Natural history and prognostic factors in 305 Swedish patients with primary sclerosing cholangitis.

Broomé, Ulrika; Olsson, Rolf; Lööf, Lars; Bodemar, Göran; Hultcrantz, Rolf; Danielsson, Åke; Prytz, Hanne; Sandberg-Gertzén, Hanna; Wallerstedt, Sven; Lindberg, Greger

doi:10.1136/gut.38.4.610

Cited by 760 publications

(608 citation statements)

References 25 publications

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“…5 A majority of PSC patients have UC (60-100%), whereas a much smaller number of UC patients develop the liver disease (2.4-7.5%) in Caucasian populations. 6 Both diseases share certain autoimmune features including their close association with other autoimmune diseases and the presence of both an antineutrophil cytoplasmic antibody (ANCA) and antibody to tropomyosin. 7 Other studies on the mucosal immune system in UC patients have demonstrated large numbers of IgG plasma cells and elevated interleukin-5 (IL-5) levels, properties that support a humoral or T helper (Th)2 immune response.…”

Section: Introductionmentioning

confidence: 99%

CCR5-Δ32 mutation is strongly associated with primary sclerosing cholangitis

et al. 2004

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Section: Introductionmentioning

confidence: 99%

CCR5-Δ32 mutation is strongly associated with primary sclerosing cholangitis

et al. 2004

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“…The prevalence of the tumor in PSC varies from 4% to 20%. [41][42][43][44][45] The autopsy prevalence is much higher with cholangiocarcinoma, it being reported in 30% to 42% of patients with PSC undergoing autopsy. [45][46][47][48] Cholangiocarcinoma may develop in one third of patients with PSC if follow-up is extended long enough.…”

Section: Malignancymentioning

confidence: 99%

Primary sclerosing cholangitis

1999

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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.…”

mentioning

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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Natural history and prognostic factors in 305 Swedish patients with primary sclerosing cholangitis.

Cited by 760 publications

References 25 publications

CCR5-Δ32 mutation is strongly associated with primary sclerosing cholangitis

CCR5-Δ32 mutation is strongly associated with primary sclerosing cholangitis

Primary sclerosing cholangitis

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

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