Background: Eosinophilic enterocolitis (EEC) is an emerging distinct inflammatory bowel disease of unknown etiology. There are no published data on the effect of infliximab (IFX) or adalimumab (ADA) for the treatment of refractory cases.
Methods: A report of all pediatric cases with EEC treated with anti-tumor necrosis factor, identified after an open international call.
Results: We describe here the first 8 children with refractory EEC who were treated with IFX (75% boys; mean age at diagnosis 8.6 ± 4.03 [range 1.6-14 years]; mean age at IFX treatment 11.7 ± 4.4 [range 4.2-16 years]). Allergic and infectious causes of EEC were excluded in all cases. Rapid and complete clinical remission was documented in 6 (75%) children following the induction infusions: 3 (38%) with endoscopic remission, 2 (25%) with endoscopic improvement, and 1 unknown. Four of the 6 responders had secondary loss of response and were switched to ADA, 3 of whom with sustained remission using high doses. Overall, the 6 responders were followed for a median of 7 years (range 4-12; interquartile range 6.4-8.8 years) without evidence of developing Crohn disease or ulcerative colitis. The only case with macroscopic findings on endoscopy was a primary nonresponder.
Conclusions: IFX and ADA may be effective in cases of refractory idiopathic EEC; however, because this is an uncontrolled report, further prospective studies are warranted.