Primary sclerosing cholangitis: Natural history, prognostic factors and survival analysis

Wiesner, Russell H.; Grambsch, Patricia M.; Dickson, E. Rolland; Ludwig, Jürgen; MacCarty, Robert L.; Hunter, Ellen B.; Fleming, Thomas R.; Fisher, Lloyd D.; Beaver, Sandra J.; LaRusso, Nicholas F.

doi:10.1002/hep.1840100406

Cited by 607 publications

(429 citation statements)

References 24 publications

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“…Some asymptomatic patients survive for prolonged periods of time without developing significant complications of the disease. 11 However, the majority of patients with symptomatic PSC have a progressive disease that typically results in liver failure within 10 to 12 years. 12 No specific treatment has been shown to improve survival in patients with PSC.…”

Section: Pbcmentioning

confidence: 99%

Liver transplantation

Carithers

2000

Liver Transpl

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Liver transplantation has revolutionized the care of patients with end-stage liver disease. Liver transplantation is indicated for acute or chronic liver failure from any cause. Because there are no randomized controlled trials of liver transplantation versus no therapy, the efficacy of this surgery is best assessed by carefully comparing postoperative survival with the known natural history of the disease in question. The best examples of this are in primary biliary cirrhosis and primary sclerosing cholangitis, for which well-validated disease-specific models of natural history are available. There are currently relatively few absolute contraindications to liver transplantation. These include severe cardiopulmonary disease, uncontrolled systemic infection, extrahepatic malignancy, severe psychiatric or neurological disorders, and absence of a viable splanchnic venous inflow system. One of the most frequently encountered contraindications to transplantation is ongoing destructive behavior caused by drug and alcohol addiction. The timing of the surgery can have a profound impact on the mortality and morbidity of patients undergoing liver transplantation. Because of the long waiting lists for donor organs, the need to project far in advance when transplantation might be required has proven to be one of the greatest challenges to those treating patients with end-stage liver disease. Three important questions must be addressed in a patient being considered for liver transplantation: (1) when should the patient be referred for possible transplantation? (2) when should the patient be listed for transplantation? and (3) when is the patient too sick to have a reasonable chance of surviving the perioperative period?

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Section: Pbcmentioning

confidence: 99%

Liver transplantation

Carithers

2000

Liver Transpl

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“…PSC follows a progressive course that can culminate in cirrhosis, portal hypertension, liver failure, and/or cholangiocarcinoma (CCA). Median transplant‐free survival from time of PSC diagnosis is variable, ranging from approximately 12‐21 years in most studies, with more recent estimates being toward the top end of this range 4, 5, 6, 7, 8. It has been reported that 40%‐58% of patients with PSC will develop focal high‐grade strictures, referred to as a “dominant stricture” (DS), during the course of disease 5, 9, 10, 11, 12.…”

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Dominant strictures in primary sclerosing cholangitis: A multicenter survey of clinical definitions and practices

Hilscher

Tabibian

Carey

et al. 2018

Hepatology Communications

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Dominant strictures (DSs) of the biliary tree occur in approximately 50% of patients with primary sclerosing cholangitis (PSC) and may cause significant morbidity. Nevertheless, the definition and management of DSs lacks consensus. We aimed to better understand current perceptions and practices regarding PSC‐associated DSs. We conducted an anonymous, 23‐question, survey‐based study wherein electronic surveys were distributed to 131 faculty in the Division of Gastroenterology and Hepatology at the three Mayo Clinic campuses (Rochester, Scottsdale, and Jacksonville) as well as the affiliated practice network. Responses were aggregated and compared, where applicable, to practice guidelines of the American Association for the Study of Liver Diseases and European Association for the Study of the Liver. A total of 54 faculty (41.2%) completed the survey, of whom 24 (44.4%) were hepatologists, 21 (38.9%) gastroenterologists, and 9 (16.7%) advanced endoscopists. One of the major study findings was that there was heterogeneity among participants' definition, evaluation, management, and follow‐up of DSs in PSC. The majority of participant responses were in accordance with societal practice guidelines, although considerable variation was noted. Conclusion: Despite the prevalence and morbidity of DSs in PSC, clinical perceptions and practices vary widely among hepatologists, gastroenterologists, and advanced endoscopists who manage these patients, even within a single health care system. Further studies are needed to address these variations, develop general and evidence‐based consensus, and increase adherence to societal guidelines. (Hepatology Communications 2018;2:836‐844)

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“…It is a progressive disorder in which approximately 50% of symptomatic patients eventually develop cirrhosis and liver failure. 1 Liver transplantation is an excellent treatment for advanced liver disease caused by PSC, and is one of the most frequent indications for liver transplantation in the United States. [2][3][4][5][6][7] Cholangiocarcinoma (CCA) is a recognized complication of PSC.…”

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confidence: 99%

“…[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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Primary sclerosing cholangitis: Natural history, prognostic factors and survival analysis

Cited by 607 publications

References 24 publications

Liver transplantation

Liver transplantation

Dominant strictures in primary sclerosing cholangitis: A multicenter survey of clinical definitions and practices

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

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