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Textbook of Autoinflammation pp 647–665Cite as

Behçet Disease

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Abstract

Behçet disease, a multifactorial systemic inflammatory disorder of unknown etiology, is characterized by recurrent oral and genital aphthous ulcers, and uveitis as cardinal features, and also other manifestations involving the skin and mucosal tissues, eyes, joints, blood vessels, lungs, central nervous and gastrointestinal systems. Some of its recurrent manifestations overlap with the clinical findings of autoinflammatory disorders. However bilateral posterior or panuveitis with typical features, variable vessel vasculitis with a preference for the venous side, and parenchymal neurologic involvement as subacute brainstem syndrome constitute its distinctive clinical findings. Behçet disease is strongly associated with human leukocyte antigen (HLA)-B*51, and an epistatic interaction between HLA-B*51 and one of the endoplasmic reticulum-associated aminopeptidase 1 (ERAP1) haplotypes implicate the critical role of peptide-HLA complex in the disease pathogenesis. Additional non-HLA genetic variants associated with Behçet disease contribute to the changes in sensitivity to microbial and other environmental triggers resulting in a hyperinflammatory response involving mainly innate immunity, Th1 and Th17 type adaptive response and endothelial activation. Treatment of Behçet disease is empiric, and should be tailored according to the severity of manifestations. Clinical findings and their recurrences can be managed by anti-inflammatory and immunosuppressive drugs, including corticosteroids, colchicine, apremilast, azathioprine, cyclosporine as well as monoclonal anti-tumor necrosis factor agents and interferon α, and when necessary with other targeted biologic treatments.

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Abbreviations

5-ASA:

5-Aminosalicylate

ASC:

Apoptosis-associated speck-like protein containing CARD

CAPS:

Cryopyrin-associated periodic syndrome

CARD:

Caspase activation and recruitment domains

ERAP:

Endoplasmic reticulum aminopeptidase

FMF:

Familial Mediterranean fever

HA20:

Haploinsufficiency of A20

HIV:

Human immunodeficiency virus

HLA:

Human leukocyte antigen

hnRNP:

Heterogeneous nuclear ribonucleoprotein

IFN:

Interferon

IL:

Interleukin

JAK:

Janus kinase

MHC:

Major histocompatibility complex

MKD:

Mevalonate kinase deficiency

NF-M:

Neurofilament-medium

NK:

Natural killer

NLRP3:

Nucleotide-binding oligomerization domain, leucine rich repeat and pyrin domain containing 3

PAPA:

Pyogenic arthritis, pyoderma gangrenosum and acne

STAT:

Signal transducer and activator of transcripton

TNF:

Tumor necrosis factor

TRAPS:

Tumor necrosis factor receptor-associated periodic syndrome

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Authors and Affiliations

  1. Division of Rheumatology, Department of Internal Medicine, Istanbul Faculty of Medicine, Istanbul University, Istanbul, Turkey

    Ahmet Gül

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  1. Ahmet Gül
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Editors and Affiliations

  1. Pediatric Rheumatology Unit, Shaare Zedek Medical Center and Hebrew University, Jerusalem, Israel

    Philip J. Hashkes

  2. Department Paediatrics, Division of Rheumatology, The Hospital for Sick Children and University of Toronto, Toronto, ON, Canada

    Ronald M. Laxer

  3. Department of Internal Medicine, Radboudumc Expertisecenter for Immunodeficiency and Autoinflammation, Radboud University Medical Center, Nijmegen, The Netherlands

    Anna Simon

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Gül, A. (2019). Behçet Disease. In: Hashkes, P., Laxer, R., Simon, A. (eds) Textbook of Autoinflammation. Springer, Cham. https://doi.org/10.1007/978-3-319-98605-0_35

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