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Adamantiades–Behçet’s Disease

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Albert and Jakobiec's Principles and Practice of Ophthalmology

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Abstract

Behcet’s disease is an immune complex disease characterized by occlusive vasculitis. The disease is most prevalent in the Middle East and the Far East populations but occurs worldwide. The etiology of the disease is multifactorial: infectious agents, immunological mechanisms, and a genetic predisposition, with HLA-B51 as one known risk factor. Behcet’s disease can involve blood vessels of nearly all types, including small and large arteries. Therefore, it can affect several organs, including the eyes, mouth, skin, joints, lungs, genitals, and gastrointestinal tract. Diagnosis is based on clinical findings, including recurrent oral ulcerations, evidence of anterior or posterior uveitis, retinal vasculitis, and recurrent genital ulceration. Ocular disease has serious implications and can lead to legal blindness within 4 years if left untreated. Treatment is anti-inflammatory medications such as corticosteroids, cytotoxic agents, cyclosporine, and colchicine. The key to vision preservation is prompt, aggressive therapy. Early treatment suppresses inflammation, reduces the frequency and severity of recurrences, and can halt any involvement of the retina and optic nerve.

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Authors and Affiliations

  1. Massachusetts Eye Research and Surgery Institution, Waltham, MA, USA

    Charles Stephen Foster & Ketaki Panse

  2. Ocular Immunology and Uveitis Foundation (OIUF), Waltham, MA, USA

    Charles Stephen Foster

  3. Department of Ophthalmology, Harvard Medical School, Boston, MA, USA

    Charles Stephen Foster

  4. Midwest Retina, Columbus, OH, USA

    Dino D. Klisovic

  5. Ocular Immunology and Uveitis Foundation, Waltham, MA, USA

    Ketaki Panse

Authors
  1. Charles Stephen Foster
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  2. Ketaki Panse
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  3. Dino D. Klisovic
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Correspondence to Charles Stephen Foster .

Editor information

Editors and Affiliations

  1. Casey Eye Institute, Oregon Health & Science, Portland, OR, USA

    Daniel M. Albert

  2. Department of Ophthalmology, Massachusetts Eye and Ear, Massachusetts General Hospital, Brigham and Women’s Hospital, Harvard Medical School, Boston, MA, USA

    Joan W. Miller

  3. Department of Ophthalmology, University of Illinois College of Medicine, University of Illinois at Chicago, Chicago, IL, USA

    Dimitri T. Azar

  4. Department of Ophthalmology, Massachusetts Eye and Ear, Harvard Medical School, Boston, MA, USA

    Lucy H. Young

Section Editor information

  1. Penn State College of Medicine, Penn State Eye Center, Hershey, Pennsylvania, USA

    Jeffrey M. Sundstrom

  2. Department of Ophthalmology, Penn State College of Medicine, Hershey, Pennsylvania, USA

    David A. Quillen

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Foster, C.S., Panse, K., Klisovic, D.D. (2022). Adamantiades–Behçet’s Disease. In: Albert, D.M., Miller, J.W., Azar, D.T., Young, L.H. (eds) Albert and Jakobiec's Principles and Practice of Ophthalmology. Springer, Cham. https://doi.org/10.1007/978-3-030-42634-7_301

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