Clinical features of juvenile Adamantiades-Behçet's disease in Greece.

Objective Adamantiades-Bencet disease (A-BD) is a chronic relapsing vasculitis of unknown etiology. This disease is relatively rare in children and only recently 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 literature. Methods A special structured protocol was applied at presentation and during the follow-up of series of Greek patients with A-BD. Among the 70 patients 18 were under the age of 16 years. Results All of our patients fulfilled the International Study Group criteria for.BD. Eighteen patients developed the disease before the age of 16 years and were considered as juvenile cases. The first symptoms in our patients were oral aphthae, arthritis and pleurisypericarditis. During the follow-up, genital ulcers, folliculitis, erythema nodosum, eye lesions, central nervous system involvement, vasculitis, orchiepididymitis and intestinal involvement were noted. A pathergy test was positive in 22% of the patients. When our juvenile cases were compared with the adult cases, differences were found but were statistically significant only for CNS involvement. Comparison of our results with those of other studies also showed differences, some of which were statistically significant. Conclusion The onset ofA-BD occurred in 26% of our patients before the age of 16 years. Statistically significant differences in various clinical features were found when our juvenile cases were compared to adults and juveniles in other series in the literature. These differences reflect the different populations studied and, possibly, genetic and environmental factors which contribute to differing expressions of the disease.