Abstract
Adamantiades-Behçet’s disease is a chronic, relapsing inflammatory disorder of unknown etiology with classic triad findings: recurrent oral and genital aphthous ulcers, ocular inflammation, and skin lesions. It frequently involves the CNS and GI tract. Ocular inflammation is present in 67–95% in the form of anterior uveitis and devastating retinal vasculitis.
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Keywords
Overview
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Definition
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A chronic, relapsing inflammatory disorder of unknown etiology with classic triad findings
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Recurrent oral and genital aphthous ulcers
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Ocular inflammation
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Skin lesions
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International criteria:
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Behçet’s Research Committee of Japan
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Complete, incomplete, suspect, possible
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International Study Group for Behçet’s Disease Criteria
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Oral ulcers +2 of: genital ulcers, eye lesions, skins lesions, pathergy test
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Frequently involves CNS and GI tract as well
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Ocular inflammation in 67–95%
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Anterior uveitis
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Devastating retinal vasculitis
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Symptoms
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Blurring
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Scotomas
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Redness
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Periorbital pain
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Photophobia
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Tearing with rare ocular discharge
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Diplopia (with neurologic involvement)
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Laterality
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Unilateral progressing to bilateral, 80%
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Course
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Recurrent inflammation, not typically chronic
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Ocular flares are often severe
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Age of onset
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25–35 years worldwide (range 2 months to 72 years)
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Gender/race
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Historically M > F, may be more even distribution
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Most common in Eastern Mediterranean and East Asian
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Systemic association
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Systemic vasculitis
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Oral and/or genital aphthous ulcers
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Various mild to severe organ involvement including skin, heart, CNS, GI, lungs, GU, joints
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Exam: Ocular
Anterior Segment
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Acute anterior uveitis (AU):
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Non-granulomatous
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May progress to “shifting” hypopyon if untreated, 19–31%
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More common:
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Cataract
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Posterior synechiae
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Peripheral anterior synechiae
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Iris atrophy
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Less common:
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Scleritis
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Episcleritis
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Filamentary keratitis
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Neovascularization of iris
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From posterior inflammation
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Poor prognostic sign
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Posterior Segment
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Posterior or panuveitis
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Vitritis with acute inflammation
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Retinal and/or vitreous hemorrhage
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Venous and capillary dilatation
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Obliterative necrotizing retinal vasculitis (RV)
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May involve arteries and veins simultaneously (also capillaries)
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Ghost vessels, “silver-wired” vessels
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CRVO or BRVO
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CRAO or BRAO
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NVE, NVD
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CME
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Chorioretinal scarring
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Retinal tears and detachment
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Papillitis, later progressive optic atrophy
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Neovascular glaucoma
Exam: Systemic
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Oral aphthous ulcers, required for diagnosis
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Skin
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Erythema nodosum
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Hyperpigmented/hypopigmented scarring
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Pathergy (40%)
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Acne vulgaris or folliculitis, on thorax or face
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Vasculitis (8–38%)
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Any vessels (arteries, veins, capillaries), any size
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Superficial thrombophlebitis, upper or lower extremities
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Neurologic (3–10%, neurologic or vascular in origin)
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Cranial nerve palsies (CN VI, CN VII, transient)
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Papillitis, papilledema
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Audiovestibular dysfunction
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Venous sinus thromboses, intracranial hypertension
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Pyramidal brainstem lesions
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Seizures
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Psychiatric disorders
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Genitourinary
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Ulcers
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Epididymitis
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Glomerulonephritis
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IgA nephropathy
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Amyloidosis
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Renal vein thrombosis
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Gastrointestinal
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Diarrhea
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Hemorrhages
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Ulcers in esophagus, stomach, intestine; may perforate
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Pulmonary (18%)
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Hemoptysis, dyspnea, chest pain, fever, cough
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Vascular lesions, pulmonary emboli
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Aneurysmal bronchial fistula
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Musculoskeletal
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Arthritis – knee, sacroiliitis, ankylosing spondylitis, non-migrating
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Imaging
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OCT: CME, CNV, macular atrophy (after vascular insult)
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FA: CME, retinal vasculitis (may see arteritis, phlebitis, and/or capillaritis), papillitis, vascular occlusion/delay, neovascularization, chorioretinitis
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Diffuse dye leakage may be seen after inflammation subsides
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ICG: hypocyanescent choroidal lesions
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ERG: decreases in overall standard and pattern ERG
Laboratory and Radiographic Testing
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No definitive serologic or laboratory testing
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May be elevated acute phase reactant proteins: ESR, CRP, complement
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HLA-B51 association (not diagnostic)
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Elevated soluble CD25 may precede recurrence
Differential Diagnosis
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HLA-B27 associated uveitis
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Reactive arthritis
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Sarcoidosis
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Systemic lupus erythematosus
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ANCA vasculitides
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Viral retinitis
Treatment
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Acute AU: frequent topical corticosteroid +/− cycloplegia
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Q1h steroid and atropine 1% BID with hypopyon
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Severe AU or posterior involvement
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Requires aggressive and urgent therapy
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Systemic corticosteroids, oral or intravenous (or both)
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Initiation of immunomodulatory therapy
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May coordinate with other specialists
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Immunomodulatory therapy
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Antimetabolites
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Azathioprine or mycophenolate
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Calcineurin inhibitors
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Cyclosporine may supplement antimetabolite therapy
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Biologics (especially with RV)
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TNFα inhibitors – adalimumab, infliximab
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CD20 inhibition – rituximab
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Anti-IL1β – anakinra, canakinumab (used in some cases)
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Alkylating agents
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Chlorambucil or cyclophosphamide
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Other therapeutic measures
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Colchicine
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Plasmapheresis
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Interferon α-2a
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Dapsone
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Pendoxyphilline
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Penicillin
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Thalidomide
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Referral/Co-management
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Rheumatology
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Cardiology
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Neurology
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Dermatology
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ENT
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Gastroenterology
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Urology
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Pulmonology
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Kaya, M. (2021). Adamantiades-Behçet’s Disease. In: Foster, C.S., Anesi, S.D., Chang, P.Y. (eds) Uveitis. Springer, Cham. https://doi.org/10.1007/978-3-030-52974-1_7
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DOI: https://doi.org/10.1007/978-3-030-52974-1_7
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