130 First description of de novo inflammatory bowel disease (IBD) in two adolescent patients after lung transplant (LTX)

Autor: Fevronia Kiparissi, Doxa Kotzia, Helen Spencer
Rok vydání: 2019
Předmět:
Zdroj: Abstracts.
Popis: Introduction Inflammatory bowel disease (IBD) is an immune-mediated disease with genetic and environmental risk factors. The lung and gastrointestinal tract (GIT) can share a similar epithelial barrier function. Development of IBD following other types of solid organ transplant has been previously described. We describe de novo IBD in two paediatric lung transplant (LTX) recipients. Cases Patient A: 14.5yo male underwent LTX for Surfactant protein C deficiency and severe interstitial lung disease (ILD) at age 11.5yo. He developed perianal pain and rectal bleeding. Endoscopy showed pan-enteropathy with no evidence of post-transplant proliferative disease (PTLD), despite high blood EBV levels. MRI showed a horseshoe collection in the lower anal canal with two intersphincteric fistulae. He was treated with antibiotics, pre-emptive Rituximab, collection drainage at EUA, and Seton insertion. Infliximab was started with excellent clinical response. Developed PJP pneumonitis with worsening of lung function and associated acute cellular rejection. GIT symptoms remain well controlled. Patient B: 17yo female with CF diagnosed following meconium ileus requiring laparotomy and resection of terminal ileum; underwent LTX at 14.5yo. Three years post LTX, presented with abdominal pain and weight loss. Colonoscopy showed focal right-sided chronic active colitis with patchy EBV expression. Small bowel biopsy showed chronic inflammation with architectural distortion of terminal ileum, but was inconclusive for PTLD. MRI/USS/VCE: normal. No improvement with Rituximab. IBD treatment started with exclusive enteral nutrition, which was discontinued after 2 weeks, once oral Budesonide introduced and symptoms improved. Conclusion Histologic and Radiological findings suggest IBD in both patients already on immunosuppressive medication following LTX. Both patients had evidence of EBV viraemia. However, treatment for possible PTLD did not resolve symptoms. IBD treatment improved GIT symptoms. There is little experience in biologic treatment in paediatric LTX patients. Future research could help identify unique disease characteristics and epithelial barrier function abnormalities.
Databáze: OpenAIRE