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

TY - JOUR

T1 - Clinical course of inflammatory bowel disease and impact on liver disease outcomes in patients with autoimmune sclerosing cholangitis

AU - Nayagam, Jeremy S.

AU - Mandour, Mandour O.

AU - Taylor, Alison

AU - Heneghan, Michael A.

AU - Dubois, Patrick CA

AU - Hayee, Bu

AU - Lee, Huey Miin

AU - Vadamalayan, Babu

AU - Samyn, Marianne

AU - Joshi, Deepak

AU - Kent, Alexandra J.

N1 - Funding Information: No financial support was provided for this project. Publisher Copyright: © 2022 Elsevier Masson SAS

PY - 2022/8/1

Y1 - 2022/8/1

N2 - 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.

AB - 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.

KW - Biologic

KW - Colitis

KW - Crohn's disease

KW - Primary sclerosing cholangitis

UR - http://www.scopus.com/inward/record.url?scp=85135565605&partnerID=8YFLogxK

U2 - 10.1016/j.clinre.2022.101980

DO - 10.1016/j.clinre.2022.101980

M3 - Article

C2 - 35728760

AN - SCOPUS:85135565605

SN - 2210-7401

VL - 46

JO - Clinics and research in hepatology and gastroenterology

JF - Clinics and research in hepatology and gastroenterology

IS - 7

M1 - 101980

ER -