scholarly journals Increased risk of cancer in patients with primary sclerosing cholangitis

2021 ◽  
Author(s):  
Aiva Lundberg Båve ◽  
Annika Bergquist ◽  
Matteo Bottai ◽  
Anna Warnqvist ◽  
Erik von Seth ◽  
...  
Keyword(s):  
Colorectal Cancer ◽  
Pancreatic Cancer ◽  
Primary Sclerosing Cholangitis ◽  
Sclerosing Cholangitis ◽  
Cox Regression ◽  
Hepatobiliary Cancer ◽  
Kaplan Meier ◽  
Increased Risk ◽  
Inflammatory Bowel ◽  
Risk Of Cancer

Abstract Background and aims Primary sclerosing cholangitis (PSC) is associated with an increased risk of hepatobiliary and colorectal cancer, but the risks of other cancer forms have not been explored. The aim of this study was to evaluate the risk of intestinal and extraintestinal cancers in a large, well-defined cohort of PSC patients. Material and method A matched cohort study of Swedish PSC patients was performed with up to ten comparators for each patient, matched for sex, age, and residency. The data were retrieved from national registers. Patients were followed from PSC diagnosis until cancer diagnosis, liver transplantation, first emigration date, death, or December 31, 2016. The risk of cancer was estimated using the Kaplan–Meier method and Cox regression models. Results In total, 1432 PSC patients with a verified diagnosis and 14,437 comparators were studied. The mean follow-up time was 15.9 years. Eighty-eight percent of the PSC patients had concomitant inflammatory bowel disease. PSC patients ran significantly increased risks of developing any cancer [HR 3.8, 95% confidence interval (CI) 3.3–4.3], hepatobiliary cancer (HR 120.9, 95% CI 72.0–203.1), colorectal cancer (HR 7.5, 95% CI 5.6–10.0), pancreatic cancer (HR 8.0, 95% CI 3.2–20.2), gastric cancer (HR 4.2, 95% CI 1.5–11.3), small bowel cancer (HR 21.1, 95% CI 3.5–128.2), and lymphoma (HR 3.0, 95% CI 1.6–5.7). PSC was not associated with a lower risk of any cancer form. Conclusions PSC patients have a four times overall increased risk of developing cancer compared to the general population, with increased risk of developing hepatobiliary, colorectal, and pancreatic cancer, as well as lymphoma.

scholarly journals Is there an excess risk for colorectal cancer in patients with ulcerative colitis and concomitant primary sclerosing cholangitis? A population based study

Gut ◽  
1997 ◽  
Vol 41 (4) ◽  
pp. 522-525 ◽  
Author(s):  
D Kornfeld ◽  
A Ekbom ◽  
T Ihre
Keyword(s):  
Colorectal Cancer ◽  
Ulcerative Colitis ◽  
Primary Sclerosing Cholangitis ◽  
Sclerosing Cholangitis ◽  
Cumulative Risk ◽  
Population Based ◽  
Colorectal Cancers ◽  
Population Based Study ◽  
Increased Risk ◽  
Swedish Cancer Registry

Background—Patients with ulcerative colitis have an increased risk of colorectal cancer. Duration, age, and extent of the disease at diagnosis are the only established risk factors. Patients with ulcerative colitis and concomitant primary sclerosing cholangitis (PSC) have been reported to have a higher frequency of colonic DNA aneuploidy and/or dysplasia than expected, findings indicating an increased risk of colorectal cancer compared with other patients with ulcerative colitis.Methods—A population based cohort consisting of 125 patients with a verified diagnosis of PSC was followed up by linkage to the Swedish Cancer Registry for the occurrence of colorectal cancer.Results—There were 12 colorectal cancers. Six cancers were diagnosed prior to the diagnosis of PSC. Among the 104 patients with an intact colon at the time of the diagnosis of PSC there was a cumulative risk for colorectal cancer of 16% after 10 years. Among the 58 patients with a diagnosis of ulcerative colitis and colorectal cancer prior to the diagnosis of PSC, there were five colorectal cancers corresponding to a cumulative risk of 25% after 10 years.Conclusions—Patients with ulcerative colitis and concomitant PSC seem to constitute a subgroup with a high risk for colorectal cancer.

Clinical Characteristics, Associated Malignancies and Management of Primary Sclerosing Cholangitis in Inflammatory Bowel Disease Patients: A Multicentre Retrospective Cohort Study

2019 ◽  
Vol 13 (12) ◽  
pp. 1492-1500 ◽  
Author(s):  
Ivan Guerra ◽  
Luis Bujanda ◽  
Jesús Castro ◽  
Olga Merino ◽  
Joan Tosca ◽  
...  
Keyword(s):  
Inflammatory Bowel Disease ◽  
Incidence Rate ◽  
Primary Sclerosing Cholangitis ◽  
Bowel Disease ◽  
Sclerosing Cholangitis ◽  
Clinical Characteristics ◽  
Additional Data ◽  
Poor Survival ◽  
Increased Risk ◽  
Inflammatory Bowel

Abstract Background and Aims Primary sclerosing cholangitis [PSC] is usually associated with inflammatory bowel disease [IBD]. An increased risk of malignancies, mainly colorectal cancer [CRC] and cholangiocarcinoma [CCA], has been reported in PSC-IBD patients. Our aim was to determine the clinical characteristics and management of PSC in IBD patients, and the factors associated with malignancies. Methods PSC-IBD patients were identified from the Spanish ENEIDA registry of GETECCU. Additional data were collected using the AEG-REDCap electronic data capture tool. Results In total, 277 PSC-IBD patients were included, with an incidence rate of 61 PSC cases per 100 000 IBD patient-years, 69.7% men, 67.5% ulcerative colitis and mean age at PSC diagnosis of 40 ± 16 years. Most patients [85.2%] were treated with ursodeoxycholic acid. Liver transplantation was required in 35 patients [12.6%] after 79 months (interquartile range [IQR] 50–139). It was more common in intra- and extrahepatic PSC compared with small-duct PSC (16.3% vs 3.3%; odds ratio [OR] 5.7: 95% confidence interval [CI] = 1.7–19.3). The incidence rate of CRC since PSC diagnosis was 3.3 cases per 1000 patient-years [95% CI = 1.9–5.6]. Having symptoms of PSC at PSC diagnosis was the only factor related to an increased risk of CRC after IBD diagnosis [hazard ratio= 3.3: 95% CI = 1.1–9.9]. CCA was detected in seven patients [2.5%] with intra- and extrahepatic PSC, with median age of 42 years [IQR 39–53], and presented a lower life expectancy compared with patients without CCA and patients with or without CRC. Conclusions PSC-IBD patients with symptoms of PSC at PSC diagnosis have an increased risk of CRC. CCA was only diagnosed in patients with intra- and extrahepatic PSC and was associated with poor survival.

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