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Q1: Presentation & diagnosis (2 min)
A 29-year-old man of Turkish origin presents with recurrent oral and scrotal ulcers and a painful red eye. Take us through your diagnostic approach. Cover: the gateway question (recurrent oral aphthae — at least three episodes per year; they are universal and heal without scarring), the ICBD clinical criteria (ocular 2, genital aphthosis 2, oral aphthosis 2, skin 1, neuro 1, vascular 1, pathergy 1 optional; score at least 4), the discriminating point that genital ulcers scar, the pathergy test (papule/pustule at needle-prick at 24 to 48 hours), the Silk Road geography and HLA-B51 association, and the absence of any confirmatory blood test.
Q2: Organ involvement & complications (2 min)
Which organs are involved in Behçet's and what are the dangerous complications? Cover: ocular (panuveitis, hypopyon, retinal vasculitis — blindness); vascular (venous thrombosis, thrombophlebitis migrans, pulmonary artery aneurysm — the classic cause of fatal haemoptysis and the major cause of death, Budd-Chiari); neurological (parenchymal brainstem neuro-Behçet — poor prognosis; cerebral venous sinus thrombosis); gastrointestinal (ileocaecal ulcers mimicking Crohn's — perforation); articular (non-erosive arthritis); skin (erythema nodosum, papulopustular). Emphasise that young men with vascular disease have the worst prognosis.
Q3: Management (3 min)
How do you manage Behçet's syndrome? Cover the organ-based, severity-stratified (EULAR) approach: mucocutaneous = colchicine (especially erythema nodosum), topical steroids, apremilast, dapsone; ocular (sight-threatening) = azathioprine first-line, corticosteroids for flares, anti-TNF (infliximab/adalimumab) or interferon-alpha for resistant; major vascular = corticosteroids + cyclophosphamide (anti-TNF alternative); neuro-Behçet = steroids + cyclophosphamide/azathioprine, avoid ciclosporin (neurotoxic); GI = steroids, 5-ASA, azathioprine, thalidomide/anti-TNF, surgery for perforation. Multidisciplinary care, PJP prophylaxis and bone protection for prolonged steroids.
Q4: Thrombosis in Behçet's — a controversy (2 min)
A patient with Behçet's develops a deep vein thrombosis but has a normal coagulation screen. Why, and how does it change management? Cover: thrombosis is inflammatory (vasculitic) — neutrophilic vessel-wall inflammation and endothelial dysfunction drive thrombus, so INR/APTT/platelets are normal. Immunosuppression is the mainstay, not anticoagulation. Anticoagulation is controversial and individualised because coexisting arterial aneurysms bleed; European (EULAR) practice favours immunosuppression with selective anticoagulation, whereas some Asian centres use anticoagulation more liberally.
Q5: Differentials (1 min)
How do you distinguish Behçet's from its key mimics? Cover: Crohn's disease (also oral/genital ulcers, erythema nodosum, eye disease — but transmural skip lesions, perianal disease, granulomas on biopsy, no pulmonary artery aneurysm); reactive arthritis (painless circinate balanitis/keratoderma, asymmetric oligoarthritis, HLA-B27, post-infective); SLE (painless oral ulcers, glomerulonephritis, ANA/dsDNA positive); and recurrent aphthous stomatitis (oral ulcers only). Emphasise the combination of scarring genital ulcers, pathergy, venous thrombosis/aneurysm, and negative autoantibodies that points to Behçet's.