Clinical features of juvenile Adamantiades-Behcet's disease in Greece

Objective Adamantiades-Behcet's disease (A-BD) is a chronic relapsing vasculitis of u nknown etiology. This disease is relatively rare in children and only recen tly have series of patients been reported. The objective of this study was to describe the clinical features of the disease in juvenile patients, and to compare them with adult cases and with those juveniles reported in the l iterature. Methods A special structured protocol was applied at presentation and during the fo llow-up of a series of Greek patients with A-BD. Among the 70 patients 18 w ere under the age of 16 years. Results All of our patients fulfilled the International Study Group criteria for ED . Eighteen patients nel eloped the disease before the age of 16 pears and w ere considered as juvenile cases. The first symptoms in our patients were o ral aphthae, arthritis and pleurisypericarditis. During the follow-up, geni tal ulcers, folliculitis, erythema nodosum, eye lesions, central nervous sy stem involvement, vasculitis, orchiepididymitis and intestinal involvement were noted. A pathergy test was positive in 22% of the patients. When our j uvenile cases were compared with the adult cases, differences were found bu t were statistically significant only for CNS involvement. Comparison of ou r results with those of other studies also showed differences, so,ne of whi ch were statistically significant. Conclusion The onset of A-BD occurred in 26% of our patients before the age of 16 year s. Statistically significant differences in various clinical features were found when our juvenile cases were compared to adults and juveniles in othe r series in the literature These differences reflect the different populati ons studied and, possibly genetic and environmental factors which contribut e to differing expressions of the disease.