Behçet's disease with multiple splenic abscesses in a child

Area of Science:

• Pediatric Rheumatology
• Gastroenterology
• Immunology

Background:

• Behçet's disease is a rare multisystemic inflammatory disorder.
• Splenic abscesses are an uncommon but serious complication, particularly in pediatric cases.
• This case highlights the diagnostic challenges and treatment strategies for aseptic splenic abscesses in a child with Behçet's disease.

Purpose of the Study:

• To report a rare case of multiple aseptic splenic abscesses in a pediatric patient with Behçet's disease.
• To discuss the diagnostic workup, including imaging and laboratory tests.
• To evaluate the efficacy of different immunosuppressive therapies in managing this complex condition.

Main Methods:

• Case report of a 5-year-old male with fever, abdominal pain, diarrhea, and recurrent stomatitis.
• Diagnostic investigations included colonoscopy, abdominal CT scan, and ultrasound-guided fine needle aspiration.
• Pathergy and HLA-B51 tests were performed.
• Treatment involved initial antibiotics, followed by steroids, infliximab, and adalimumab.

Main Results:

• The patient presented with symptoms consistent with Behçet's disease and was diagnosed with multiple aseptic splenic abscesses.
• Steroid treatment initially resolved symptoms and abscesses, but recurrence occurred upon tapering.
• Infliximab provided temporary improvement, while adalimumab led to sustained symptom resolution and improved colonoscopic findings.
• No recurrence was observed during a 3-month follow-up after adalimumab initiation.

Conclusions:

• Aseptic splenic abscesses can be a manifestation of Behçet's disease in children.
• A multimodal treatment approach combining immunosuppressive agents is crucial for managing such cases.
• Biologic therapies like adalimumab show promise for long-term disease control in pediatric Behçet's disease with splenic involvement.