Twenty-eight cases of juvenile-onset Adamantiades-Behçet disease in Germany.

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) 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.