Behçet Syndrome
Rheumatology · Other Rheumatologic Diseases 🎯 Practice these cards
Behçet syndrome is classically characterized by recurrent painful oral ulcers, ____ ulcers, inflammatory ____ disease, and ____.
Behçet syndrome is a variable-vessel systemic vasculitis defined by recurrent painful oral ulcers, genital ulcers (which tend to scar), inflammatory eye disease (uveitis/retinal vasculitis), and pathergy; vascular and CNS involvement are the ominous, organ-threatening features.
Behçet syndrome prevalence is highest in ____, at roughly ____ per 100,000, along the historic ____.
Behçet syndrome prevalence tracks the historic Silk Route from East Asia to the Mediterranean and is highest in Turkey (20-600 per 100,000; about 1 in 250 adults). Disease outside endemic regions tends to be milder; onset is unusual before the late teens or after age 50.
The principal genetic risk factor for Behçet syndrome is the MHC class I antigen ____, present in ____% of patients; it is not used diagnostically because ~____% of the normal endemic population also carries it.
HLA-B51 (a class I MHC antigen) is the main genetic risk factor for Behçet syndrome, present in 50-60% of patients, but it is also carried by ~20% of the healthy endemic population — it is neither sensitive nor specific and is not used as a diagnostic test.
Pathergy — a papule or pustule forming ____ hours after a sterile needle prick — strongly suggests Behçet syndrome but is often ____ in non-endemic areas.
Pathergy is the formation of a sterile papule or pustule 24-48 hours after the skin is disrupted with a sterile needle (classically 20-gauge). It reflects neutrophil hyperreactivity and strongly suggests Behçet syndrome, but results are frequently negative in non-endemic areas.
In the ICBD score, oral ulcers, genital ulcers, and eye lesions each score ____ points (skin, vascular, CNS, and pathergy score 1 each); ____ or more total points establishes the diagnosis.
International Criteria for Behçet Disease (ICBD): recurrent oral ulcers 2 points, genital ulcers 2, eye lesions 2, skin lesions 1, vascular lesions 1, CNS lesions 1, pathergy 1. A total of >=4 points establishes the diagnosis.
The ISG criteria require recurrent ____ ulcers plus ____ of four additional features (genital ulcers, skin lesions, eye lesions, positive pathergy).
International Study Group criteria (sensitivity/specificity ~95-96%): recurrent oral ulcers (>=3 episodes in 12 months) PLUS two of four — recurrent genital ulcers, characteristic skin lesions, eye lesions, or a positive pathergy test.
The most specific lesion of Behçet syndrome is the ____ ulcer, which typically appears on the scrotum or labia and tends to ____.
Genital ulcers are the most specific lesion of Behçet syndrome: typically on the scrotum or labia, larger and deeper than oral ulcers, slower to heal, and prone to disfiguring scarring.
Acute thrombosis in Behçet syndrome is treated primarily with ____ rather than anticoagulants, because the mechanism is ____ rather than thrombotic.
Thrombosis in Behçet syndrome is inflammatory, not thrombotic: acute events are treated with immunosuppression (azathioprine; cyclophosphamide for severe disease) rather than anticoagulants. Anticoagulation may be added only if bleeding risk is low and pulmonary artery aneurysms have been excluded.
____, a phosphodiesterase-4 inhibitor, is FDA approved for ____ ulcers in Behçet syndrome.
Apremilast, an oral phosphodiesterase-4 inhibitor, is FDA approved for oral ulcers in Behçet syndrome. Colchicine (better for arthritis/erythema nodosum than for ulcers) and thalidomide are other steroid-sparing mucocutaneous options; 5-ASA derivatives can treat GI mucosal disease.
Eye involvement in Behçet syndrome requires early aggressive treatment: brief courses of ____ for induction plus long-term ____; TNF inhibitors are used for severe disease.
Ocular Behçet disease requires early aggressive treatment: brief systemic glucocorticoid courses for induction plus long-term azathioprine; infliximab or adalimumab (alone or with glucocorticoids and azathioprine) for severe or relapsing disease; cyclosporine combinations or interferon-alpha monotherapy are alternatives.
Most new Behçet manifestations appear within the first ____ years; tapering can be attempted after ____ years of control; the highest-risk demographic is ____.
Most new Behçet manifestations appear within the first 5 years; the natural course then tends toward diminishing symptoms and often remission. Taper or stop medications after 2-3 years of control. Young male sex predicts the worst prognosis.
The EULAR 2025 update encourages early use of monoclonal ____ antibodies for organ-threatening Behçet disease, while maintaining a ____ approach for mucocutaneous and joint disease.
The EULAR 2025 recommendations for Behçet syndrome encourage early use of monoclonal anti-TNF-alpha antibodies for organ- or life-threatening manifestations (eye, vascular, CNS), while keeping a step-up approach — apremilast, then TNF inhibitors — for refractory mucocutaneous and joint disease.
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