Long-Term Stabilization after Thoracic Endovascular Aortic Repair for a Behçet-Related Thoracic Aortic Aneurysm: A...

Behçet disease is a relapsing systemic vasculitis that can rarely cause aortic aneurysms or pseudoaneurysms. Endovascular repair is increasingly used for Behçet-related aortic lesions, but the long-term interaction between aneurysm remodeling, endoleak, and systemic inflammatory activity remains clinically important.

A 34-year-old man presented with persistent back pain due to an impending rupture of a newly developed saccular descending thoracic aortic aneurysm. Emergency thoracic endovascular aortic repair was performed before Behçet disease was definitively diagnosed. Postoperatively, persistent fever, elevated inflammatory markers, recurrent oral aphthous ulcers, and intestinal ulcerations led to the diagnosis of Behçet disease. The aneurysm initially regressed after corticosteroid therapy but rapidly re-expanded during steroid tapering, in parallel with fever, back pain, and increased C-reactive protein and erythrocyte sedimentation rate, despite no demonstrable type I or III endoleak. Additional endovascular reinforcement and intensified immunosuppressive therapy were performed. A type II endoleak from the left first lumbar artery was subsequently detected, but the aneurysm continued to regress under inflammatory control. More than 13 years after the initial repair, the treated aneurysm remains completely regressed.

This case suggests that sac enlargement after endovascular repair for a Behçet-related aortic aneurysm should be evaluated as a potentially multifactorial event, including occult endoleak, persistent sac pressurization, stent-graft-related mechanical factors, and systemic inflammatory activity. In this case, durable stabilization was observed following combined endovascular treatment and sustained immunosuppressive therapy.