Inflammatory Bowel Disease Is Associated With Poor Outcomes of Patients With Primary Sclerosing Cholangitis

Ngu, Jing Hieng; Gearry, Richard B.; Wright, Andrew Jeffrey; Stedman, Catherine

doi:10.1016/j.cgh.2011.08.027

Cited by 89 publications

(65 citation statements)

References 35 publications

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“…Outside the USA, comparable incidence and prevalence rates have been reported in Canada, 16 Northern Europe, 17,18 and New Zealand, 19 and recent data suggest that these rates are rising (at least in North American and European countries). 20 In Japan and Singapore, the available published data suggest that PSC is rare.…”

Section: Epidemiologymentioning

confidence: 66%

“…50,114,115 The classic teaching is that there is no relationship between the severity of bowel disease and the severity of liver disease. While this may be true, it is not to say that one has no influence on the other, as illustrated by the following examples: (i) the presence of IBD (irrespective of severity) has been associated with greater PSC-related morbidity and mortality; 19,70 (ii) having IBD and an intact colon before LT appears to be a predictor of recurrent PSC post-LT; 116 (iii) patients with PSC-IBD typically demonstrate a unique phenotype of IBD characterized by pancolitis with rectal sparing and backwash ileitis (and a greater risk of post-colectomy pouchitis); 115,117 (iv) PSC does not appear to be associated with small bowel-only Crohn’s disease; 118,119 and (v) colitis is usually milder (even if more extensive) in patients with PSC-IBD compared to those with IBD alone. 120 …”

Section: Associated Diseasesmentioning

confidence: 99%

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Primary sclerosing cholangitis: A review and update

Tabibian

Bowlus

2017

Liver Research

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Primary sclerosing cholangitis (PSC) is a rare, chronic, cholestatic liver disease of uncertain etiology characterized biochemically by cholestasis and histologically and cholangiographically by fibro-obliterative inflammation of the bile ducts. In a clinically significant proportion of patients, PSC progresses to cirrhosis, end-stage liver disease, and/or hepatobiliary cancer, though the disease course can be highly variable. Despite clinical trials of numerous pharmacotherapies over several decades, safe and effective medical therapy remains to be established. Liver transplantation is an option for select patients with severe complications of PSC, and its outcomes are generally favorable. Periodic surveillance testing for pre- as well as post-transplant patients is a cornerstone of preventive care and health maintenance. Here we provide an overview of PSC including its epidemiology, etiopathogenesis, clinical features, associated disorders, surveillance, and emerging potential therapies.

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Section: Epidemiologymentioning

confidence: 66%

Section: Associated Diseasesmentioning

confidence: 99%

Primary sclerosing cholangitis: A review and update

Tabibian

Bowlus

2017

Liver Research

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“…Whilst the disease can present at any age, the mean age of presentation is 40 years. Prevalence rates are of the order of 0.22 per 100,000 to as high as 16.2 per 100,000 [2,3,4]. A recent systematic review found the incidence to be as high as 1.3 per 100,000 per year in some populations [5].…”

Section: Epidemiologymentioning

confidence: 99%

Primary Sclerosing Cholangitis

Williamson

Chapman

2014

Dig Dis

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Primary sclerosing cholangitis (PSC) is a chronic, cholestatic liver disease caused by diffuse inflammation and fibrosis that can involve the entire biliary tree. It is a progressive disorder which can ultimately lead to biliary cirrhosis, portal hypertension and hepatic failure. PSC is a complex genetic disorder with male predominance. Environmental predisposing factors include non-smoking. It is closely associated with inflammatory bowel disease (IBD), particularly ulcerative colitis, which occurs in about two thirds of PSC cases. Recent studies have suggested that PSC-IBD is a separate disease entity from IBD alone with distinctive genetic and phenotypic characteristics. Most PSC patients are asymptomatic at presentation; clinical symptoms include fatigue, jaundice, weight loss, right upper quadrant pain and pruritis. Serum biochemical tests indicate cholestasis, and diagnosis is usually established by cholangiography. In symptomatic patients, median survival from presentation to death or liver transplantation is about 12 years. It is a premalignant condition, and the majority of deaths are from malignancy, particularly cholangiocarcinoma or colonic cancer. PSC has no curative treatment. Medical treatment with ursodeoxycholic acid may slow progression of the disease and reduce colonic dysplasia, though trials lack statistical significance. Liver transplantation is the only option in young patients with PSC and advanced liver disease.

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“…Many patients are listed for symptoms such as recurrent cholangitis. Concurrent IBD may be associated with a worse prognosis and lower transplant-free survival 32. Moreover, the risk of developing pouchitis in PSC-IBD is significant, and the presence of an ileoanal pouch increases the risk of hepatic artery thrombosis and graft dysfunction.…”

Section: Primary Sclerosing Cholangitismentioning

confidence: 99%

Large-duct cholangiopathies: aetiology, diagnosis and treatment

Menon

Holt

2019

Frontline Gastroenterol

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Cholangiopathies describe a group of conditions affecting the intrahepatic and extrahepatic biliary tree. Impairment to bile flow and chronic cholestasis cause biliary inflammation, which leads to more permanent damage such as destruction of the small bile ducts (ductopaenia) and biliary cirrhosis. Most cholangiopathies are progressive and cause end-stage liver disease unless the physical obstruction to biliary flow can be reversed. This review considers large-duct cholangiopathies, such as primary sclerosing cholangitis, ischaemic cholangiopathy, portal biliopathy, recurrent pyogenic cholangitis and Caroli disease.

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Inflammatory Bowel Disease Is Associated With Poor Outcomes of Patients With Primary Sclerosing Cholangitis

Cited by 89 publications

References 35 publications

Primary sclerosing cholangitis: A review and update

Primary sclerosing cholangitis: A review and update

Primary Sclerosing Cholangitis

Large-duct cholangiopathies: aetiology, diagnosis and treatment

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