Behçet's disease; A rare refractory patient with vena cava superior syndrome treated with infliximab: a case report and review of the literature.

Objectives

Behçet's disease (BD) is a multi-systemic inflammatory disorder which can affect all types and sizes of vessels.The usage of TNF-α antagonists is increasing in different involvements of BD that is inadequately controlled by standard immunosuppressive regimens.

Methods

Here we reported a rare BD case that is controlled by only infliximab (IFX) treatment.

Results

A 24-years-old male patient was diagnosed as BD with recurrent oral and genital ulcerations,uveitis,femoral vein thrombosis and HLA-B5 positivity.He had had terminal ileitis,epididymitis,bilateral internal iliac artery aneurysm and superior sagittal sinus thrombosis after the diagnosis.He admitted to our outpatient clinic with dyspnea,orthopnea,chest pain and facial swelling.We detected facial edema,plethora,venous distention on neck and chest.We suspected vena cava superior syndrome(VCSS) and performed CT angiography that demonstrated high-grade vena cava superior stenosis and thrombus where SVC opens to the right atrium.Patient was anticoagulated with warfarin and a shunt was implanted between the left brachiocephalic vein and right atrium.Colchicine and interferon-alfa treatment were continued and 1mg/kg steroid was added.But the patients' symptoms relapsed one month later and his shunt was revised with balloon angioplasty.5mg/kg IFX was started.We  performed a CT-angiography annually for two years.VCSS and other symptoms of BD are resolved and have not relapsed yet.

Conclusions

Vascular involvement of BD are generally treated with immunosuppressive agents such as steroids and immunomodulators.IFX was found effective and well tolerated in the treatment of intestinal,neurological and vascular involvement of BD especially in patients with poor response or intolerance to conventional therapy.This case report supports IFX as a new therapeutic option for patients with vascular BD.