Abstract:

Objective Pediatric intestinal Behcet’s syndrome (PIBS) is a rare but severe systemic vascular inflammatory disease characterized by diverse clinical manifestations and difficult early diagnosis. To investigate the clinical features, endoscopic and pathological outcomes, treatment and prognosis of PIBS. Methods Case series study was conducted. The clinical data of 13 children with PIBS diagnosed in the department of gastroenterology from December 2017 to December 2024 were retrospectively analyzed, and followed up until June 2025. Primary outcome measures clinical response rate, mucosal healing rate under endoscopic observation. Results There were 13 children with PIBS, 5 males and 8 females, with a median age of onset of 89.0 (50.0-96.0) months and a median diagnosis time of 10.0 (2.0-24.5) months; The clinical manifestations were abdominal pain (84.6%), diarrhea (30.8%), recurrent oral ulcers (92.3%), and fever (84.6%), with 1 case with intestinal obstruction, intestinal perforation and abdominal abscess, and 1 case with spleen abscess; Endoscopy showed multiple deep and large circular ulcers at the end of the ileum or ileocecal region. Pathological results showed only one case of vasculitis; the rest were chronic active nonspecific inflammation. In terms of treatment, 10 patients received only glucocorticoid combined with immunosuppressants at the beginning, of which 2 patients were treated with biologics in the later stage, and the other 3 patients received biologics initially. Conclusions PIBS lacks distinctive clinical features and has a high rate of complications; however, it exhibits certain characteristics on endoscopy and histopathology. Pediatricians should increase their awareness of this condition, as early diagnosis and standardized treatment can improve prognosis.

Key words: intestinal Behcet’s syndrome, endoscopy, pathology, child