© Springer Nature Switzerland AG 2021

C. S. Foster et al. (eds.)Uveitishttps://doi.org/10.1007/978-3-030-52974-1_7

7. Adamantiades-Behçet’s Disease

Mahmut Kaya¹  

(1)

Department of Ophthalmology, Dokuz Eylul University, Izmir, Turkey

 

Keywords

Adamantiades-Behçet’s diseaseUveitis

Overview

• Definition

  • A chronic, relapsing inflammatory disorder of unknown etiology with classic triad findings

    • Recurrent oral and genital aphthous ulcers

    • Ocular inflammation

    • Skin lesions

  • International criteria:

    • Behçet’s Research Committee of Japan

      • Complete, incomplete, suspect, possible

    • International Study Group for Behçet’s Disease Criteria

      • Oral ulcers +2 of: genital ulcers, eye lesions, skins lesions, pathergy test

  • Frequently involves CNS and GI tract as well

  • Ocular inflammation in 67–95%

    • Anterior uveitis

    • Devastating retinal vasculitis

• Symptoms

  • Blurring

  • Scotomas

  • Redness

  • Periorbital pain

  • Photophobia

  • Tearing with rare ocular discharge

  • Diplopia (with neurologic involvement)

• Laterality

  • Unilateral progressing to bilateral, 80%

• Course

  • Recurrent inflammation, not typically chronic

  • Ocular flares are often severe

• Age of onset

  • 25–35 years worldwide (range 2 months to 72 years)

• Gender/race

  • Historically M > F, may be more even distribution

  • Most common in Eastern Mediterranean and East Asian

• Systemic association

  • Systemic vasculitis

  • Oral and/or genital aphthous ulcers

  • Various mild to severe organ involvement including skin, heart, CNS, GI, lungs, GU, joints

Exam: Ocular

Anterior Segment

• Acute anterior uveitis (AU):

  • Non-granulomatous

  • May progress to “shifting” hypopyon if untreated, 19–31%

• More common:

  • Cataract

  • Posterior synechiae

  • Peripheral anterior synechiae

  • Iris atrophy

• Less common:

  • Scleritis

  • Episcleritis

  • Filamentary keratitis

  • Neovascularization of iris

    • From posterior inflammation

    • Poor prognostic sign

Posterior Segment

• Posterior or panuveitis

  • Vitritis with acute inflammation

• Retinal and/or vitreous hemorrhage

• Venous and capillary dilatation

• Obliterative necrotizing retinal vasculitis (RV)

  • May involve arteries and veins simultaneously (also capillaries)

  • Ghost vessels, “silver-wired” vessels

  • CRVO or BRVO

  • CRAO or BRAO

  • NVE, NVD

• CME

• Chorioretinal scarring

• Retinal tears and detachment

• Papillitis, later progressive optic atrophy

• Neovascular glaucoma

Exam: Systemic

• Oral aphthous ulcers, required for diagnosis

• Skin

  • Erythema nodosum

  • Hyperpigmented/hypopigmented scarring

  • Pathergy (40%)

  • Acne vulgaris or folliculitis, on thorax or face

• Vasculitis (8–38%)

  • Any vessels (arteries, veins, capillaries), any size

  • Superficial thrombophlebitis, upper or lower extremities

• Neurologic (3–10%, neurologic or vascular in origin)

  • Cranial nerve palsies (CN VI, CN VII, transient)

  • Papillitis, papilledema

  • Audiovestibular dysfunction

  • Venous sinus thromboses, intracranial hypertension

  • Pyramidal brainstem lesions

  • Seizures

  • Psychiatric disorders

• Genitourinary

  • Ulcers

  • Epididymitis

  • Glomerulonephritis

  • IgA nephropathy

  • Amyloidosis

  • Renal vein thrombosis

• Gastrointestinal

  • Diarrhea

  • Hemorrhages

  • Ulcers in esophagus, stomach, intestine; may perforate

• Pulmonary (18%)

  • Hemoptysis, dyspnea, chest pain, fever, cough

  • Vascular lesions, pulmonary emboli

  • Aneurysmal bronchial fistula

• Musculoskeletal

  • Arthritis – knee, sacroiliitis, ankylosing spondylitis, non-migrating

Imaging

• OCT: CME, CNV, macular atrophy (after vascular insult)

• FA: CME, retinal vasculitis (may see arteritis, phlebitis, and/or capillaritis), papillitis, vascular occlusion/delay, neovascularization, chorioretinitis

  • Diffuse dye leakage may be seen after inflammation subsides

• ICG: hypocyanescent choroidal lesions

• ERG: decreases in overall standard and pattern ERG

Laboratory and Radiographic Testing

• No definitive serologic or laboratory testing

• May be elevated acute phase reactant proteins: ESR, CRP, complement

• HLA-B51 association (not diagnostic)

• Elevated soluble CD25 may precede recurrence

Differential Diagnosis

• HLA-B27 associated uveitis

  • Reactive arthritis

• Sarcoidosis

• Systemic lupus erythematosus

• ANCA vasculitides

• Viral retinitis

Treatment

• Acute AU: frequent topical corticosteroid +/− cycloplegia

  • Q1h steroid and atropine 1% BID with hypopyon

• Severe AU or posterior involvement

  • Requires aggressive and urgent therapy

  • Systemic corticosteroids, oral or intravenous (or both)

  • Initiation of immunomodulatory therapy

    • May coordinate with other specialists

• Immunomodulatory therapy

  • Antimetabolites

    • Azathioprine or mycophenolate

  • Calcineurin inhibitors

    • Cyclosporine may supplement antimetabolite therapy

  • Biologics (especially with RV)

    • TNFα inhibitors – adalimumab, infliximab

    • CD20 inhibition – rituximab

    • Anti-IL1β – anakinra, canakinumab (used in some cases)

  • Alkylating agents

    • Chlorambucil or cyclophosphamide

• Other therapeutic measures

  • Colchicine

  • Plasmapheresis

  • Interferon α-2a

  • Dapsone

  • Pendoxyphilline

  • Penicillin

  • Thalidomide

Referral/Co-management

• Rheumatology

• Cardiology

• Neurology

• Dermatology

• ENT

• Gastroenterology

• Urology

• Pulmonology