Gastrointestinal Involvement in Pediatric Behçet's Disease: A Focused Analysis from a Comprehensive Systematic Review.

Abstract

Background: Gastrointestinal (GI) involvement is a clinically relevant but still incompletely characterized manifestation of pediatric Behcet's disease (PedBD). This systematic review provides a focused analysis of GI involvement in PedBD (addressing its frequency, clinical symptomatology, anatomical/endoscopic features, and therapeutic management) as a dedicated sub-analysis of a broader systematic review. Methods: A systematic search of MEDLINE, EMBASE, CINAHL, CENTRAL, and ClinicalTrials.gov was conducted, following PRISMA 2020 guidelines. Observational studies reporting GI involvement in patients with PedBD (disease onset before 16 years of age) were included, and data on frequency, symptoms, anatomical/endoscopic findings, and treatment of GI-PedBD were extracted and synthesized. Results: Thirty studies were included. Most studies were not focused on GI manifestations in PedBD as a (primary) study aim. The frequency of GI involvement ranged widely across the selected articles with a median of approximately 15% among studies with ≥50 pediatric BD patients. Abdominal pain and diarrhea were the most consistently reported symptoms. Endoscopic data indicated frequent involvement of the ileocecal region and terminal ileum, with mucosal ulcers and erosions as the predominant findings. Therapeutic data specific to GI-PedBD were limited to two studies only. As an appendix to the systematic review in the discussion, relevant case reports were described: anti-TNF agents, particularly adalimumab, showed favorable outcomes in refractory cases. Conclusions: GI involvement in PedBD is not negligible and can be severe. Its clinical and endoscopic characterization remains incomplete due to substantial heterogeneity in reporting practices across studies. Anti-TNF agents may represent an effective therapeutic option, especially for refractory GI-PedBD. Prospective and multicenter studies with standardized GI assessment protocols are needed to better characterize this clinically relevant subgroup of PedBD patients.