Phenotypic subgroups and classification criteria performance in paediatric Behçet's disease: insights on systemic...

Area of Science:

• Pediatric Rheumatology
• Vasculitis Research
• Immunology

Background:

• Behçet's disease (BD) is a multisystem vasculitis with varied pediatric presentations.
• Early diagnosis of pediatric BD is challenging due to diverse symptoms and limitations of current diagnostic criteria.
• Understanding age-related differences and diagnostic delays is crucial for timely intervention.

Purpose of the Study:

• To compare clinical characteristics of pediatric Behçet's disease based on age at onset.
• To investigate factors contributing to diagnostic delays in pediatric BD.
• To evaluate the performance of existing diagnostic criteria (ISG, ICBD, PEDBD) in pediatric BD.

Main Methods:

• Retrospective analysis of 38 pediatric BD patients.
• Categorization into early-onset (<6 years) and late-onset (≥6 years) groups.
• Evaluation of demographic data, clinical features, lab results, treatments, and diagnostic criteria fulfillment using statistical tests.

Main Results:

• Early-onset pediatric BD patients exhibited similar clinical profiles and diagnostic delays compared to late-onset patients.
• Mucocutaneous features were less common in ICBD-negative patients; PEDBD positivity indicated mucocutaneous-dominant phenotypes.
• Current criteria (ISG, ICBD) showed limited sensitivity for systemic presentations (CNS, vascular involvement), with few patients meeting criteria.

Conclusions:

• Pediatric Behçet's disease clinical presentation is consistent across age groups, though diagnostic delays are common.
• Existing diagnostic criteria are insufficient for identifying neurologic and vascular phenotypes in pediatric BD.
• Pediatric-specific revisions and validation of diagnostic criteria in larger cohorts are needed.