3Behçet's disease
Section snippets
Epidemiology and pathogenesis
The geographical distribution of Behçet's disease is distinctive: it is most prevalent along the Silk Road, an ancient trading route between the Mediterranean and East Asia, where it is a major cause of morbidity.2 In Turkey, the country with the highest incidence of the disease, the prevalence is estimated to be between 110 and 420 per 100 0002., 3., whereas that in Japan is 13–20 per 100 000, and the prevalence in the UK and USA is estimated at 1–2 per 100 000.4 It is unknown among
Pathology
The common histopathological lesion underlying the clinical manifestations of Behçet's disease is vasculitis, involving particularly the venules. Lesions are characterized by perivascular lymphocytic and monocytic cellular infiltration, with or without fibrin deposition in the vessel wall and surrounding tissue necrosis.59 Significant neutrophil infiltration is also seen, particularly in early lesions including those of the pathergy reaction.60
Active systemic disease is associated with many
Recurrent aphthous ulceration
Recurrent aphthous ulceration is the sine qua non of Behçet's disease. Oral ulcers are usually the earliest sign of disease and may precede the onset of systemic symptoms by many years. Oral ulcers are similar to common mouth ulcers in appearance and localization, although they may be more extensive and painful, evolving quickly from a flat ulcer to a large sore. Lesions may occur singly or in crops, and subside without scarring. The most common sites of oral ulceration are the tongue, lips and
Pregnancy
Pregnancy is often stated to be associated with disease remission, but its influence is variable, and in 20% of patients pregnancy is associated with disease exacerbation.89 No increase in the complications of pregnancy have been observed.90 Transient neonatal Behçet's disease in the infants of affected mothers has, however, been described, suggesting that disease may be mediated by a placental transfer of maternal antibody.174., 175.
Children
Behçet's disease is uncommon in children. It affects both
Disease definition
There is no specific test for Behçet's disease, and the diagnosis is based upon clinical criteria. A number of classifications have been formulated, each with its own list of clinical features.93., 94., 95., 96. In1990, these were amalgamated into the International Study Group Classification criteria (Table 1).97 These have now been widely adopted, and although intended for the definition of patients participating in research programmes rather than for the diagnosis of individual patients, they
Treatment
Although the number of randomized controlled trials in Behçet's disease is increasing, treatment remains predominantly empirical, and considerable differences exist in practical approaches to treatment. The primary goals of management are symptom control, early suppression of inflammation and prevention of end-organ damage, the treatment options being anti-inflammatory agents and immunosuppressants. Drugs are frequently used in combination in order to maximize efficacy while minimizing side
Topical treatment
Oral ulceration can often be treated by the topical application of corticosteroids, using creams or mouthwashes (5 mg prednisolone in 20 ml water, four times daily102), or by direct application through a conventional corticosteroid inhaler. Similarly, genital ulceration often responds to topical corticosteroid therapy, although long-term use may be complicated by skin atrophy. Topical sucralfate suspension is an alternative topical therapy for aphthous ulceration.103 Topical mydriatic agents
Prognosis
Behçet's disease is characterized by relapses and remission, and its clinical course is highly variable. Overall, the disease is more severe in Mediterranean and Eastern cohorts than in Western populations, and it is generally more severe in males than females. A recently published, 20-year outcome study of 387 Turkish patients revealed an overall mortality of 9.8% in Behçet's disease.70 This is higher than previously reported76 and probably reflects the long duration of follow-up and the
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2021, Pharmacological ResearchCitation Excerpt :Moreover, the existence of a polygenic substrate highlights the similarities with the classical autoimmune conditions, although different loci seem to be involved. Although other loci within the MHC region have been proposed, including MICA genes and other non-classical MHC genes such as TNF gene variants, these results must be interpreted with caution due to the strong linkage disequilibrium that exists within the MHC region [16]. The results of genome-wide association studies (GWAS) have demonstrated an association between BD and ERAP1 polymorphisms [17,18].
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