Two cases of Behçet’s disease with major vessel involvement

Aim of the workWe attempt here to describe 2 cases of Behçet’s disease (BD) with pulmonary vascular complications.Case presentationTwo cases of Behçet’s disease with major vessel involvement are presented. The first case presented with hemoptysis and investigations revealed the presence of pulmonary artery aneurysm (PAA). The patient received pulsed methylprednisolone 1 gm/day for 3 successive days and pulse cyclophosphamide 600 mg monthly for 6 months, and oral maintenance therapy 1 mg/kg prednisolone daily. The patient’s symptoms improved within 2 weeks and a follow up CT angiography after 1 year revealed normal pulmonary arteries. The second case presented with dilated tortuous veins over the chest wall and was diagnosed to have superior vena cava (SVC) obstruction. The patient was prescribed pulsed methylprednisolone 1 gm/day for 3 successive days and pulse cyclophosphamide 600 mg/month for 6 months and oral prednisolone 1 mg/kg/day maintenance therapy, in addition to, oral warfarin therapy 5 mg/day to maintain his INR at 2.5. The patient was discharged after stabilization of his condition.ConclusionSince a significant proportion of patients develops complications in arterial and venous vessels, vascular lesions should be included in the diagnostic criteria of BD. Challenges remain to identify those patients at risk, to understand the cause and best treatment of thrombosis in these patients, and to develop well tolerated and effective therapies to arrest or reverse the course of vasculitis in BD.