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

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

doi:10.1016/j.clinre.2022.101980

Cited by 2 publications

(1 citation statement)

References 25 publications

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“…The biliary cohort (PSC, overlap, or ASC) in our study were more likely to be (i) male; (ii) asymptomatic; and (iii) have IBD at disease onset, which is consistent with the disease phenotype described in the literature. 2 , 25 Importantly, none of the AIH-2 patients had biliary progression nor did they develop IBD in the duration of our study. There were well-defined expected differences in the AIH-2 cohort compared with the other AILD subgroups: younger age at presentation, aggressive clinical phenotype, higher rates of acute liver failure, and LT. 15 , 26 Disease recurrence and the need for re-grafting also surpassed other AILD phenotypes.…”

Section: Discussionmentioning

confidence: 66%

Biliary disease progression in childhood onset autoimmune liver disease: A 30-year follow-up into adulthood

Warner,

Rajanayagam,

Russell

et al. 2024

JHEP Reports

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Section: Discussionmentioning

confidence: 66%

Biliary disease progression in childhood onset autoimmune liver disease: A 30-year follow-up into adulthood

Warner,

Rajanayagam,

Russell

et al. 2024

JHEP Reports

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Outcome of Very Early Onset Inflammatory Bowel Disease Associated With Primary Sclerosing Cholangitis: A Multicenter Study From the Pediatric IBD Porto Group of ESPGHAN

Catassi,

D’Arcangelo,

Norsa

et al. 2023

Inflammatory Bowel Diseases

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Background Whether primary sclerosing cholangitis related to inflammatory bowel disease (PSC-IBD) diagnosed before 6 years (ie, VEO-IBD) has a distinct phenotype and disease course is uninvestigated. We aimed to analyze the characteristics and natural history of VEO-PSC-IBD, compared with early and adolescent-onset PSC-IBD. Methods This is a multicenter, retrospective, case-control study from 15 centers affiliated with the Porto and Interest IBD group of ESPGHAN. Demographic, clinical, laboratory, endoscopic, and imaging data were collected at baseline and every 6 months. Inflammatory bowel disease–related (clinical remission, need for systemic steroids and biologics, and surgery) and PSC-related (biliary and portal hypertensive complications, need for treatment escalation and liver transplantation, cholangiocarcinoma, or death) outcomes were compared between the 2 groups. Results Sixty-nine children were included, with a median follow-up of 3.63 years (interquartile range, 1-11): 28 with VEO-PSC-IBD (23 UC [82%], 2 IBD-U [7%] and 3 [11%] CD), and 41 with PSC-IBD (37 UC [90%], 3 IBDU [7.5%] and 1 [2.5%] CD). Most patients with UC presented with pancolitis (92% in VEO-PSC-UC vs 85% in PSC-UC, P = .2). A higher number of patients with VEO-PSC-IBD were diagnosed with PSC/autoimmune hepatitis overlap syndrome than older children (24 [92%] vs 27 [67.5%] PSC-IBD, P = .03), whereas no other differences were found for PSC-related variables. Time to biliary strictures and infective cholangitis was lower in the VEO-PSC-IBD group (P = .01 and P = .04, respectively), while no difference was found for other outcomes. No cases of cholangiocarcinoma were reported. Conclusions Primary sclerosing cholangitis related to inflammatory bowel disease has similar baseline characteristics whether diagnosed as VEO-IBD or thereafter. A milder disease course in terms of biliary complications characterizes VEO-PSC-IBD.

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

Cited by 2 publications

References 25 publications

Biliary disease progression in childhood onset autoimmune liver disease: A 30-year follow-up into adulthood

Biliary disease progression in childhood onset autoimmune liver disease: A 30-year follow-up into adulthood

Outcome of Very Early Onset Inflammatory Bowel Disease Associated With Primary Sclerosing Cholangitis: A Multicenter Study From the Pediatric IBD Porto Group of ESPGHAN

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