Behçet's disease in children: single-center experience

Area of Science:

• Pediatric Rheumatology
• Immunology
• Internal Medicine

Background:

• Behçet's disease is a rare multisystemic inflammatory disorder.
• Pediatric onset Behçet's disease presents unique diagnostic and management challenges.
• Understanding the disease's characteristics in children is crucial for effective care.

Purpose of the Study:

• To summarize the demographics, clinical, and laboratory findings in children diagnosed with Behçet's disease.
• To evaluate the efficacy, duration, and adverse effects of various treatment modalities.
• To highlight the importance of early diagnosis and appropriate treatment in pediatric Behçet's disease.

Main Methods:

• Retrospective review of 34 pediatric patients diagnosed with Behçet's disease between 1980 and 2013.
• Analysis of demographic data, clinical manifestations, and laboratory findings.
• Assessment of treatment approaches, efficacy, and adverse events.

Main Results:

• Recurrent oral aphthae were the most common initial symptom (97%).
• Frequent mucocutaneous findings included pseudofolliculitis, genital ulcers, and pathergy positivity.
• Common systemic involvements were joint, ocular, and vascular manifestations.
• Colchicine was the most frequently used drug (88%), with diarrhea as a common side effect.
• Steroids, azathioprine, and anti-TNF-alpha agents were used for specific organ involvements.

Conclusions:

• Pediatric Behçet's disease exhibits distinct clinical and demographic patterns.
• Early diagnosis and tailored treatment are essential to minimize morbidity and mortality.
• Monitoring treatment efficacy and adverse effects is critical for managing pediatric Behçet's disease.