Pediatric Behҫet disease: Update in diagnosis and management

Area of Science:

• Rheumatology and Immunology
• Pediatric Autoimmune and Inflammatory Diseases

Background:

• Behçet disease is a multisystem inflammatory disorder characterized by variable vessel vasculitis.
• It primarily affects oral and genital mucosa, skin, and eyes, posing diagnostic challenges due to lack of specific tests and overlap with other autoinflammatory conditions.

Purpose of the Study:

• To provide guidance on the diagnostic workup for pediatric Behçet disease.
• To outline a multidisciplinary approach for managing pediatric Behçet disease.
• To review evidence-based treatment strategies for refractory mucocutaneous manifestations in children.

Main Methods:

• Literature review of diagnostic criteria and management guidelines for Behçet disease.
• Analysis of treatment strategies for pediatric patients with mucocutaneous involvement.
• Synthesis of recommendations for a multidisciplinary care approach.

Main Results:

• Diagnosis of pediatric Behçet disease requires a high index of suspicion and exclusion of mimics.
• Multidisciplinary collaboration is crucial for comprehensive patient care.
• Evidence-based treatments focus on reducing inflammation and preventing flares, particularly for mucocutaneous symptoms.

Conclusions:

• Effective management of pediatric Behçet disease relies on timely diagnosis and a coordinated multidisciplinary approach.
• Targeted treatment strategies are essential for controlling inflammation and improving outcomes in children with refractory symptoms.