R Treudler1, C E Orfanos, C C Zouboulis. 1. Department of Dermatology, University Medical Center Benjamin Franklin, The Free University of Berlin, Berlin, Germany.
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.
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.
Authors: A Altenburg; A Mahr; C Maldini; C E Kneifel; L Krause; I Kötter; T Stache; N G Bonitsis; C C Zouboulis Journal: Ophthalmologe Date: 2012-06 Impact factor: 1.059
Authors: Elizabeth E Adams; Vincent P R Aluquin; C April Bingham; James R Stone; Linda B Pauliks Journal: Pediatr Cardiol Date: 2009-10-27 Impact factor: 1.655