Our results provide further evidence of the genetic components of ABD and the significance of the specific onset sign concerning the prognosis of the disease. The combination of vascular onset sign (ocular changes, erythema nodosum, and thrombophlebitis) with male gender is significantly associated with the occurrence of a severe course. In patients with a non-vascular onset sign (oral aphthous ulcers, genital ulcers or articular involvement), positivity is a negative prognostic marker (Fig. 1).

Multinational multicentre studies should be established to investigate the impact of these components

Figure 1. Risk factors for severe course of Adamantiades-Behget's disease

Male gender is a widely accepted risk factor for severe course of ABD2-5. On the other hand, non-aphthous ulcers onset has also been reported to be a risk factor by Dilsen4 HLAB5! is not associated with the disease itself1 but has been disputed as a prognostic factor1'5'8. This study has found HLAB51 to be, indeed, a risk factor for the majority of patients who develop the disease with a non-vascular onset sign, thus confirming our previous data1,6. Other demographic and clinical data, such as age of onset4, or skin lesions, arthritis, posterior attacks, and other complications could not be identified as risk factors for developing a severe course of ABD in our patients.

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