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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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.
The authors affirm that patients provided informed consent for publication of the images in Fig. 3.
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A small-molecule inhibitor of PDE4.
Inflammation of the epididymis, a tube at the back of the testicle that carries sperm.
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.
Inflammation of the subcutaneous fat.
A disorder of the entheses, which are the connective tissues between bones and tendons or ligaments.
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.
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.
Accumulation of inflammatory cells in the anterior chamber of the eye.
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.
Weakness of one side of the body.
Inability to speak due to damage to the part of the brain responsible for speaking.
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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