Twenty-Eight Cases of Juvenile-Onset Adamantiades-Behçet Disease in Germany

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Abstract
Background and Objective: Adamantiades-Behçet disease is a rare entity at a juvenile age. We aimed to enlighten epidemiological and clinical characteristics of juvenile-onset disease in Germany. Methods: Data from the German Registry were used to compare clinical and epidemiological features of patients with juvenile-onset (≤16 years) and adult-onset (>16 years) disease diagnosed according to the criteria of the International Study Group. Results: Twenty-eight (17%) of 168 patients of the German Registry exhibited the onset of the disease and 8 (5%) of them the complete symptom complex at a juvenile age. Juvenile-onset disease was characterized by an increase in familial cases (25 vs. 8% in patients with adult-onset; p = 0.047). The frequency of diagnostic signs was similar between the two study groups. In juvenile-onset disease, delayed development of the complete symptom complex (median value 35 months vs. 12 months after onset; p = 0.014) and lower prevalence of severe complications (9 vs. 29%; p = 0.042) were detected. Conclusions: The major clinical features of juvenile-onset and adult-onset disease in Germany are comparable, but in juvenile-onset disease, the course is delayed and patients experience less severe complications. In addition, there is a higher rate of familial occurrence of the disease in patients with juvenile-onset.