R. Klii

Hughes-Stovin syndrome revealing the presence of Behçet’s Disease

Background: Hughes-Stovin Syndrome (HSS) is a rare clinical disorder characterized by deep venous thrombosis and multiple pulmonary and/or bronchial aneurysms. Aneurysms in systemic circulation can also be seen. Case presentation: We report the first case of HSS with aortic aneurysm in a 55-year-old man who initially presented with deep venous thrombosis. The diagnosis of HSS revealing Behçet’s disease was made given the history of recurrent oral and genital ulcers. Treatment consisted of 3 daily pulses of methylprednisolone (1g) followed by oral prednisone (1mg/kg daily) and 6 monthly pulses of cyclophosphamide. Oral anticoagulation treatment was held 3 months and then was stopped with good outcome. Conclusion: Systemic aneurysms in Hughes Stovin is a worth knowing complication which may reveal Behçet’s disease.

0103: Cardiovascular involvement in Behçet disease: a retrospective study

Background Behcet disease (BD) is a multisystemic inflammatory disorder classified among the vasculitides which can affect all types and sizes of blood vessels. Objective Our aim is to study the characteristics of cardio vascular involvement in BD. Methods medical records of 180 patients diagnosed BD according to the International Study Group of Behcet Disease, in the Internal Medicine Department of F.Bourguiba hospital in Monastir, were retrospectively analyzed. The diagnosis of vascular involvement was made by Doppler ultrasonography and/ or computed tomography. Results there were 65% males and 35% females. The mean age at diagnosis was 30 years. Familiar history of BD was found in 22.5% of cases. Vascular involvement was found in 32% of patients revealing the diagnosis in 3% of cases. Superficial venous thrombosis was found in 25% of cases. Deep venous thrombosis was diagnosed in 62.5% of patients as following: upper limb in 3.1%, lower limb in 34.4%, bilateral lower limb in 6.3% and inferior vena cava in 18.7%. Arterial manifestations were found in 21.9% of cases with pulmonary embolism in 12.5% of patients and pulmonary arterial aneurysm in 9.4%. Cardiac involvement was as following: myocardial infarction and coronary aneurysm in 1.6% and intracardiac thrombosis in 1.6%. The medical treatment was based on anticoagulants and colchicine in all cases; corticosteroids in 45.2% of cases with immunosuppressive drugs in 15.6%. Arterial pulmonary embolisation was performed in two cases. Surgical treatment was necessary in 6 cases: IVC filter (3 cases), lobectomy (2 cases). Conclusion vascular involvement is a common complication of Behcet’s disease which could lead to worse prognosis if not diagnosed early.