Validation of the PEDiatric Behçet's Disease classification criteria: an evidence-based approach.

Autor:	Matucci-Cerinic C; UOC Rheumatology and Autoinflammatory diseases, IRCCS Istituto G. Gaslini, Genoa, Italy.; Department of Neurosciences, Rehabilitation, Ophthalmology, Genetics, Maternal and Child Health (DINOGMI), University of Genoa, Genoa, Italy., Palluy H; Pediatric rheumatology and CEREMAIA, Bicêtre hospital, APHP, University Paris Saclay, Paris, France., Al-Mayouf SM; Dept of Pediatrics, King Faisal Specialist Hospital and Research Center, College of Medicine, Alfaisal University, Riyadh, Saudi Arabia., Brogan PA; University College London, Great Ormond Street Institute of Child Health, London, UK., Cantarini L; Rheumatology Unit, Dept of Medical Sciences, Surgery and Neurosciences, University of Siena, Italy., Gul A; Division of Rheumatology, Department of Internal Medicine; Istanbul Faculty of Medicine, Istanbul University, Istanbul, Turkey., Kasapcopur O; Department of Pediatric Rheumatology, Cerrahpasa Medical School, Istanbul University-Cerrahpasa, Turkey., Kuemmerle-Deschner J; Division of Pediatric Rheumatology, Department of Pediatrics and autoinflammation reference center Tuebingen, University Hospital Tuebingen, Tuebingen, Germany., Ozen S; Department of Pediatric Rheumatology, Hacettepe University, Ankara, Turkey., Saadoun D; Department of Internal Medicine and Clinical Immunology and CEREMAIA, AP-HP Groupe Hospitalier Pitié-Salpêtrière, Sorbonne University, Paris, France., Shahram F; Rheumatology Research Center, Shariati Hospital, Tehran University of Medical Sciences, Tehran, Iran., Bovis F; Department of Health Sciences (DISSAL), University of Genoa, Genoa, Italy., Mosci E; Gaslini Trial Center, IRCCS Istituto Giannina Gaslini, Genova, Italy., Ruperto N; Gaslini Trial Center, IRCCS Istituto Giannina Gaslini, Genova, Italy., Gattorno M; UOC Rheumatology and Autoinflammatory diseases, IRCCS Istituto G. Gaslini, Genoa, Italy., Kone-Paut I; Pediatric rheumatology and CEREMAIA, Bicêtre hospital, APHP, University Paris Saclay, Paris, France.
Jazyk:	angličtina
Zdroj:	Rheumatology (Oxford, England) [Rheumatology (Oxford)] 2023 Nov 22. Date of Electronic Publication: 2023 Nov 22.
DOI:	10.1093/rheumatology/kead609
Abstrakt:	Objectives: to validate the PEDiatric Behçet's Disease classification criteria (PEDBD) with an evidence-based approach. Methods: 210 pediatric patients (70 Behçet's disease (BD), 40 Periodic Fever, Aphthous stomatitis, Pharyngitis, Adenitis, 35 familial Mediterranean fever, 26 hyper-IgD syndrome, 22 TNF-Receptor associated Periodic fever Syndrome, 17 undefined recurrent fevers) were randomly selected from the Eurofever Registry. A set of 11 experienced clinicians/researchers blinded to the original diagnosis evaluated the patients. Using the table consensus as gold standard (agreement ≥ 80%), the PEDBD, ISG and ICBD criteria were applied to BD patients and to confounding diseases with other autoinflammatory conditions in order to define their sensitivity, specificity and accuracy. Results: At the end of the third round, a consensus was reached in 139/210 patients (66.2%). The patients with a consensus ≥80% were classified as confirmed-BD (n = 24), and those with an agreement of 60-79% as probable-BD (n = 10). When comparing these patients with the confounding diseases group, an older age at disease onset, the presence of oral and genital ulcers, skin papulo-pustular lesions, a positive pathergy test and posterior uveitis were BD distinctive elements. The ISG, ICBD and PEDBD criteria were applied to confirmed-BD and to the confounding disease group, showing a sensitivity of 0.50, 0.79 and 0.58, a specificity of 1.00, 0.97, 0.99, and an accuracy of 0.91, 0.94 and 0.92, respectively. Conclusions: the PEDBD criteria were very specific, while the ICBD resulted to be more sensitive. The complexity of childhood BD suggests larger prospective international cohorts to further evaluate the performance of the criteria. (© The Author(s) 2023. Published by Oxford University Press on behalf of the British Society for Rheumatology.)
Databáze:	MEDLINE
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