Inflammatory Bowel Disease in Primary Sclerosing Cholangitis

Sinakos, Emmanouil; Samuel, Sunil; Enders, Felicity; Loftus, Edward V.; Sandborn, William J.; Lindor, Keith D.

doi:10.1097/mib.0b013e3182802893

Cited by 75 publications

(34 citation statements)

References 19 publications

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“…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%

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: Associated Diseasesmentioning

confidence: 99%

Primary sclerosing cholangitis: A review and update

Tabibian

Bowlus

2017

Liver Research

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“…In a recent cohort (2003–2007), most patients were diagnosed with PSC first; in an earlier cohort (1993–1997), most patients were diagnosed with IBD first. 37 …”

Section: Diagnosis Of Pscmentioning

confidence: 99%

Pathogenesis of Primary Sclerosing Cholangitis and Advances in Diagnosis and Management

et al. 2013

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Primary sclerosing cholangitis (PSC), first described in the mid-1850’s, is a complex liver that is heterogeneous in its presentation. PSC is characterized by chronic cholestasis, associated with chronic inflammation of the biliary epithelium, resulting in multi-focal bile duct strictures that can affect the entire biliary tree. Chronic inflammation leads to fibrosis involving the hepatic parenchyma and biliary tree, which can lead to cirrhosis and malignancy. The etiology of PSC is not fully understood which in part explains a lack of effective medical therapy for this condition. However, we have begun to better understand the molecular pathogenesis of PSC. The recognition of specific clinical subtypes and their pattern of progression could improve phenotypic and genotypic classification of the disease. We review our current understanding of this enigmatic disorder and discuss important topics for future studies.

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“…Yet, pancolitis and rectal sparing are described more frequently, that is, in about 65 and 30%, respectively, of UC PSC patients, compared to 50 and 10% of UC patients without PSC. Backwash ileitis may occur slightly more often in UC PSC (17 vs. 12%), but the data are controversial [4,15,16]. Possibly due to the milder and sometimes subclinical course of the IBD despite pancolitis, the risk of colorectal cancer (CRC) seems to be substantially higher in UC PSC.…”

Section: Background Westmentioning

confidence: 99%

Ulcerative Colitis with and without Primary Sclerosing Cholangitis: Two Different Diseases?

Tanaka

Mertens

2016

Inflamm Intest Dis

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Background: Primary sclerosing cholangitis (PSC) is a chronic cholestatic liver disease of unknown origin and an important hepatobiliary complication of inflammatory bowel diseases (IBD), especially ulcerative colitis (UC). When further differentiated, about 3-8% of UC patients suffer from PSC, whereas among Crohn's disease patients the reported prevalence of PSC is probably between 1 and 3.5%. Although it was reported from Japan that the frequency of PSC in UC was only 34%, the same registry data indicated that the prevalence was up to 57% among young patients with PSC even in Japan, which is comparable to the 60-80% in Europe and the US. Additionally, the clinical features of UC in patients with PSC are different from those in patients without PSC, for instance, rectal sparing and right-sided dominance. Summary: The strong link between atypical IBD and PSC suggests that the pathogenesis of PSC involves pathology of the gut, including abnormal gut microbiota and aberrant activation of mucosal lymphocytes. These seem to be different in UC PSC as compared to the pathology of typical UC. Key Messages: The key to solving the question ‘Is there a difference between East and West?' are genetic studies, genome-wide association studies of PSC in particular, which have already been performed in the West and are strongly warranted in the East.

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Inflammatory Bowel Disease in Primary Sclerosing Cholangitis

Abstract: IBD in PSC has unique characteristics, and the clinical features of this unique presentation have remained stable over time. A shift in the timing of diagnosis of the 2 diseases has occurred in recent years, with PSC being more often diagnosed first.

Cited by 75 publications

References 19 publications

Primary sclerosing cholangitis: A review and update

Primary sclerosing cholangitis: A review and update

Pathogenesis of Primary Sclerosing Cholangitis and Advances in Diagnosis and Management

Ulcerative Colitis with and without Primary Sclerosing Cholangitis: Two Different Diseases?

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