Behçet Syndrome

Question · easy

A 28-year-old man who emigrated from Turkey has a 4-year history of recurrent painful oral ulcers (5 or 6 episodes per year), two episodes of scrotal ulceration that healed with scarring, and one episode of painful bilateral blurred vision diagnosed as panuveitis. He takes no medications. Which of the following is the most likely diagnosis?

Reactive arthritis Systemic lupus erythematosus Crohn disease Behçet syndrome

Question · medium

Which of the following statements about the pathergy test in Behçet syndrome is correct?

A papule or pustule forms at a sterile needle-prick site within 24 to 48 hours, but the test is frequently negative in non-endemic populations It requires a skin biopsy demonstrating leukocytoclastic vasculitis with heavy IgA deposition Positivity requires the simultaneous presence of HLA-B51 It is positive in more than 90% of patients evaluated in North America

Question · medium

A 32-year-old man with established Behçet syndrome presents with a painful swollen leg; duplex ultrasonography confirms extensive femoropopliteal deep-vein thrombosis. CT angiography of the chest shows no pulmonary artery aneurysms, and he has no bleeding risk factors. In addition to considering anticoagulation, which of the following is the cornerstone of managing his thrombotic event?

Inferior vena cava filter placement plus aspirin Lifelong warfarin as the sole therapy Immunosuppressive therapy (e.g., azathioprine, or cyclophosphamide for severe disease) to control vessel-wall inflammation Catheter-directed thrombolysis

Question · hard

Using the International Criteria for Behçet Disease (ICBD) point score, which of the following patients meets the threshold for diagnosis?

A patient with recurrent oral ulcers, erythema nodosum, and a positive pathergy test A patient with recurrent oral ulcers and a single brainstem lesion attributed to another cause A patient with recurrent genital ulcers and pseudofolliculitis only A patient with recurrent oral ulcers and chronic arthralgias only

Question · hard

A 24-year-old man with Behçet syndrome develops acute posterior uveitis threatening the macula. Consistent with current evidence and the EULAR 2025 recommendations, which of the following is the most appropriate initial management?

Topical prednisolone eye drops alone with reassessment in 3 months Apremilast monotherapy Systemic glucocorticoids for rapid induction plus early long-term immunosuppression with azathioprine and/or a monoclonal anti-TNF-alpha antibody (infliximab or adalimumab) Long-term oral anticoagulation