Primary Sclerosing Cholangitis: Evolving Concepts in Diagnosis and Treatment

Harnois, Denise M.; Lindor, Keith D.

doi:10.1159/000171586

Cited by 27 publications

(8 citation statements)

References 69 publications

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“…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. 12 In recent series, transplant outcomes for patients with PSC equal or surpass those reported for PBC, with 3-year survival rates greater than 90%.…”

Section: Pbcmentioning

confidence: 99%

“…12 No specific treatment has been shown to improve survival in patients with PSC. 12 In recent series, transplant outcomes for patients with PSC equal or surpass those reported for PBC, with 3-year survival rates greater than 90%. 13 Using disease-specific prognostic models developed for PSC, survival after liver transplantation has been far superior to that predicted for patients treated conservatively.…”

Section: Pbcmentioning

confidence: 99%

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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%

Section: Pbcmentioning

confidence: 99%

Liver transplantation

Carithers

2000

Liver Transpl

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“…Primary sclerosing cholangitis (PSC) is a chronic cholestatic disease of unknown etiology characterized by inflammation, fibrosis, and destruction of the intrahepatic and extrahepatic bile ducts. [1][2][3][4] Irregular narrowing and dilation of the biliary duct system produces the characteristic finding of a beaded pattern on cholangiogram. PSC is a major cause of bile duct stenosis and malignant degeneration resulting in cholangiocarcinoma is a well-recognized sequela of PSC.…”

mentioning

confidence: 99%

Primary sclerosing cholangitis as a cause of false positive bile duct brushing cytology: Report of two cases.

Layfield¹,

Cramer²

2005

Diagnostic Cytopathology

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Primary sclerosing cholangitis (PSC) is a chronic cholestatic liver disease of unknown etiology characterized by ongoing inflammation, destruction, and fibrosis of intrahepatic and extrahepatic bile ducts. Irregular narrowing and dilation of the biliary duct system produces the characteristic beaded pattern seen on cholangiogram. Malignant degeneration resulting in cholangiocarcinoma is a well-recognized sequela of PSC. Bile duct brushing cytology is the primary screening technique for cholangiocarcinoma. It is associated with a relatively low sensitivity but high specificity. Few false positive bile duct brushings have been reported in the literature, with the majority of these having occurred in a background of PSC. We report two patients with PSC in whom bile duct brush cytologies were falsely positive for carcinoma.

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“…There is no benefit for patients with more advanced disease [119]. Unwarranted surgical intervention may complicate a future liver transplantation [120]. …”

Section: Treatment Of Pscmentioning

confidence: 99%

Medical Treatment of Primary Biliary Cirrhosis and Primary Sclerosing Cholangitis

Holtmeier

Leuschner²

2001

Digestion

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Treatment of primary biliary cirrhosis (PBC) and primary sclerosing cholangitis (PSC) with ursodeoxycholic acid (UDCA) has been in common use since 1985. In PBC, treatment with UDCA improves laboratory data, liver histology, enables a longer transplantation-free interval and prolongs disease survival. Because UDCA is unable to cure the disease newer drugs or combination therapies are still needed. Studies with UDCA and immunosuppressants such as prednisone, budesonide and azathioprine have shown that in selected patients combination therapy may be superior to UDCA monotherapy. PSC is treated successfully with UDCA and endoscropic dilatation of the bile duct strictures. Treatment of extrahepatic manifestations of cholestatic liver disease such as pruritus, fatigue, osteoporosis and steatorrhea can be problematic and time-consuming.

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Primary Sclerosing Cholangitis: Evolving Concepts in Diagnosis and Treatment

Cited by 27 publications

References 69 publications

Liver transplantation

Liver transplantation

Primary sclerosing cholangitis as a cause of false positive bile duct brushing cytology: Report of two cases.

Medical Treatment of Primary Biliary Cirrhosis and Primary Sclerosing Cholangitis

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