Inflammatory bowel disease in patients with primary sclerosing cholangitis: Clinical characterization in liver transplanted and nontransplanted patients

Jørgensen, Kristin Kaasen; Grzyb, Krzysztof; Lundin, Knut E.A.; Clausen, O. P. F.; Aamodt, Geir; Schrumpf, E.; Vatn, Morten H.; Boberg, Kirsten Muri

doi:10.1002/ibd.21699

Cited by 113 publications

(87 citation statements)

References 37 publications

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“…Interestingly, when a patient undergoes liver transplantation for PSC, their IBD may worsen in severity, raising the issue of whether the presence of PSC has a protective effect on the course of IBD [26,27]. However, a recent study contradicts this, displaying a lower index of clinical activity in those with PSC-IBD who have undergone liver transplantation compared with those who have not [28]. …”

Section: Ibd In Pscmentioning

confidence: 81%

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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Section: Ibd In Pscmentioning

confidence: 81%

Primary Sclerosing Cholangitis

Williamson

Chapman

2014

Dig Dis

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“…Biopsies should be taken at the index-endoscopy even without macroscopic signs of inflammation (126,131,132).…”

Section: Endoscopic Modalitymentioning

confidence: 99%

Role of endoscopy in primary sclerosing cholangitis: European Society of Gastrointestinal Endoscopy (ESGE) and European Association for the Study of the Liver (EASL) Clinical Guideline

Aabakken¹,

Karlsen²,

Albert³

et al. 2017

Journal of Hepatology

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“…133,[137][138][139] In the report of Sano et al, 138 11 (55%) of 20 PSC-IBD patients showed right-sided colitis on colonoscopy, and 13 (87%) of 15 showed right-sidepredominant inflammation on histologic examination. On the other hand, some authors reported tendency for pancolitis in PSC-UC, and no statistically significant difference in the frequency of rectal sparing and backwash ileitis, using stricter histologic definitions.…”

Section: Clinicopathologic Characteristicsmentioning

confidence: 96%

“…[140][141][142] Liver transplantation does not appear to reduce either CAC risk or disease activity in this population. 137,143 Therefore, IBD patients with concurrent PSC are subject to immediate annual surveillance colonoscopy and biopsy of the entire colon. 46,142 IBD IN ASIA The incidence and prevalence of IBD in Asia have risen in the last 3 to 4 decades, though they are still lower in Asia than in the West.…”

Section: Cancer Risk and Surveillancementioning

confidence: 99%

Clinical Aspects of Idiopathic Inflammatory Bowel Disease: A Review for Pathologists

Lee¹,

Westerhoff²,

Shen³

et al. 2016

Archives of Pathology &Amp; Laboratory Medicine

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Context.—Idiopathic inflammatory bowel disease manifests with different clinical phenotypes showing varying behavior and risk for neoplasia. The clinical questions that are posed to pathologists differ depending on phase of the disease and the clinical circumstances. Understanding the clinical aspects of the dynamic disease process will enhance the role of pathology in optimizing the care of patients with inflammatory bowel disease. Objective.—To review clinical and surgical aspects of inflammatory bowel disease that are relevant to practicing pathologists. Data Sources.—The literature was reviewed. Conclusions.—Diagnosis and management of inflammatory bowel disease require an integrated evaluation of clinical, endoscopic, radiologic, and pathologic features. Therefore, close interaction between clinicians and pathologists is crucial. Having this team approach improves understanding of the pertinent clinical and surgical aspects of the disease and assists in the recognition of unusual presentation of variants, as well as mimics of idiopathic inflammatory bowel disease, by pathologists.

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Inflammatory bowel disease in patients with primary sclerosing cholangitis: Clinical characterization in liver transplanted and nontransplanted patients

Abstract: PSC-IBD may represent a distinct entity of colitis in which low endoscopic activity may mask an active histologic inflammation that possibly contributes to an increased risk of malignancy. Circumstances related to liver transplantation seem to act favorably on colonic inflammation in PSC.

Cited by 113 publications

References 37 publications

Primary Sclerosing Cholangitis

Primary Sclerosing Cholangitis

Role of endoscopy in primary sclerosing cholangitis: European Society of Gastrointestinal Endoscopy (ESGE) and European Association for the Study of the Liver (EASL) Clinical Guideline

Clinical Aspects of Idiopathic Inflammatory Bowel Disease: A Review for Pathologists

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