Behcet disease
Behcet disease is a variable-vessel vasculitis, the only vasculitis that can involve arteries and veins of any size, marked by recurrent oral and genital ulceration, uveitis, skin lesions, and pathergy. There is no confirmatory laboratory test; diagnosis is clinical. Treatment is organized by organ severity, from colchicine for mucocutaneous disease to urgent immunosuppression and tumor necrosis factor inhibitors for sight-, organ-, or life-threatening involvement.
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What this article covers
- Overview
Definition: Behcet disease is a variable-vessel vasculitis, able to involve arteries and veins of any size, the feature that sets it apart from every other named vasculitis.
- Epidemiology
Silk Route distribution: endemic areas run along the historic Silk Route from East Asia to the Mediterranean, concentrated in patients of Turkish, Eastern Mediterranean, and East Asian descent.
- Risk factors
Silk Route ancestry: Turkish, Eastern Mediterranean, and East Asian descent carry the highest disease frequency.
- Pathophysiology
Mixed immune mechanism: HLA-B51 supports an autoimmune process, but recurrent flares without a defined autoantibody have led many experts to describe Behcet disease as combining autoimmune and autoinflammatory mechanisms.
- Clinical features
Oral ulceration: recurrent, painful aphthous ulcers occur in virtually every patient and are the most consistent feature of the disease.
- Differential diagnosis
Recurrent aphthous stomatitis: shares painful oral ulcers, but lacks the genital ulceration, ocular disease, pathergy, and vascular or central nervous system involvement that mark Behcet disease.
- Diagnosis
There is no confirmatory laboratory test; the diagnosis is established clinically, using the International Criteria for Behcet Disease, a point-based scheme in which recurrent oral ulceration…
- Management
First-line, mucocutaneous disease (oral and genital ulcers, skin lesions, arthritis): colchicine 0.6 mg PO once or twice daily (0.6 to 1.2 mg/day), better for genital ulcers, erythema nodosum, and arthritis than for oral ulcers alone.
- Complications
Blindness: posterior uveitis and retinal vasculitis are the most feared threats to vision.
- Prognosis
Severity tracks organ involvement: mucocutaneous-only disease is generally not life-threatening, while vascular, ocular, and central nervous system involvement carry the most ominous outlook.
- Prevention
No primary prevention exists, since the definitive cause is unknown; prevention centers on early treatment of organ-threatening disease and monitoring for drug toxicity.
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