BEHCET'S DISEASE COMPLICATED WITH CEREBRAL VENOUS SINUS THROMBOSIS: A REPORT OF NINE CASES

Background: Behçet's disease (BD) is a chronic and multisystemic inflammatory disorder which can affect the central nervous system. Cerebral venous sinus thrombosis (CVT) is a frequent neurological manifestation of BD. Objectives: In this study, we aimed at investigating the epidemiologic, clinical, laboratory and management characteristics of our Behcet's patients with CVT. Methods: Medical records of patients with BD at department of rheumatology of the Ondokuz Mayıs university hospital from 2013 to 2015 were reviewed to identify CVT. Among 173 patients with BD, nine patients (one female and eight male) of angiographically proven CVT were identified from the records. Clinical, laboratory and management characteristics of the patients were recorded according to pre-defined protocol. Results: CVT was observed in 5% (9/173) of our patients. The mean age at diagnosis was 26 (19-39) years, the mean age at onset of disease was 25 (18-39) years and the average duration of disease was 40 (3-216) months. The most common clinical presentations were headache in 7 (77%) of cases. Eight patients (89%) were affected superior sagittal sinus and transvers sinus, two patients (22.9%) were affected sigmoid sinus and cavernous sinus. Genital ulcer was found in 77% (7/9), ocular involvement in 22% (2/9), superficial thrombophlebitis in 11% (1/9) of cases. There were not found erythema nodosum and arthritis. Four patients (44%) had pulmonary thromboembolism, three patients (33%) had vena cava thrombosis, two patients (22%) had femoral and iliac vein thrombosis, one patient (11%) had cardiac thrombosis and jugular vein thrombosis, and one patient (11%) had pulmonary artery aneurysm. Erythrocyte sedimentation rate and C-reactive protein were high in all patients on admission. All patients received with corticosteroids and 8 patients received with anticoagulation therapy except one patient with pulmonary artery aneurysm. The 7 patients receiving cyclophosphamide bolus, one patient receiving anti-TNF therapy, and one patient refused immunosuppressive treatment. The patients with treated corticosteroid, immunosuppressant (cyclophosphamide or anti-TNF therapy) and/or anticoagulation treatment evolved positively with clinical improvement and good tolerance. Conclusions: CVT in patients with BD may result in serious neurologic outcomes. The most common clinical presentations were headache. CVT in BD patients is male predominance, mainly multiple and venous thrombosis is more common in other site. Corticosteroid, immunosuppressant and/or anticoagulation treatment represent effective therapy. Disclosure of Interest: None declared DOI: 10.1136/annrheumdis-2015-eular.4430Citation: Annals of the Rheumatic Diseases, volume 74, supplement 2, year 2015, page 403Session: Other orphan diseases (Poster Presentations )