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

Dimitri Poddighe, Donato Rigante, Micol Romano, Zaure Mukusheva, Nicola Ughi, Erdal Sağ, Selcan Demir, Natalie Mariam Zitoun, David Piskin, Erkan Demirkaya

Diagnostics, 2026

Full text Open access: Gold Metadata (CSL-JSON)
DOI 10.3390/diagnostics16193184
Data confidence 85 high
How this score is calculated

It is built from checkable properties of the record itself: whether the article has a DOI, how many independent sources confirmed its data, whether the required fields are filled in, and how recent the latest confirmation is. Disagreement between sources and a missing identifier lower it. A value of 80 or above means the record was published without further checking; 60 to 79 means it is published at reduced confidence, with a manual check advisable. A record verified by an editor by hand is never pushed below 90 by a recalculation. This scores our data about the article, not the article itself.

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.