Juvenile-onset Behçet's syndrome and mimics

Area of Science:

• Pediatric Rheumatology
• Immunology
• Genetics

Background:

• Behçet's syndrome (BS) affects up to 20% of patients in childhood, frequently presenting with recurrent oral ulcers.
• Childhood BS exhibits a heterogeneous presentation, with mucocutaneous disease common, while vascular and neurological involvement are rarer.
• Musculoskeletal and gastrointestinal issues are more prevalent in pediatric BS than adult forms, yet are often excluded from classification criteria.

Purpose of the Study:

• To highlight the distinct clinical manifestations of Behçet's syndrome in children compared to adults.
• To emphasize the need for updated diagnostic criteria that incorporate features more common in pediatric BS.
• To underscore the importance of investigating monogenic autoinflammatory conditions that mimic BS in children.

Main Methods:

• Clinical case review and analysis of reported pediatric Behçet's syndrome cases.
• Comparison of disease phenotypes between pediatric and adult-onset Behçet's syndrome.
• Review of current diagnostic criteria and treatment approaches for Behçet's syndrome.

Main Results:

• Recurrent oral ulceration is the most common presenting feature in childhood BS.
• Mucocutaneous disease, including genital ulcers and skin lesions, is a frequent phenotype in pediatric patients.
• Musculoskeletal and gastrointestinal involvement are observed more frequently in children but are not typically included in standard classification criteria.

Conclusions:

• Pediatric Behçet's syndrome presents with unique characteristics, necessitating tailored diagnostic and management strategies.
• The discovery of monogenic mimics highlights the need for genetic studies to differentiate and understand autoinflammatory conditions presenting as BS.
• Further research is required to refine classification criteria and treatment protocols for childhood Behçet's syndrome.