Abstract
Introduction: IBD is an immune-mediated disease with genetic/environmental risk factors. The lung and GIT share a similar epithelial barrier function. IBD after other types of solid organ Tx has been descibed. We describe de novo IBD in two paediatric LTX recipients.
Method: -15.5yo male had LTX for Surfactant protein C deficiency and severe ILD 4y ago. Developed perianal pain and rectal bleeding. Pan-enteropathy with no evidence of post TX proliferative disease (PTLD) on endoscopy, despite high blood EBV level. Collection in the anal canal with two fistulae on MRI, antibiotics/pre-emptive Rituximab given, collection drainage at EUA, and Seton insertion. Infliximab had excellent response. Developed PJP pneumonitis, with associated worsening of lung function, acute cellular rejection. GIT symptoms well controlled.
-18yo female with CF underwent LTX 4y ago. 3y post LTX, presented with abdominal pain and weight loss. Right-sided chronic active colitis with EBV expression on colonoscopy. Bowel biopsy showed chronic inflammation with distortion of terminal ileum; inconclusive for PTLD. MRI/USS/VCE: normal. No improvement with Rituximab. IBD treatment started with exclusive enteral nutrition, stopped after 2 weeks, once oral Budesonide introduced and symptoms improved.
Conclusion: Histologic and radiological findings suggest IBD in the patients already on immunosuppressive medication following LTX. Both had evidence of EBV viraemia. PTLD treatment was not effective. IBD treatment improved GIT symptoms. Little experience in biologic treatment in paediatric LTX patients. Research could help identify unique disease characteristics and epithelial barrier function abnormalities.
Footnotes
Cite this article as: European Respiratory Journal 2020; 56: Suppl. 64, 2736.
This abstract was presented at the 2020 ERS International Congress, in session “Respiratory viruses in the "pre COVID-19" era”.
This is an ERS International Congress abstract. No full-text version is available. Further material to accompany this abstract may be available at www.ers-education.org (ERS member access only).
- Copyright ©the authors 2020