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

Abstract

Abstract Objectives To validate the PEDiatric Behçet’s Disease classification criteria (PEDBD) with an evidence-based approach. Methods A total of 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 and 0.99, and an accuracy of 0.91, 0.94 and 0.92, respectively. Conclusions The PEDBD criteria were very specific, while the ICBD were more sensitive. The complexity of childhood BD suggests larger prospective international cohorts to further evaluate the performance of the criteria.