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Behçet’s disease

20 November 2012, by SAADOUN D. & WECHSLER B.

Behcet’s disease (BD) is a chronic and relapsing vasculitis characterized by oral and genital ulcerations, ocular inflammation with cutaneous, vascular and nervous system manifestations. International classification criteria for BD [1] (Table I) defined in 1990 are based on clinical characteristics, including oral and genital ulcerations and ocular involvement. The Pathergy test is not very sensitive [2]. Visceral involvements (neurological, cardiac and vascular involvements) are the determining factors of severity of the disease.

BD is particularly prevalent in Mediterranean and Japanese countries. However, BD can be found all over the world [3, 4]. Symptoms onset usually occur between 18 and 40 years old, even if paediatric cases have been described [5].

The pathogeny is not well understood to date. Genetic factors are implicated since increase of HLA-B51 antigen and of MICA A allele are observed in BD patients and a recent GWAS study has shown an association between the disease and single nucleotide polymorphism of interleukin (IL) 10, IL-12 and IL-23 receptors [6, 7]. Underlying infectious factors (notably streptococcus sanguis or herpes virus) may possibly participate to the pathogenesis of the disease [8]. Recently, a marked increase in Th17 cells and a decrease of regulatory T cells have been shown [9]. This study also established the critical role of IL-21 in driving inflammatory lesions by promoting Th17 effectors and by suppressing regulatory T cells.

The outcome of the disease can be erratic with recurrent flares of disease. The main risks are increase mortality with large vascular involvements, and a high morbidity related to sequelae of neurological and ocular involvements [10]. The treatment depends on disease manifestations.

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