Abstract
Behçet disease is the only primary vasculitis that affects both arteries and veins of any size. We present our treatment protocol in disease with vascular involvement in seven pediatric patients. All seven patients met the international criteria for the disease before the age of 16 years. Only one was a girl. The vascular involvement was as follows: Two patients had superficial vein thrombosis, two patients had atrial or ventricular thrombosis, one had arterial involvement with pulmonary aneurysms, and two had thrombosis of the venous sinuses in the central nervous system. The median duration of vascular involvement was 4 months (range 3-24 months) after the diagnosis of BD and was concomitant with diagnosis in three patients. All received colchicine and steroids. The ones with thrombosis in the venous system received 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. All except the patient with pulmonary arterial involvement received a course of anticoagulation treatment as well. These patients have been followed up for a period of at least 18 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 patients with Behçet disease. Effective management has enabled disease-free survival in the presented patients.
Talk to us
Join us for a 30 min session where you can share your feedback and ask us any queries you have
Disclaimer: All third-party content on this website/platform is and will remain the property of their respective owners and is provided on "as is" basis without any warranties, express or implied. Use of third-party content does not indicate any affiliation, sponsorship with or endorsement by them. Any references to third-party content is to identify the corresponding services and shall be considered fair use under The CopyrightLaw.