Behcet Disease: treatment of vascular involvement in children

Behcet disease is the only primary vasculitis that affects both arteries and veins of any size. We present our treatment strategy in Behcet disease with vascular involvement in seven childhood patients. All seven patietns met the international criteria for the disease before the age of 16. Only one was a girl. The vascular involvement was as follows: Two patients had superficial vein thrombosis, two patients had atrial or ventricular thrombosis, two had sinus vein thrombosis and one had arterial involvement with pulmonary aneurysms.

The disease was diagnosed 4 months (range 3–24 months) before the vascular involvement in 4 patients and concomitant with diagnosis in 3 patients. All received colchicine, steroids and anticoagulant therapy. The ones with thrombosis in the venous system recieved additional azathioprine whereas those with pulmonary arterial or cardiac involvement initially received cyclophosphamide for 150–180 mg/kg total dose (IV or oral) and then were switched to azathioprine for a further 6 months. These patients have been followed up for period of 12 months (ranging 4–30 months) and so far are free of vascular relapses. One has developed a severe uveitis necessitating further therapy.

In conclusion features of vascular involvement should be carefully sought for in patietns with Behcet disease. Effective management is required for this feature of the disease that has a high morbidity.