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A Contemporary Review of Behcet’s Syndrome

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Abstract

Behcet’s syndrome (BS) is a chronic systemic inflammatory vasculitis with a wide range of clinical manifestations including recurrent oral and genital ulcers; cutaneous lesions; and ophthalmic, neurologic, and gastrointestinal involvement. BS has a global distribution but is particularly prevalent in so-called Silk Road populations. Disease onset is usually around the third or fourth decade of life, and the sex ratio is roughly 1:1. Both environmental and genetic factors contribute to the etiology of BS, although the detailed mechanisms remain unclear. At present, there is no laboratory examination with diagnostic value for BS; therefore, a diagnosis is made based on clinical manifestations. The International Study Group diagnostic criteria published in 1990 is the most widely used and recognized, but in order to improve sensitivity, the International Criteria for Behcet’s Disease is developed in 2014. Evaluating disease activity in BS is an important basis for treatment selection and monitoring, the simplified Behcet’s Disease Current Activity Form (2006 version) is a well-established scoring method. Given that multiple organs are affected in BS, it must be differentiated from other diseases with similar manifestations or that may be induced by drug treatment. The goal of BS treatment is to eradicate triggers and/or aggravating factors, alleviate and control clinical symptoms, prevent and treat any damage to organs, slow disease progression, and improve the patient’s quality of life. The clinical management of BS depends on the affected organs and disease severity. In this review, we summarize the current state of knowledge of BS pathogenesis and therapeutic options.

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Abbreviations

5-ASA:

5-Aminosalicylic acid

ADA:

Adalimumab

AZA:

Azathioprine

BS:

Behcet’s syndrome

BDCAF:

Behcet’s Disease Current Activity Form

BU:

Behcet’s uveitis

CSA:

Cyclosporine A

CTX:

Cyclophosphamide

DVT:

Deep venous thrombosis

EN:

Erythema nodosum

GC:

Glucocorticoid

HA20:

Haploinsufficiency of A20

ICBD:

International Criteria for Behcet’s Disease

IFN-α:

Interferon alpha

IFX:

Infliximab

IL:

Interleukin

ISG:

International Study Group

MMF:

Mycophenolate

MTX:

Methotrexate

NBS:

Neuro-Behcet’s syndrome

OPT:

Oral pathergy test

PD-1:

Programmed death 1

PT:

Pathergy test

SPT:

Skin pathergy test

THD:

Thalidomide

TNF-α:

Tumor necrosis factor alpha

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The authors received financial support from The Nanjing Incubation Program for National Clinical Research Center (grant no. 2019060001).

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  1. Department of Allergy and Rheumatology, Hospital for Skin Diseases, Institute of Dermatology, Chinese Academy of Medical Sciences, Peking Union Medical College, 12 Jiangwangmiao Street, Nanjing, 210042, China

    Jingjing Chen & Xu Yao

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Chen, J., Yao, X. A Contemporary Review of Behcet’s Syndrome. Clinic Rev Allerg Immunol 61, 363–376 (2021). https://doi.org/10.1007/s12016-021-08864-3

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