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Behçet syndrome

Nature Reviews Disease Primers volume 7, Article number: 67 (2021) Cite this article

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Abstract

Behçet syndrome is a systemic vasculitis with an unknown aetiology affecting the small and large vessels of the venous and arterial systems. The presence of symptom clusters, regional differences in disease expression and similarities with, for example, Crohn’s disease suggest that multiple pathological pathways are involved in Behçet syndrome. These disease features also make formulating disease criteria difficult. Genetic studies have identified HLA-B*51 as a genetic risk factor. However, the low prevalence of HLA-B*51 in many patients with bona fide disease, especially in non-endemic regions, suggests that other factors must also be operative in Behçet syndrome. Despite lacking a clear aetiological mechanism and definition, management of manifestations that include major vascular disease, eye disease and central nervous system involvement has improved with the help of new technology. Furthermore, even with our incomplete understanding of disease mechanisms, the prognoses of patients with Behçet syndrome, including those with eye disease, continue to improve. New treatment options and a better understanding of the underlying pathogenesis for various manifestations of this condition are required to further improve the management of the disease, which will improve patient quality of life.

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Fig. 1: Prevalence of Behçet syndrome.
Fig. 2: Schematic representation of skewed helper T cell responses observed in patients with Behçet syndrome.
Fig. 3: Mucocutaneous manifestations of Behçet syndrome.
Fig. 4: Ocular manifestations of Behçet syndrome.
Fig. 5: Pulmonary artery aneurysm in Behçet syndrome as seen on conventional radiography and CT.

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Authors and Affiliations

  1. Division of Rheumatology, New York University School of Medicine, New York, NY, USA

    Yusuf Yazici

  2. Division of Rheumatology, Department of Internal Medicine and Behçet’s Disease Research Center, Istanbul University — Cerrahpasa, Istanbul, Turkey

    Gulen Hatemi

  3. Sorbonne University, IHU FOReSIGHT, Pitié-Salpêtrière Hospital UMR 7211 UPMC/CNRS, U972 INSERM, Transimmunom Laboratory of Excellence Sorbonne University, Paris, France

    Bahram Bodaghi

  4. Department of Internal Medicine, Yonsei University College of Medicine, Seodaemun-gu, Seoul, Korea

    Jae Hee Cheon

  5. Department of Immunology and Medicine, St. Marianna University School of Medicine, Miyamae-ku, Kawasaki, Japan

    Noburu Suzuki

  6. University College London, London, UK

    Nicola Ambrose

  7. (Rheumatology) Academic Hospital Istanbul, Istanbul, Turkey

    Hasan Yazici

Authors
  1. Yusuf Yazici
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  2. Gulen Hatemi
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  3. Bahram Bodaghi
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  4. Jae Hee Cheon
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  5. Noburu Suzuki
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  6. Nicola Ambrose
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  7. Hasan Yazici
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Contributions

Introduction (H.Y.); Epidemiology (Y.Y.); Mechanisms/pathophysiology (N.A. and N.S.); Diagnosis, screening and prevention (Y.Y., G.H., B.B. and J.H.C.); Management (G.H., B.B. and J.H.C.); Quality of life (Y.Y. and G.H.); Outlook (H.Y.); Overview of Primer (Y.Y., H.Y. and G.H.).

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Correspondence to Yusuf Yazici.

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Competing interests

Y.Y. has received consulting fees from Amgen and research support from Amgen, BMS and Genentech. G.H. has received research grant, lecture fees and fees for serving on an advisory board from Celgene; consulting fees from Bayer, Johnson & Johnson and UCB Pharma; and lecture fees from Abbvie, Amgen, Novartis and UCB Pharma. B.B. has received consulting fees from AbbVie/Allergan, Alimera, Horus Pharma, Novartis, Santen and Thea. All other authors declare no competing interests.

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Nature Reviews Disease Primers thanks E. Giacomo, M. Stanford, P. Yang, I. Kötter, S. Hou, L. Cantarini and the other, anonymous, reviewer(s) for their contribution to the peer review of this work.

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Glossary

Apremilast

A small-molecule inhibitor of PDE4.

Epididymitis

Inflammation of the epididymis, a tube at the back of the testicle that carries sperm.

Endocarditis

Inflammation of the inner layer of the heart.

Reiter’s syndrome

An inflammatory syndrome typically presenting with conjunctivitis, urethritis and arthritis.

MAGIC syndrome

A rare condition with oral and genital ulcers and cartilage inflammation.

Sweet syndrome

A skin condition with neutrophilic infiltrations and fever.

Erythema multiforme

A group of hypersensitivity disorders characterized by symmetrical red, patchy lesions, primarily on the arms and legs.

Pyoderma gangrenosum

Non-infectious inflammatory, neutrophilic skin disease characterized by ulcerating lesions.

Erythema nodosum

An inflammatory panniculitis characterized by inflammation of the fat cells under the skin, resulting in tender red nodules or lumps that are usually seen on both shins.

Superficial thrombophlebitis

Blood clots of the small blood vessels on the skin.

Panniculitis

Inflammation of the subcutaneous fat.

Enthesopathy

A disorder of the entheses, which are the connective tissues between bones and tendons or ligaments.

Episcleritis

An inflammatory condition affecting the episcleral tissue between the conjunctiva (the clear mucous membrane lining the inner eyelids and sclera) and the sclera (the white part of the eye) that occurs in the absence of an infection.

Uveitis

Inflammation of the uvea, which is the pigmented layer that lies between the inner retina and the outer fibrous layer composed of the sclera and cornea in the eye.

Hypopyon

Accumulation of inflammatory cells in the anterior chamber of the eye.

Papillitis

Inflammation of the optic nerve head.

Retrobulbar optic neuritis

A form of optic neuritis in which the optic nerve that is at the back of the eye becomes inflamed.

Budd–Chiari syndrome

A condition caused by the occlusion of the hepatic veins.

Hemiparesis

Weakness of one side of the body.

Dysarthria

Inability to speak due to damage to the part of the brain responsible for speaking.

Ataxia

A neurological sign consisting of lack of voluntary coordination of muscle movements that can include gait abnormalities, speech changes and abnormalities in eye movements.

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Yazici, Y., Hatemi, G., Bodaghi, B. et al. Behçet syndrome. Nat Rev Dis Primers 7, 67 (2021). https://doi.org/10.1038/s41572-021-00301-1

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