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| Disease found: | Behcet's Syndrome |
| Current as of: | Mon Sep 16 2024 |
| 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) [more info] |
| Signs and Symptoms: | Common triad of aphthous ulcers, genital ulcers, uveitis (risk of blindness); may also see erythema nodosum (inflammatory nodules on shin), skin pustules, polyarthritis. Risk of thrombosis, aneurysm. Neurologic deficits are rare but serious. [more info] |
| Diagnosis: | Current International Study Group criteria require: aphthous ulcers at least 3x in 12 months, PLUS two of the following: genital ulcers, uveitis or retinal vasculitis, erythema nodosum or skin pustles, positive pathergy test. [more info] |
| Treatment: | 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 immunosuppression; 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. [more info] |
| Clinical Management: | General lifestyle modifications to reduce inflammation and promote immune health. Disease is chronic and relapsing; regular follow-up to identify and treat flares is indicated. [more info] |
| Referral: | Rheumatology; 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. [more info]. Referral to Medical Genetics Department, if available. TeleRare Health for virtual care and consults is also recommended. |
| Clinical Trials: | Several clinical trials are recruiting; currently the majority are outside of the US. |