Inflammatory bowel disease with primary sclerosing cholangitis: A Danish population-based cohort study 1977-2011

Jakob Ørskov Sørensen; Ole Haagen Nielsen; Mikael Andersson; Mark Andrew Ainsworth; Henriette Ytting; Erika Bélard; Tine Jess

doi:10.1111/liv.13548

Inflammatory bowel disease with primary sclerosing cholangitis: A Danish population-based cohort study 1977-2011

Jakob Ørskov Sørensen, Ole Haagen Nielsen, Mikael Andersson, Mark Andrew Ainsworth, Henriette Ytting, Erika Bélard, Tine Jess

Department of Clinical Medicine

21 Citations (Scopus)

Abstract

BACKGROUND AND AIMS: Inflammatory bowel disease (IBD) may be complicated by primary sclerosing cholangitis (PSC). We aimed to assess the characteristics of Danish PSC-IBD patients and to compare their prognosis with IBD patients without PSC.

METHODS: A retrospective nationwide population-based cohort of 257 PSC-IBD patients was assessed through Danish national registries and manual scrutiny of patient files.

RESULTS: For all PSC-IBD patients diagnosed after 1976 (n = 222) and 8231 IBD controls (ie, without PSC), the cumulative probability of resective surgery, liver transplantation, cancer, and survival from 1977 through 2011 was estimated and compared by log-rank test and Cox regression. PSC-IBD patients primarily had ulcerative colitis (UC) (72%), were diagnosed in young adulthood (median age at IBD diagnosis, 23 years), and 9% were smokers. Among PSC-UC patients 78% had pancolitis at diagnosis. Among patients with PSC and Crohn's disease (CD) 91% had colonic involvement. The PSC-IBD patients had a significantly higher probability of receiving resective surgery (HR; 2.13, 95% CI: 1.50-3.03); of developing colorectal cancer (CRC) (HR; 21.4, 95% CI: 9.6-47.6), of cholangiocarcinoma (HR; 190, 95% CI: 54.8-660), and of dying (HR; 4.39, 95% CI: 3.22-6.00) as compared to non-PSC-IBD controls. The 25-year cumulative risk of liver transplantation was high (53%).

CONCLUSIONS: This unselected population-based study shows that PSC-IBD patients not only have an extensive phenotype of IBD, they are also treated more intensively than other patients with IBD. However, the prognosis remains poor and without any apparent improvement over calendar time.

Original language	English
Journal	Liver International
Volume	38
Issue number	3
Pages (from-to)	532-541
ISSN	1478-3223
DOIs	https://doi.org/10.1111/liv.13548
Publication status	Published - Mar 2018

Keywords

Adolescent
Adult
Aged
Child
Child, Preschool
Cholangitis, Sclerosing/complications
Colectomy
Colorectal Neoplasms/complications
Denmark/epidemiology
Female
Humans
Inflammatory Bowel Diseases/complications
Liver Transplantation
Male
Middle Aged
Prognosis
Proportional Hazards Models
Retrospective Studies
Risk Factors
Young Adult

UN SDGs

This output contributes to the following UN Sustainable Development Goals (SDGs)

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10.1111/liv.13548

Cite this

@article{e9543b9fb5704cb6897bedbe8ff9a55d,

title = "Inflammatory bowel disease with primary sclerosing cholangitis: A Danish population-based cohort study 1977-2011",

abstract = "BACKGROUND AND AIMS: Inflammatory bowel disease (IBD) may be complicated by primary sclerosing cholangitis (PSC). We aimed to assess the characteristics of Danish PSC-IBD patients and to compare their prognosis with IBD patients without PSC.METHODS: A retrospective nationwide population-based cohort of 257 PSC-IBD patients was assessed through Danish national registries and manual scrutiny of patient files.RESULTS: For all PSC-IBD patients diagnosed after 1976 (n = 222) and 8231 IBD controls (ie, without PSC), the cumulative probability of resective surgery, liver transplantation, cancer, and survival from 1977 through 2011 was estimated and compared by log-rank test and Cox regression. PSC-IBD patients primarily had ulcerative colitis (UC) (72%), were diagnosed in young adulthood (median age at IBD diagnosis, 23 years), and 9% were smokers. Among PSC-UC patients 78% had pancolitis at diagnosis. Among patients with PSC and Crohn's disease (CD) 91% had colonic involvement. The PSC-IBD patients had a significantly higher probability of receiving resective surgery (HR; 2.13, 95% CI: 1.50-3.03); of developing colorectal cancer (CRC) (HR; 21.4, 95% CI: 9.6-47.6), of cholangiocarcinoma (HR; 190, 95% CI: 54.8-660), and of dying (HR; 4.39, 95% CI: 3.22-6.00) as compared to non-PSC-IBD controls. The 25-year cumulative risk of liver transplantation was high (53%).CONCLUSIONS: This unselected population-based study shows that PSC-IBD patients not only have an extensive phenotype of IBD, they are also treated more intensively than other patients with IBD. However, the prognosis remains poor and without any apparent improvement over calendar time.",

keywords = "Adolescent, Adult, Aged, Child, Child, Preschool, Cholangitis, Sclerosing/complications, Colectomy, Colorectal Neoplasms/complications, Denmark/epidemiology, Female, Humans, Inflammatory Bowel Diseases/complications, Liver Transplantation, Male, Middle Aged, Prognosis, Proportional Hazards Models, Retrospective Studies, Risk Factors, Young Adult",

