysm; review article; vascular complications.