Behçet's syndrome

It may be asked why rheumatologists have interested themselves in Behçet's syndrome (BS). The obvious answer must be that BS is a vasculitis and a common feature with which it may present is an inflammatory arthritis. Most papers on BS start with a statement that it is a multi-system inflammatory disease of uncertain aetiology, now classified as a vasculitis. It has been shown to be uncommon in western Europe and the USA, while being much more prevalent in the eastern Mediterranean countries, South East Asia and Japan in particular. Young onset (before the age of 25 yr) and males have been shown to have more severe disease [1, 2]. Although the commonest age of development is in the third decade of life, children may be affected with apparently identical features [3]. It may seem curious that there remains a debate on which terminology, Behçet's syndrome (BS) or Behçet's disease (BD), is preferable. Although these two terms are sometimes thought to be readily exchangeable they are also the subject of debate and disagreement. Some colleagues regard BD as a specific disease and should be accepted as such. To others, including the authors, the lack of a known pathogenesis or a definitive diagnostic test, and the variability in prevalence and incidence of the condition and its constituent manifestations, suggest that the terminology BS is preferable. As with many eponymous titles Behçet was not the first to describe the condition; that can probably be credited to Hippocrates in the 5th century bc , while descriptions of almost certainly the same condition were published from Europe and the Far East in the early 20th century [4–14].