Behcet’s Disease - Diagnosis and Management Summary
Autoinflammatory ...

Behcet’s Disease - Diagnosis and Management Summary

Autoinflammatory condition characterized by recurrent aphthae, vasculitis, and skin/GI/neuro/joint sx

 • Epi: W > M, 20-40 y/o, ↑ Turkey, Middle East, and Asian countries

 • Sx: Recurrent painful oral ulcers and ≥2 of the following: painful genital ulcers (specific), ocular disease (most commonly uveitis or retinitis]), skin lesions (pustules, folliculitis, papules, erythema nodosum), negative pathergy test (skin pustule formation to needle prick [NB: not sensitive in Caucasians])

Other manifestations include: GI (similar to IBD), neurologic disease (both parenchymal and extra-parenchymal), vascular disease (ATE/VTE, vasculitis [small/medium/large vessels], aneurysms [esp. pulmonary artery, OJRD 2011;6:15]) or arthritis (nonerosive, asymmetric). Less common: kidneys, heart, lung disease

• Dx: Clinical dx only, no specific laboratory tests exist; may have ↑ ESR/CRP

• Rx 

    o Mild (arthritis, ulcers): Colchicine 1-2 mg daily, low dose prednisone. Apremilast (PDE-4 inhibitor) for ulcers 

    o Severe: Prednisone 1mg/kg/d; may add 2nd line agents: AZA, anti-TNF, IFNα, CYC, CP, MTX. If organ failure (esp. ophthalmic involvement): IV pulse steroids x3 days

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