Behçet's disease with severe arterial involvement in a child

• Published in: Clinical rheumatology 2002-05, Vol.21 (2), p.176-179
• Main Authors: Beşbaş, N, Ozyürek, E, Balkanci, F, Ozen, S, Saatçi, I, Ozaltin, F, Bakkaloğlu, A
• Format: Article
• Language: English
• Subjects: Adolescent; Angiography; Aortic Aneurysm, Abdominal - diagnostic imaging; Aortic Aneurysm, Abdominal - etiology; Aortic Aneurysm, Abdominal - surgery; Arterial Occlusive Diseases - diagnostic imaging; Arterial Occlusive Diseases - etiology; Arterial Occlusive Diseases - surgery; Behcet Syndrome - complications; Behcet Syndrome - diagnosis; Blood Vessel Prosthesis; Femoral Artery; Follow-Up Studies; Humans; Iliac Artery; Intracranial Aneurysm - diagnostic imaging; Intracranial Aneurysm - etiology; Intracranial Aneurysm - surgery; Male; Medical treatment; Mortality; Risk Assessment; Severity of Illness Index; Vascular Surgical Procedures
• ISSN: 0770-3198; 1434-9949
• DOI: 10.1007/s10067-002-8278-3

Behçet's disease is a vasculitis characterised by its thrombotic tendency. In some patients, manifestations of vascular lesions may dominate the clinical picture instead of the classic triad. We describe a 13-year-old boy with a 1.5-year history of Behçet's disease presenting with complaints of acute abdominal pain, severe headache and decreased vision. His work-up revealed a saccular aneurysm of the distal abdominal aorta, thrombosis in the right common iliac, external iliac and femoral arteries, and thrombosis of the superior sagittal sinus. Aortoiliac bypass with a Dacron graft was performed with success. He also received cyclosporin A and anticoagulant therapy. Severe vasculitis may become overt at any age in patients with Behçet's disease. Early diagnosis and management is important to prevent morbidity and mortality.