author = "S{\o}rensen, {Jakob {\O}rskov} and Nielsen, {Ole Haagen} and Mikael Andersson and Ainsworth, {Mark Andrew} and Henriette Ytting and Erika B{\'e}lard and Tine Jess",

note = "{\textcopyright} 2017 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.",

year = "2018",

month = mar,

doi = "10.1111/liv.13548",

language = "English",

volume = "38",

pages = "532--541",

journal = "Liver International",

issn = "1478-3223",

publisher = "Wiley-Blackwell",

number = "3",

}

TY - JOUR

T1 - Inflammatory bowel disease with primary sclerosing cholangitis

T2 - A Danish population-based cohort study 1977-2011

AU - Sørensen, Jakob Ørskov

AU - Nielsen, Ole Haagen

AU - Andersson, Mikael

AU - Ainsworth, Mark Andrew

AU - Ytting, Henriette

AU - Bélard, Erika

AU - Jess, Tine

PY - 2018/3

Y1 - 2018/3

N2 - BACKGROUND AND AIMS: Inflammatory bowel disease (IBD) may be complicated by primary sclerosing cholangitis (PSC). We aimed to assess the characteristics of Danish PSC-IBD patients and to compare their prognosis with IBD patients without PSC.METHODS: A retrospective nationwide population-based cohort of 257 PSC-IBD patients was assessed through Danish national registries and manual scrutiny of patient files.RESULTS: For all PSC-IBD patients diagnosed after 1976 (n = 222) and 8231 IBD controls (ie, without PSC), the cumulative probability of resective surgery, liver transplantation, cancer, and survival from 1977 through 2011 was estimated and compared by log-rank test and Cox regression. PSC-IBD patients primarily had ulcerative colitis (UC) (72%), were diagnosed in young adulthood (median age at IBD diagnosis, 23 years), and 9% were smokers. Among PSC-UC patients 78% had pancolitis at diagnosis. Among patients with PSC and Crohn's disease (CD) 91% had colonic involvement. The PSC-IBD patients had a significantly higher probability of receiving resective surgery (HR; 2.13, 95% CI: 1.50-3.03); of developing colorectal cancer (CRC) (HR; 21.4, 95% CI: 9.6-47.6), of cholangiocarcinoma (HR; 190, 95% CI: 54.8-660), and of dying (HR; 4.39, 95% CI: 3.22-6.00) as compared to non-PSC-IBD controls. The 25-year cumulative risk of liver transplantation was high (53%).CONCLUSIONS: This unselected population-based study shows that PSC-IBD patients not only have an extensive phenotype of IBD, they are also treated more intensively than other patients with IBD. However, the prognosis remains poor and without any apparent improvement over calendar time.

AB - BACKGROUND AND AIMS: Inflammatory bowel disease (IBD) may be complicated by primary sclerosing cholangitis (PSC). We aimed to assess the characteristics of Danish PSC-IBD patients and to compare their prognosis with IBD patients without PSC.METHODS: A retrospective nationwide population-based cohort of 257 PSC-IBD patients was assessed through Danish national registries and manual scrutiny of patient files.RESULTS: For all PSC-IBD patients diagnosed after 1976 (n = 222) and 8231 IBD controls (ie, without PSC), the cumulative probability of resective surgery, liver transplantation, cancer, and survival from 1977 through 2011 was estimated and compared by log-rank test and Cox regression. PSC-IBD patients primarily had ulcerative colitis (UC) (72%), were diagnosed in young adulthood (median age at IBD diagnosis, 23 years), and 9% were smokers. Among PSC-UC patients 78% had pancolitis at diagnosis. Among patients with PSC and Crohn's disease (CD) 91% had colonic involvement. The PSC-IBD patients had a significantly higher probability of receiving resective surgery (HR; 2.13, 95% CI: 1.50-3.03); of developing colorectal cancer (CRC) (HR; 21.4, 95% CI: 9.6-47.6), of cholangiocarcinoma (HR; 190, 95% CI: 54.8-660), and of dying (HR; 4.39, 95% CI: 3.22-6.00) as compared to non-PSC-IBD controls. The 25-year cumulative risk of liver transplantation was high (53%).CONCLUSIONS: This unselected population-based study shows that PSC-IBD patients not only have an extensive phenotype of IBD, they are also treated more intensively than other patients with IBD. However, the prognosis remains poor and without any apparent improvement over calendar time.

KW - Adolescent

KW - Adult

KW - Aged

KW - Child

KW - Child, Preschool

KW - Cholangitis, Sclerosing/complications

KW - Colectomy

KW - Colorectal Neoplasms/complications

KW - Denmark/epidemiology

KW - Female

KW - Humans

KW - Inflammatory Bowel Diseases/complications

KW - Liver Transplantation

KW - Male

KW - Middle Aged

KW - Prognosis

KW - Proportional Hazards Models

KW - Retrospective Studies

KW - Risk Factors

KW - Young Adult

U2 - 10.1111/liv.13548

DO - 10.1111/liv.13548

M3 - Journal article

C2 - 28796371

SN - 1478-3223

VL - 38

SP - 532

EP - 541

JO - Liver International

JF - Liver International

IS - 3

ER -

Inflammatory bowel disease with primary sclerosing cholangitis: A Danish population-based cohort study 1977-2011

Abstract

Keywords

UN SDGs

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