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Validation of the PEDiatric Behçet's Disease classification criteria: an evidence-based approach
Caterina Matucci-Cerinic1,2, Helene Palluy3, Sulaiman M Al-Mayouf4
1UOC Rheumatology and Autoinflammatory Diseases, IRCCS Istituto G. Gaslini, Genoa, Italy.
Insights
This study validated classification criteria for pediatric Behçet's disease (BD). The International Criteria for Behçet's Disease (ICBD) showed higher sensitivity, while the PEDiatric Behçet's Disease (PEDBD) criteria were more specific for childhood BD.
Area of Science:
- Rheumatology
- Pediatric Autoimmune Diseases
- Clinical Criteria Validation
Background:
- Behçet's disease (BD) is a complex systemic vasculitis with variable presentations.
- Accurate classification criteria are crucial for diagnosis and management of pediatric BD.
- Existing criteria may require refinement for the pediatric population.
Purpose of the Study:
- To validate the PEDiatric Behçet's Disease (PEDBD) classification criteria using an evidence-based approach.
- To compare the performance of PEDBD criteria against established criteria (ISG, ICBD) in a pediatric cohort.
- To identify key clinical features distinguishing pediatric BD from other autoinflammatory conditions.
Main Methods:
- A cohort of 210 pediatric patients, including 70 with BD and 140 with other autoinflammatory conditions, was analyzed.
- Experienced clinicians, blinded to original diagnoses, evaluated patients using consensus as a gold standard.
- The PEDBD, ISG, and ICBD criteria were applied to assess sensitivity, specificity, and accuracy.
Main Results:
- The International Criteria for Behçet's Disease (ICBD) demonstrated higher sensitivity (0.79) compared to PEDBD (0.58) and ISG (0.50).
- The PEDBD criteria exhibited superior specificity (0.99) and accuracy (0.92) compared to ISG (0.91) and ICBD (0.94).
- Distinctive features for pediatric BD included older age at onset, oral/genital ulcers, skin lesions, positive pathergy test, and posterior uveitis.
Conclusions:
- The PEDBD criteria are highly specific for pediatric Behçet's disease.
- The ICBD criteria offer greater sensitivity in classifying childhood BD.
- Larger prospective international studies are recommended to further evaluate and refine these classification criteria.
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.
