Uveitis in paediatric Behçet disease: a large multicentric Italian cohort

Area of Science:

• Ophthalmology
• Rheumatology
• Pediatrics

Background:

• Behçet disease (BD) is a rare childhood inflammatory disorder.
• Uveitis in pediatric BD is a significant cause of vision loss if untreated.

Purpose of the Study:

• To describe a cohort of pediatric BD patients experiencing uveitis.
• To evaluate treatment outcomes and disease characteristics in this population.

Main Methods:

• A multicentric retrospective study involving six Italian pediatric rheumatology units.
• Data collected on demographics, clinical features, and ocular characteristics, assessed using Standardization Uveitis Nomenclature.
• Follow-up data analyzed through March 2023.

Main Results:

• 33% of 97 pediatric BD patients had uveitis, with a median onset age of 11.5 years.
• Uveitis preceded BD diagnosis in over half of cases (54.5%); 51.5% were HLA B51 positive.
• Panuveitis was common (48.5%), and biologic agents like adalimumab were frequently used, achieving remission in 85.7% of treatments.

Conclusions:

• Pediatric BD uveitis is a sight-threatening condition, often presenting as panuveitis.
• Biologic therapies are crucial for managing ocular inflammation in pediatric BD.
• Long-term monitoring is essential due to potential for treatment relapses.