Yonatan Butbul Aviel1, Ezgi Deniz Batu2, Betül Sözeri3, Nuray Aktay Ayaz4, Lemor Baba5, Gil Amarilyo6, Seval Şimşek3, Liora Harel6, Serife Gul Karadag4, Yelda Bilginer2, Seza Özen7. 1. Department of Pediatrics B, Ruth Rappaport Children's Hospital, Rambam Health Care Campus, Haifa, Israel; Pediatric Rheumatology Service, Ruth Rappaport Children's Hospital, Rambam Health Care Campus, Haifa, Israel; The Ruth and Bruce Rappaport Faculty of Medicine, Technion - Israel Institute of Technology, Haifa, Israel. 2. Department of Pediatrics, Division of Rheumatology, Hacettepe University Faculty of Medicine, Ankara 06100, Turkey. 3. Department of Pediatrics, Division of Rheumatology, Umraniye Training and Research Center, University of Health Sciences, Istanbul, Turkey. 4. Department of Pediatrics, Division of Rheumatology, Kanuni Sultan Suleyman Research and Training Hospital, University of Health Sciences, Istanbul, Turkey. 5. Sackler Faculty of Medicine, Tel Aviv University, Ramat Aviv, Israel. 6. Sackler Faculty of Medicine, Tel Aviv University, Ramat Aviv, Israel; Pediatric Rheumatology Unit, Schneider Children's Medical Center of Israel, Petach Tikva, Israel. 7. Department of Pediatrics, Division of Rheumatology, Hacettepe University Faculty of Medicine, Ankara 06100, Turkey. Electronic address: sezaozen@hacettepe.edu.tr.
Abstract
BACKGROUND: Behçet's disease (BD) is a variable vessel vasculitis which is rare in children. OBJECTIVE: We aimed to compare the main characteristics of pediatric BD patients from Turkey versus Israel. METHODS: Three centers from Turkey and two centers from Israel participated in this study. The BD diagnosis was before 16 years of age and based on expert opinion. Disease activity was assessed with BD current activity form (BDCAF). RESULTS: A total of 205 patients were included (165 from Turkey; 40 from Israel). HLA-B51 positivity (68.3% vs. 46.2%, p = 0.028), male gender (52% vs. 30%, p = 0.012), and skin involvement (55.2% vs. 22.5%, p<0.001) were more frequent among patients from Turkey compared to patients from Israel. Tests of pathergy and HLA-B51 were more frequently performed in patients from Turkey than patients from Israel (93.3% vs. 32.5%, p<0.001 and 97.6% vs. 65%, p<0.001; respectively). For BD classification in the whole group, International Criteria for BD (ICBD) had the highest sensitivity (73.2%), followed by pediatric BD (PED-BD) (47.8%), and The International Study Group (ISG) (42%) criteria sets. The most commonly prescribed drug was colchicine in the whole group (96.6%). Significantly more patients were treated with corticosteroids (50% vs. 28.5%, p = 0.006), methotrexate (17.5% vs. 3%, p = 0.002), and nonsteroidal anti-inflammatory drugs (12.5% vs. 1.8%, p = 0.007) in Israel than in Turkey. The median BDCAF values were higher at the first visit for patients from Turkey compared to those in Israel (4 vs. 2, p<0.001). CONCLUSION: This is the largest cohort of pediatric BD reported to date. The disease characteristics significantly differ among pediatric BD patients from Turkey and Israel, which may be due to different ethnicity and environmental factors.
BACKGROUND: Behçet's disease (BD) is a variable vessel vasculitis which is rare in children. OBJECTIVE: We aimed to compare the main characteristics of pediatric BDpatients from Turkey versus Israel. METHODS: Three centers from Turkey and two centers from Israel participated in this study. The BD diagnosis was before 16 years of age and based on expert opinion. Disease activity was assessed with BD current activity form (BDCAF). RESULTS: A total of 205 patients were included (165 from Turkey; 40 from Israel). HLA-B51 positivity (68.3% vs. 46.2%, p = 0.028), male gender (52% vs. 30%, p = 0.012), and skin involvement (55.2% vs. 22.5%, p<0.001) were more frequent among patients from Turkey compared to patients from Israel. Tests of pathergy and HLA-B51 were more frequently performed in patients from Turkey than patients from Israel (93.3% vs. 32.5%, p<0.001 and 97.6% vs. 65%, p<0.001; respectively). For BD classification in the whole group, International Criteria for BD (ICBD) had the highest sensitivity (73.2%), followed by pediatric BD (PED-BD) (47.8%), and The International Study Group (ISG) (42%) criteria sets. The most commonly prescribed drug was colchicine in the whole group (96.6%). Significantly more patients were treated with corticosteroids (50% vs. 28.5%, p = 0.006), methotrexate (17.5% vs. 3%, p = 0.002), and nonsteroidal anti-inflammatory drugs (12.5% vs. 1.8%, p = 0.007) in Israel than in Turkey. The median BDCAF values were higher at the first visit for patients from Turkey compared to those in Israel (4 vs. 2, p<0.001). CONCLUSION: This is the largest cohort of pediatric BD reported to date. The disease characteristics significantly differ among pediatric BDpatients from Turkey and Israel, which may be due to different ethnicity and environmental factors.