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For this meeting of two Working Groups of the ISBD, the background to our present knowledge is reviewed, including the following: the original description of Behçet's disease (BD) by Hippocrates in the 5th century BC and the 20th century description with the adoption of the eponymous title of 'Behçet's disease'; the lack of a specific diagnostic test but noting the pathergy test and the association with HLA-B51; description of the multisystem manifestations of the disease and the prevalence of these manifestations, including the differences in different parts of the world; and the pathological classification of BD as a vasculitis.

Nevertheless the diagnosis and classification of the condition depends on the acumen of the physician in diagnosis of the individual patient in the routine clinical situation, and the classification of groups of patients with the disease for inclusion into clinical studies and trials. The older 'diagnostic schemes' (e.g. Japanese, Mason and Barnes, O'Duffy, Dilsen) should be discarded and the International Classification of BD used as an entry criterion for clinical studies and trials.

The natural history of BD still requires further study with particular reference to being able to predict the type of disease (mucocutaneous, ocular, neuro, etc.), the localization of manifestations, the likelihood of recurrences and their duration, and the overall severity of the disease in the individual patient.

Basic research – pathogenesis (?bacterial), immunology (e.g. CD4+CD28- T cells) and gender association (prevalence, severity and response to treatment) – must continue and may proceed more quickly if coordinated on a multicentre, multinational basis. Therapeutic trials continue but to date there are surprisingly few controlled studies (e.g. colchicine, azathioprine and interferon-α). It is imperative that future trials be properly controlled, and open trials reserved for very preliminary 'pilot' studies. Controlled trials (either single or double blind; versus placebo or other comparison drug) are required at present, in particular for the following: IFN-α – optimum dosage regimen with regard to both efficacy and tolerance (side effects); anti-TNF-α, and possibly of antibiotics. Again these may be progressed faster on a coordinated multicentre, multinational basis but with the prior determination of updated entry criteria and outcome measures.

The ISBD, as a medical research society, and through its Working Groups, should be a vehicle for the organization and coordination of studies – single centre, multicentre in a single country, or multi-centre on a multinational basis. The Working Groups for Clinical Trials and Treatment and for Basic Research should work towards the coordination of clinical studies and trials with agreed entry criteria – diagnostic classification and disease manifestations and activity; outcome measures; and protocols, including statistical methodology. Multinational coordination through the ISBD may lead to the necessary availability of appropriate funding and secretarial assistance for such trials and studies.

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Barnes, C. Introduction. Arthritis Res Ther 5 (Suppl 2), 1 (2003).

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