Twenty-eight cases of juvenile-onset Adamantiades-Behcet disease in Germany

Background and Objective: Adamantiades-Behcet disease is a rare entity at a juvenile age. We aimed to enlighten epidemiological and clinical character istics of juvenile-onset disease in Germany, Methods: Data from the German Registry were used to compare clinical and epidemiological features of pati ents with juvenile-onset (less than or equal to 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 Reg istry exhibited the onset of the disease and 8 (5%) of them the complete sy mptom complex at a juvenile age, Juvenile-onset disease was characterized b y 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 stu dy groups. In juvenile-onset disease, delayed development of the complete s ymptom 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 ad ult-onset disease in Germany are comparable, but in juvenile-onset disease, the course is delayed and patients experience less severe complications. I n addition, there is a higher rate of familial occurrence of the disease in patients with juvenile-onset.