JPGN Journal Club September 2024: Post-Transplant Pediatric IBD and Long-Term Outcomes in Autoimmune Sclerosing Cholangitis

JPGN Journal Club, led by Dr Jake Mann, is here again for August. As always, keep in mind ESPGHAN’s other educational offerings : https://www.espghan.org/knowledge-center – in particular on IX.12, AHP Summer School ; IX.21, the Young Investigator Forum ; IX.25, the 5th Liver Transplant School ; and X.25, a Monothematic Conference on Paediatric Gastric Disease. For today’s discussion Jake has chosen a case-review series of inflammatory bowel disease manifest after non-haematologic transplantation – from J Pediatr Gastroenterol Nutr, by Wenzel et al. – and from JHEP Reports, by Skarby et al., a long-term follow- up of children with autoimmune sclerosing cholangitis (AISC ; primary sclerosing cholangitis). The JPGN article describes eight children who several years after transplantation with various organs developed principally large-bowel inflammation with a lesser small-bowel component – neither ulcerative colitis nor Crohn disease, then – and speculates that tacrolimus in toddlers and small children may skew T-cell regulatory behaviour. On balance, the article is a “We saw this”, an exercise in bird- watching, and valuable as a guide to what are certainly rare birds. Skarby et al. offer a similar stroll through the aviary, collecting 124 children and adolescents with AISC or with both autoimmune hepatitis and AISC – a very respectable number of instances of a disorder occurring in two per million – and following them for up to 21 years (median 13 [5.7–21.6] years). Long-term morbidity was low, which surprised the authors (91% transplant-free survival at 10 years). Even after discounting for shifts in care regimens over the study period, the data are a vade mecum for families affected by AISC and for their caregivers. How AISC differs from its adult counterpart remains to be investigated, and work like this sets parameters for the enquiries that must be undertaken.Literature Wenzel AA et al. Posttransplant inflammatory bowel disease after successful solid organ transplantation : Not out of the woods yet. J Pediatr Gastroenterol Nutr 2024 Aug 9. Doi : 10.1002/jpn3.12347. Online ahead of print. PMID : 39118496 Skarby AJ et al. Good long-term outcomes of primary sclerosing cholangitis in childhood. JHEP Rep 2024 May 25; 6(8):101123. Doi : 10.1016/j.jhepr.2024.101123. eCollection 2024 Aug. PMID : 39139456