PRIMARY CARE
Recommendation 1
General lifestyle modifications to reduce inflammation and promote immune health. Disease is chronic and relapsingRecommendation 2
regular follow-up to identify and treat flares is indicated.
Behcet's Syndrome
Not Verified
Disease overview
Multisystem inflammatory vasculitis of mostly unknown origin; affects vessels of varying sizes; likely involves environmental and genetic factors (Middle Eastern descent, HLA-B51 increase risk)
Common symptoms
Common triad of aphthous ulcersgenital ulcersuveitis (risk of blindness)may also see erythema nodosum (inflammatory nodules on shin)skin pustulespolyarthritis. Risk of thrombosisaneurysm. Neurologic deficits are rare but serious.
WHEN TO SUSPECT
Recommendation 1
Current International Study Group criteria requireRecommendation 2
aphthous ulcers at least 3x in 12 months, PLUS two of the followingRecommendation 3
genital ulcers, uveitis or retinal vasculitis, erythema nodosum or skin pustles, positive pathergy test.
HOW TO TEST
Recommendation 1
Current International Study Group criteria requireRecommendation 2
aphthous ulcers at least 3x in 12 months, PLUS two of the followingRecommendation 3
genital ulcers, uveitis or retinal vasculitis, erythema nodosum or skin pustles, positive pathergy test.
TREATMENT
Recommendation 1
Topical corticosteroids can help with oral and genital ulcers. Apremilast is another option for mucocutaneous ulcers. Ocular, vascular, or other systemic symptoms require long-term immunosuppressionRecommendation 2
corticosteroids are first-line to induce remission, and azathioprine is first-line as maintenance therapy. TNF-alpha inhibitors and interferon-alpha may be considered in refractory disease.
FURTHER SUPPORT
Recommendation 1
RheumatologyRecommendation 2
the American College of Rheumatology has a directory. NYU Langone has a dedicated Behcet Disease Center, (646-501-7400). American Behcet Disease Association offers patient and family support. Patients with ocular symptoms should be followed regularly by an ophthalmologist. . Referral to Medical Genetics Department, if available. Initial virtual care is also available through organizations like TeleRare Health.
CLINICAL TRIALS
Recommendation 1
Several clinical trials are recruitingRecommendation 2
currently the majority are outside of the US.
Sources
No data available
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