Survival and Risk of Cholangiocarcinoma in Patients with Primary Sclerosing Cholangitis: A Population-Based Study

Kornfeld, Dan; Ekbom, Anders; Ihre, T

doi:10.3109/00365529709011222

Cited by 125 publications

(71 citation statements)

References 19 publications

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“…Cholangiocarcinoma develops frequently in patients with primary sclerosing cholangitis (PSC) and is currently a leading cause of death in patients with this disease [1][2][3][4][5][6]. However, the widespread biliary strictures associated with PSC make the diagnosis of cholangiocarcinoma particularly difficult.…”

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confidence: 99%

Chromosome 9p21 Loss and p16 Inactivation in Primary Sclerosing Cholangitis-Associated Cholangiocarcinoma

Ahrendt

Eisenberger

Yip

et al. 1999

Journal of Surgical Research

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confidence: 99%

Chromosome 9p21 Loss and p16 Inactivation in Primary Sclerosing Cholangitis-Associated Cholangiocarcinoma

Ahrendt

Eisenberger

Yip

et al. 1999

Journal of Surgical Research

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“…[2][3][4][5][6][7] Cholangiocarcinoma (CCA) is a recognized complication of PSC. The reported frequency of CCA has ranged between 6% to 11% in the studies describing the natural history of PSC 1,[8][9][10][11] and 7% to 36% in PSC patients undergoing liver transplantation. 4,6,7,[12][13][14] The occurrence of CCA is unpredictable and is often difficult to diagnose.…”

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confidence: 99%

Cholangiocarcinoma in patients with primary sclerosing cholangitis: A multicenter case-control study

et al. 2000

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Patients with primary sclerosing cholangitis (PSC) have a significantly increased risk of developing cholangiocarcinoma (CCA). Risk factors for developing such a complication are not well defined. We conducted a multicenter, case-control study to determine the risk factors and possible predictors for CCA in patients with PSC. The demographic, clinical, and laboratory features of 26 PSC patients with CCA diagnosed over a 7-year period at eight academic centers were compared with 87 patients with PSC but no CCA (controls). There was no statistically significant difference in demographics, smoking, signs or symptoms or complications of PSC, indices of disease severity (Mayo Risk score or Child-Pugh score), frequency or duration or complications of inflammatory bowel disease (IBD), frequency of biliary surgery, or therapeutic endoscopy between the two groups. Alcohol consumption was significantly associated with CCA in patients with PSC (odds ratio: 2.95; 95% CI: 1.04-8.3). Serum carbohydrate antigen 19-9 (CA 19-9) was significantly higher in patients with CCA than those without (177 ؎ 89 and 61 ؎ 58 U/mL, respectively; P ‫؍‬ .002). A serum CA 19-9 level G 100 U/mL had 75% sensitivity and 80% specificity in identifying PSC patients with CCA. In conclusion, alcohol consumption was a risk factor for having CCA in PSC patients. The indices of severity of liver disease were not associated with CCA in patients with PSC. Serum CA 19-9 appeared to have good ability to discriminate PSC patients with and without CCA. (HEPATOLOGY 2000;31:7-11.)Primary sclerosing cholangitis (PSC) is a chronic cholestatic liver disorder that is characterized by multiple fibrotic strictures of the intra-and extrahepatic biliary tree. It is a progressive disorder in which approximately 50% of symptomatic patients eventually develop cirrhosis and liver failure. 1

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“…In particular, an excess risk of malignant lymphomas in rheumatoid arthritis, SLE, Sjögren's syndrome and coeliac disease has been reported, as have cancers of the afflicted tissues in inflammatory bowel disease (colorectal cancer), 7 coeliac disease (small intestine carcinomas) 13 and in primary sclerosing cholangitis (cholangiocarcinoma). 14 There have in fact been reports of a highly increased risk for cancer of the urinary bladder and for non-Hodgkin's lymphoma among Wegener's granulomatosis patients, but the patient series have been small and highly selected. 5,6 Since no previous study has provided a global assessment of the cancer occurrence in patients with Wegener's granulomatosis, little is known about the overall cancer incidence following this disease.…”

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Cancer incidence in a population‐based cohort of patients with Wegener's granulomatosis

Knight

Askling

Ekbom

2002

Intl Journal of Cancer

177

139

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Wegener's granulomatosis is necrotizing granulomatous vasculitis of unknown origin, which untreated has a high mortality within the first year of onset. The introduction of corticosteroids and cyclophosphamide in the treatment has considerably improved survival rates, but past studies have indicated an increased cancer risk, including an increased risk for urinary bladder cancer. No large assessment of the general cancer occurrence in Wegener's granulomatosis has been reported. The aim of our study was to assess the general incidence of cancer in patients with Wegener's granulomatosis and to put this in relation to the risk for bladder cancer. We identified a population-based cohort of 1,065 patients with Wegener's granulomatosis in the Swedish Inpatient Register. Through linkage with the Swedish Cancer Register, we followed the cohort for cancer occurrence for up to 26 years. Standardized incidence ratios (SIR) between observed and expected numbers of cancers were used as a measure of relative risk. There was a 2-fold overall increased risk for cancer in the cohort. The increase was most pronounced for bladder cancer (SIR ‫؍‬ 4.8; 95% CI 2.6 -8.1), squamous cell skin cancer (SIR ‫؍‬ 7.3; 95% CI 4.4 -12), leukemias (SIR ‫؍‬ 5.7; 95% CI 2.3-12) and for malignant lymphomas (SIR ‫؍‬ 4.2; 95% CI 4.2-8.3). The results confirm previous indications of an increased risk for cancer of the urinary bladder but also points to increased risks for cancer at other sites.

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Survival and Risk of Cholangiocarcinoma in Patients with Primary Sclerosing Cholangitis: A Population-Based Study

Abstract: Patients with PSC have a substantially decreased survival, which is most pronounced among patients without IBD.

Cited by 125 publications

References 19 publications

Chromosome 9p21 Loss and p16 Inactivation in Primary Sclerosing Cholangitis-Associated Cholangiocarcinoma

Chromosome 9p21 Loss and p16 Inactivation in Primary Sclerosing Cholangitis-Associated Cholangiocarcinoma

Cholangiocarcinoma in patients with primary sclerosing cholangitis: A multicenter case-control study

Cancer incidence in a population‐based cohort of patients with Wegener's granulomatosis

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