Isabelle Koné-Paut1, Fahrad Shahram2, Martha Darce-Bello1, Luca Cantarini3, Rolando Cimaz4, Marco Gattorno5, Jordi Anton6, Michael Hofer7, Bouchra Chkirate8, Kenza Bouayed9, Ilknur Tugal-Tutkun10, Jasmin Kuemmerle-Deschner11, Hélène Agostini12, Sylvia Federici5, Armelle Arnoux12, Celine Piedvache12, Seza Ozen13. 1. Department of Paediatric Rheumatology, CEREMAI, Bicêtre University Hospital, APHP, Paris SUD, Le Kremlin Bicêtre, France. 2. Rheumatology Research Center, Shariati Hospital, Tehran, Iran. 3. Department of Reumatologia, Sienna, Italy. 4. Department of Paediatric Rheumatology, A. Meyer Children, Florence, Italy. 5. UO Pediatria II, G. Gaslini Scientific Institute, Genoa, Italy. 6. Paediatric Rheumatology Unit, Sant Joan de Déu University Hospital, Esplugues de Llobregat, Spain. 7. Department of Paediatric Rheumatology, Vaudois University Hospital, Lausanne, Switzerland. 8. Internal Medicine and Immunology Clinic, Hassan II University Hospital, Fes, Morocco. 9. Department of Paediatrics, IBN Rochd University Hospital, Casablanca, Morocco. 10. Department of Ophthalmology, Istanbul Faculty of Medicine, Istanbul University, Istanbul, Turkey. 11. Division of Pediatric Rheumatology, Department of Pediatrics, University Hospital Tübingen, Tübingen, Germany. 12. Clinical Research Unit, Bicêtre University Hospital, APHP, Paris SUD, Le Kremlin Bicêtre, France. 13. Department of Paediatrics, Hacettepe University, Ankara, Turkey.
Abstract
BACKGROUND: We aimed to describe the main features of Behçet's disease (BD) in children in the largest prospective cohort to date and to propose a classification. METHODS: An international expert consensus group was formed to define a data set of minimal symptoms for the inclusion of patients. Patients were entered prospectively during 66 months. Experts classified patients on a consensus basis. The concordance of two international classifications was analysed in confirmed patients with BD. Comparisons of subgroups of patients helped define consensus criteria. BD-associated clinical manifestations were also investigated in three control diseases extracted from an independent data set (Eurofever). FINDINGS: In total, 42 centres from 12 countries included 230 patients; data for 219 (M/F ratio=1) could be analysed. The experts classified 156 patients (71.2%) as having confirmed BD. Males more often than females showed cutaneous, ocular and vascular symptoms and females more often genital aphthosis. Age at disease onset and skin and vascular involvement were lower for European than non-European children. Oral aphthosis was the presenting sign for 81% (179/219) of patients. The mean delay to the second symptom was 2.9±2.2 years. International classifications were not concordant with the expert classification. Our paediatric classification contains six categories, a minimum of three signs (each in a distinct category) defining paediatric BD. Three clinical signs discriminated our cohort from the Eurofever cohorts. INTERPRETATION: We present a comprehensive description of a large cohort of patients from both European and non-European countries and propose the first classification of paediatric BD for future therapeutic trials. Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://www.bmj.com/company/products-services/rights-and-licensing/
BACKGROUND: We aimed to describe the main features of Behçet's disease (BD) in children in the largest prospective cohort to date and to propose a classification. METHODS: An international expert consensus group was formed to define a data set of minimal symptoms for the inclusion of patients. Patients were entered prospectively during 66 months. Experts classified patients on a consensus basis. The concordance of two international classifications was analysed in confirmed patients with BD. Comparisons of subgroups of patients helped define consensus criteria. BD-associated clinical manifestations were also investigated in three control diseases extracted from an independent data set (Eurofever). FINDINGS: In total, 42 centres from 12 countries included 230 patients; data for 219 (M/F ratio=1) could be analysed. The experts classified 156 patients (71.2%) as having confirmed BD. Males more often than females showed cutaneous, ocular and vascular symptoms and females more often genital aphthosis. Age at disease onset and skin and vascular involvement were lower for European than non-European children. Oral aphthosis was the presenting sign for 81% (179/219) of patients. The mean delay to the second symptom was 2.9±2.2 years. International classifications were not concordant with the expert classification. Our paediatric classification contains six categories, a minimum of three signs (each in a distinct category) defining paediatric BD. Three clinical signs discriminated our cohort from the Eurofever cohorts. INTERPRETATION: We present a comprehensive description of a large cohort of patients from both European and non-European countries and propose the first classification of paediatric BD for future therapeutic trials. Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://www.bmj.com/company/products-services/rights-and-licensing/
Authors: Ekim Z Taskiran; Hafize E Sonmez; Can Kosukcu; Ece Tavukcuoglu; Gozde Yazici; Gunes Esendagli; Ezgi D Batu; Pelin O S Kiper; Yelda Bilginer; Mehmet Alikasifoglu; Seza Ozen Journal: J Clin Immunol Date: 2019-01-08 Impact factor: 8.317