ab17_2

Clinical features of juvenile Adamantiades-Behçet’s disease in Greece

G. Vaiopoulos¹, V.G. Kaklamani³, N. Markomichelakis², A. Tzonou⁴, M. Mavrikakis⁵, Ph. Kaklamanis²

¹First Department of Internal Medicine, Laiko General Hospital, Athens, Greece; ²Medical Center, Athens, Greece; ³Newton Wellesley Hospital, Boston, MA, USA; ⁴Department of Hygiene and Epidemiology, Medical School and ⁵Therapeutic Clinic, Alexandra Hospital, Athens, Greece.

ABSTRACT
Objective
Adamantiades-Behçet’s 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 a 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 pleurisy-pericarditis. 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 of A-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.

Key words
Adamantiades-Behçet’s disease, juvenile.

V.G. Kaklamani is the recipient of a scholarship from Athens University (Marouda Scholarship).

Please address correspondence and reprint requests to: Phaedon Kaklamanis, M.D., 61 Ipsilantou Street, Athens 115 21, Greece.