Behçet's Disease in Children: Diagnostic and Management Challenges

Area of Science:

• Pediatric Rheumatology
• Immunology
• Internal Medicine

Background:

• Behçet's Disease (BD) is a multisystem inflammatory disorder of unknown cause.
• Key manifestations include recurrent oral/genital ulcers and uveitis, with potential for severe eye, vascular, and neurological complications.
• Pediatric-onset BD often presents with incomplete symptoms, posing diagnostic challenges.

Purpose of the Study:

• To address the diagnostic and therapeutic challenges of pediatric-onset Behçet's Disease.
• To highlight recent advancements in treatment strategies for pediatric BD.
• To discuss the role of emerging biologic agents in managing refractory cases.

Main Methods:

• Review of diagnostic difficulties in pediatric BD, including the recent introduction of classification criteria.
• Analysis of updated treatment recommendations for pediatric BD.
• Discussion of the efficacy of anti-TNFα agents and other biologics in clinical trials.

Main Results:

• Anti-TNFα agents are effective as second-line therapy for refractory pediatric BD and as first-line treatment for severe ocular/neurological involvement.
• Newer biologic agents targeting IL-17, IL-23, IL-1, and IL-6 show promising results in clinical trials for refractory cases.
• Improved therapeutic strategies have led to better outcomes for pediatric BD patients.

Conclusions:

• Recent updates in classification criteria and treatment recommendations have significantly improved the management of pediatric Behçet's Disease.
• Biologic therapies, particularly anti-TNFα agents and agents targeting specific cytokines, represent important advancements for refractory and severe cases.
• Ongoing research into molecular pathogenesis continues to drive the development of novel therapeutic approaches.