Behçets disease in children.

Ophthalmic and clinical analysis were carried out on 16 children and 122 adult patients affected by Behçet's disease (BD) to delineate the clinical features of BD in childhood and to investigate the differences between the expression of the disease in children and adults. The mean follow-up period was 7.8 and 7 years, respectively. Pediatric onset of BD was found in 7.6% of all the cases with a male:female ratio of 1.29:1. The complete type of the disease was observed in 50% of the children. No statistical significant differences were noted between children and adults in the incidence of oral aphthae, genital ulcers, skin lesions, arthritis, gastrointestinal involvement, neuropsychiatric symptoms and the presence of HLA-B51. Thrombophlebitis was associated with the onset of the disease in adult age (P=0.022). Uveitis alone or in combination with other major symptoms was the presenting sign in a higher percentage of children (P=0.077), As in adults, in children diffuse uveitis was the most common type of ocular inflammation, while ocular complications have been found mainly in children (P=0.021), who more frequently developed cataract, maculopathy and retinal detachment (P=0.024). Both adult and young male patients have shown a lower age at onset and higher rate of optic atrophy than females. In conclusion, no significant differences have been found between children and adults in the expression of the major and most of the minor symptoms of BD. Ocular involvement in childhood may be very severe, as was confirmed by the high frequency of diffuse uveitis and ocular complications. Young males, as adult males, showed an earlier onset of the disease and a worse ocular prognosis.