Characterization, outcome, and prognosis in 273 patients with primary sclerosing cholangitis: a single center study

Tischendorf, Jens J. W.; Hecker, Hartmut; Krüger, Marcus; Manns, Michael P.; Meier, P. N.

doi:10.1055/s-2005-920038

Cited by 90 publications

(200 citation statements)

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“…It is a slowly progressive disease(3) and the median survival from diagnosis to liver transplantation or death is 9.6∼12 years (4,5). Previous studies have reported that HLA-DR4 and DRw52a are markers for rapid disease progression in PSC(6,7), but this is still controversial (8).…”

Section: Discussionmentioning

confidence: 99%

A Case of Rapidly Progressive Primary Sclerosing Cholangitis Requiring Liver Transplantation

Lim

Park

Kim

et al. 2013

Korean J Transplant

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Primary sclerosing cholangitis (PSC) is a slowly progressive cholestatic liver disease. In cases of PSC, liver transplantation is the only effective treatment that can delay the disease's natural course. We report a case of rapidly progressive PSC requiring liver transplantation. A 52-year-old woman visited our hospital with abdominal pain. There was no evidence of PSC, as there was no elevation in cholestatic liver enzymes at her first visit. Although her total bilirubin was in a normal range at the initial visit, liver dysfunction progressed rapidly. Despite endoscopic procedures and ursodeoxycholic acid intake, total bilirubin levels rose to 18.9 mg/dL, and liver transplantation was performed 17 months after her first visit. PSC was pathologically confirmed after liver transplantation.

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Section: Discussionmentioning

confidence: 99%

A Case of Rapidly Progressive Primary Sclerosing Cholangitis Requiring Liver Transplantation

Lim

Park

Kim

et al. 2013

Korean J Transplant

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“…Biliary cirrhosis and its complications, such as portal hypertension and advancedstage liver failure, occur in a significant proportion of patients owing to obliteration of the biliary tract (11,15). Based on the clinical variables proven to correlate independently with prognosis, predicting survival is of great importance for defining a strategy of therapy and for timing orthotopic liver transplantation.…”

Section: Discussionmentioning

confidence: 99%

“…Because of the fluctuating nature of the disease, it is difficult to determine when liver transplantation may be most effective before the onset of debilitating disease (11,12).…”

Section: Introductionmentioning

confidence: 99%

Comparative assessment of prognostic value for revised-mayo risk model and child-pugh score in patients with primary sclerosing cholangitis

Öztaş¹,

Etık²,

Metin³

et al. 2015

Turk J Gastroenterol

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Background/Aims: The aim of this study was to compare the utility of the revised Mayo risk model (rMRM) and Child-Pugh scores (CPSs) for predicting the prognosis of disease in patients with primary sclerosing cholangitis (PSC). Materials and Methods: Patients were divided into 2 groups: Group I (37 patients; alive and not requiring liver transplantation) and Group II (8 patients; deceased or requiring liver transplantation). rMRM suggests the possible survival percentage over a 4-year period. Thus, rMRM scores and CPSs on the first visit were calculated from the data at the time of diagnosis for patients diagnosed with PSC <4 years ago. rMRM scores and CPSs of patients with >4 years of follow-up were calculated using data from the visit 4 years prior to their last follow-up. Results: Bivariate analyses showed that need for liver transplantation/mortality was correlated with either first visit CPS (r=0.481, p=0.001) or rMRM (r=0.452, p=0.002). Analysis of the area under the curve showed that both models performed similarly in terms of predicting the need for liver transplantation/mortality (rMRM: 0.780; CPS: 0.762; p=0.8). There was a significant difference in Kaplan-Meier survival rates between Group I and Group II for both risk models (rMRM: p<0.001; CPS: p<0.001) when the decisive event was death or need for liver transplantation. Conclusion: Both rMRM and CPSs are useful in risk assessment of patients with PSC. The ability to predict prognosis is similar for both risk models.

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“…Furthermore, up to 5% of patients develop CCA within 12 months of PSC diagnosis [Bergquist et al 1998Tischendorf et al 2007]. 'Small-duct PSC' has a considerably better outcome than the classical form, with a longer transplant-free and overall survival (29 years versus 17 years) [Björnsson et al 2002[Björnsson et al , 2008Broomé et al 2002;Angulo et al 2002].…”

Section: Primary Sclerosing Cholangitismentioning

confidence: 99%

Treatment of autoimmune liver disease: current and future therapeutic options

Trivedi

Hirschfield

2013

Therapeutic Advances in Chronic Disease

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Autoimmune liver disease spans three predominant processes, from the interface hepatitis of autoimmune hepatitis to the lymphocytic cholangitis of primary biliary cirrhosis, and finally the obstructive fibrosing sclerotic cholangiopathy of primary sclerosing cholangitis. Although all autoimmune in origin, they differ in their epidemiology, presentation and response to immunosuppressive therapy and bile acid based treatments. With an ongoing better appreciation of disease aetiology and pathogenesis, treatment is set ultimately to become more rational. We provide an overview of current and future therapies for patients with autoimmune liver disease, with an emphasis placed on some of the evidence that drives current practice.

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Characterization, outcome, and prognosis in 273 patients with primary sclerosing cholangitis: a single center study

Cited by 90 publications

References 0 publications

A Case of Rapidly Progressive Primary Sclerosing Cholangitis Requiring Liver Transplantation

A Case of Rapidly Progressive Primary Sclerosing Cholangitis Requiring Liver Transplantation

Comparative assessment of prognostic value for revised-mayo risk model and child-pugh score in patients with primary sclerosing cholangitis

Treatment of autoimmune liver disease: current and future therapeutic options

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