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Behçet's Disease

Abstract

Behçet’s disease is an inflammatory disorder characterized by recurrent oral aphthae, genital ulcers, uveitis, and skin lesions such as erythema nodosum. Although mucocutaneous manifestations are self-limiting, ocular involvement can cause blindness. Involvement of large vessels, gastrointestinal tract, and central nervous system is less frequent but can be life-threatening and leave irreversible damage.

Because of heterogeneity of clinical manifestations, a number of sets of diagnostic or classification criteria have been proposed and are still being discussed. The most common causes of Behçet’s disease related death are the arterial lesions, especially pulmonary arterial aneurysm, followed by chronic progressive neurological involvement. Introduction of anti-TNF mAb has greatly contributed to improvement of visual prognosis. Treatment with biologics, including TNF blockers, is also promising for other serious clinical subtypes.

Although the etiology remains unknown, both genetic and environmental factors are implicated in the development of the disease. In addition to the unique geographic distribution and familial aggregation of BD patients, recent gene-wide association studies and subsequent detail analyses have identified novel susceptible genes which are related to the immune system besides HLA-B51.

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  1. Department of Internal Medicine and Clinical Immunology, Yokohama City University Graduate School of Medicine, 3-9 Fukuura, Kanazawa-Ku, Yokohama, 236-0004, Japan

    Yoshiaki Ishigatsubo M.D., Ph.D. & Mitsuhiro Takeno

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  1. Yoshiaki Ishigatsubo M.D., Ph.D.
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    Yoshiaki Ishigatsubo

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Ishigatsubo, Y., Takeno, M. (2015). Overview. In: Ishigatsubo, Y. (eds) Behçet's Disease. Springer, Tokyo. https://doi.org/10.1007/978-4-431-54487-6_1

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