Background/Purpose: Behçet’s disease (BD) is an autoinflammatory disease characterized by a variable vessel vasculitis. In the past, several criteria have been created for adult BD classification. In 2015, the first set of BD paediatric classification criteria, the PEDBD, was proposed by an international Expert consensus. The aim of the study was to perform an external validation of the PEDBD classification criteria in a cohort of internationally validated pediatric BD, through an international Expert-based consensus process.

Methods: 210 patients (70BD, 40 PFAPA (Periodic Fever-Apthous stomatitis-Pharingytis-Adenopathies), 35 FMF, 26 HyperIgD syndrome, 22 TNF-receptor associated periodic fever syndrome, 17 undefined recurrent fevers) were randomly selected from the Eurofever Registry (patients excluded if disease onset > 16 years and if included in the first PEDBD study). A set of 11 Experts blinded to the original diagnosis, were chosen to evaluate the patients: in the 1^st round, clinical and serological data were evaluated; in the 2^nd round genetic data were added; in the 3^rd round the other Experts’ votes and comments were shown. Using the expert consensus as gold standard (agreement >80%), the PEDBD, the ISG and the ICBD criteria were applied to BD patients and to confounding diseases with a consensus, in order to define their sensitivity, specificity and accuracy.

Results: At the end of the 3^rd round, a consensus on the initial diagnosis was reached in 66.2% of patients. The BD patients with an agreement (24) were classified as confirmed-BD, and those with an agreement of 60-70% (10) as probable-BD.In confirmed-BD patients, oral ulcers were present in all the patients, genital ulcers in 77%, skin manifestations in 50%, a positive pathergy reaction in 39%, ocular manifestations in 54%, with a reesulting impaired vision in 17%, venous thrombosis in 8%, neurological manifestations in 21%, gastrointestinal manifestations in 38%, articular manifestations in 33% and fever in 50% of patients. The patients with ocular and vascular involvement were all males. HLA-B51 was positive in 69% of patients. When comparing these patients with the confounding diseases, 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 controls, resulting in the following test characteristics (Table).

Conclusion: the PEDBD were very specific in classifying BD patients, while the ICBD had a better sensitivity, especially for patients with only bipolar aphtosis. One limitation is that specific monogenetic BS mimics were not included as disease controls, thus the true accuracy of all these criteria may be lower in practice. The complexity of childhood BD suggests larger prospective international cohorts to foster the performance of the criteria, and to understand if BD clusters and ethnic variables should be added to the criteria.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/validation-of-the-pediatric-behcets-disease-classification-criteria-pedbd-an-international-consensus-based-approach/