Huge femoral artery pseudoaneurysm in a patient with Behçet's disease

BackgroundBehçet's disease (BD) is a variable vessel vasculitis and vascular involvement is one of its life threatening manifestations. Arterial involvement frequently occurs with male predominance with pseudoaneurysms being the most common presentation. Immunosuppressive therapy is the mainstay of treatment in vascular involvement.Case presentationThe case we report here is a 40 year old Iraqi BD patient with manifestations of recurrent oral and genital ulcers, bilateral anterior uveitis, and deep vein thrombosis. The pathergy test was positive. The HLA-B51 was negative, erythrocyte sedimentation rate 102 mm/1st h and C-reactive protein was 48 mg/L. After discontinuation of his medications for about 9 months, the disease presented with leg pain and swelling that was diagnosed as huge left superficial femoral artery pseudoaneurysm by Doppler ultrasonography. CT angiography revealed a 90 × 88 × 70 mm pseudoaneurysm with partial mural thrombosis. He was scheduled for emergency surgery due to severe intractable pain. he received a pulse of methylprednisolone 1 g/day for 3 days and then surgery was done in the form of exclusion, repair and femorofemoral bypass were done. Post-operatively, the patient had an uneventful course; distal pulses became palpable, pain and swelling subsided. Post-operation, prednisolone 1 mg/kg was continued and he received cyclophosphamide 750 mg intravenously. His blood homocysteine level was higher than normal 23.8 μmol/L. He was discharged with a high dose of steroid and monthly cyclophosphamide treatment.ConclusionArterial pseudoaneurysm is life-threatening in BD and should be kept in mind to prevent major complications. Vascular involvement in BD patients is probably associated with hyperhomocysteinemia.