Clinical course of inflammatory bowel disease and impact on liver disease outcomes in patients with autoimmune sclerosing cholangitis

Jeremy S. Nayagam*, Mandour O. Mandour, Alison Taylor, Michael A. Heneghan, Patrick CA Dubois, Bu Hayee, Huey Miin Lee, Babu Vadamalayan, Marianne Samyn, Deepak Joshi, Alexandra J. Kent

*Corresponding author for this work

Research output: Contribution to journalArticlepeer-review

1 Citation (Scopus)

Abstract

Background & Aims: Autoimmune sclerosing cholangitis (ASC) is a childhood sclerosing cholangitis frequently associated with inflammatory bowel disease (IBD). We describe the IBD phenotype in ASC patients and associated liver disease outcomes. Methods: Single center retrospective observational review of ASC patients, with a control population of pediatric IBD. Demographic and clinical parameters were obtained. Clinical endpoints were escalation of IBD therapy (biologic or colectomy) and transplant-free survival. Results: In 93 ASC patients (53.8% female) and median follow up of 172 months: 70% had IBD, 25.8% underwent liver transplant. Median age at liver transplant was 21.7 years, at 131 months from ASC diagnosis. There was no association between presence of IBD and transplant-free survival, whilst those requiring second-line immunomodulators for ASC had poorer long-term liver prognosis. During follow-up 22 (33.8%) ASC-IBD required biologic or colectomy. On multivariate analysis ASC was associated with a lower risk of escalation of IBD therapy (HR 0.14, 95% CI 0.05–0.42; P=.001), including biologic therapy (HR 0.21, 95% CI 0.08–0.55, P=.002), but not colectomy on univariate analysis (HR 1.54, 95% CI 0.43–5.44, P=.51). Conclusions: IBD is common in ASC and during longterm follow up a third of ASC-IBD required escalation of IBD therapy; however ASC-IBD was lower risk compared to IBD alone. IBD does not appear to impact on transplant-free survival in patients with ASC, however second-line immunomodulators for ASC are associated with poorer IBD and liver outcomes.

Original languageEnglish
Article number101980
JournalClinics and research in hepatology and gastroenterology
Volume46
Issue number7
DOIs
Publication statusPublished - 1 Aug 2022

Keywords

  • Biologic
  • Colitis
  • Crohn's disease
  • Primary sclerosing cholangitis

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