Diagnosis and Management of Behçet Disease

Clinical Presentation and Diagnosis

• The American College of Rheumatology criteria for Behçet disease require recurrent oral aphthous ulcers plus at least two of the following: recurrent genital ulceration, uveitis/retinal vasculitis, or specific skin lesions, which this patient meets 1
• Vascular involvement with cerebral thrombosis is a serious manifestation of Behçet disease, occurring in approximately one-third of patients and representing a poor prognostic factor requiring aggressive treatment 1, 2

Clinical Implications and Management

• The presence of cerebral venous thrombosis with Behçet disease should be treated with corticosteroids and immunosuppressive therapy, while anticoagulation is controversial in Behçet-related thrombosis 2, 3
• Major organ involvement (ocular, vascular, and gastrointestinal) is associated with poor prognosis and requires prompt, aggressive treatment, with young males with early disease onset experiencing more severe disease courses and requiring closer follow-up 1, 4