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Current Concepts in Primary Biliary Cirrhosis and Primary Sclerosing Cholangitis.

Sclair SN, Little E, Levy C - Clin Transl Gastroenterol (2015)

Bottom Line: Ursodeoxycholic acid (UDCA) is the recommended treatment for PBC, and recent studies with obeticholic acid showed promising results for UDCA non-responders.Unlike PBC, no therapy has been shown to alter the natural history of PSC.In addition to the lack of therapy, PSC is a pre-malignant condition and close surveillance is indicated.

View Article: PubMed Central - PubMed

Affiliation: Schiff Center for Liver Diseases, University of Miami Miller School of Medicine, Miami, Florida, USA.

ABSTRACT
Primary biliary cirrhosis (PBC) and primary sclerosing cholangitis (PSC) are chronic, cholestatic diseases of the liver with common clinical manifestations. Early diagnosis and treatment of PBC slows progression and decreases the need for transplant. However, one-third of patients will progress regardless of treatment. Bilirubin <1.0 and alkaline phosphatase <2.0 x the upper limit of normal at 1 year after treatment appear to predict 10-year survival. Ursodeoxycholic acid (UDCA) is the recommended treatment for PBC, and recent studies with obeticholic acid showed promising results for UDCA non-responders. Unlike PBC, no therapy has been shown to alter the natural history of PSC. The recommended initial diagnostic test for PSC is magnetic resonance cholangiopancreatography, typically showing bile duct wall thickening, focal bile duct dilatation, and saccular dilatation of the intra- and/or extrahepatic bile ducts. Immunoglobulin 4-associated cholangitis must be excluded when considering the diagnosis of PSC, to allow for proper treatment, and monitoring of disease progression. In addition to the lack of therapy, PSC is a pre-malignant condition and close surveillance is indicated.

No MeSH data available.


Related in: MedlinePlus

Proposed algorithm for UDCA use in clinical practice. Modified from ref. 96. *Surveillance and management options reviewed on cancer surveillance section. **Referral to cholestatic liver disease specialist and/or tertiary care center for consultation may be advisable. CA 19-9, carbohydrate antigen 19-9; MRCP, magnetic resonance cholangiopancreatography.
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fig3: Proposed algorithm for UDCA use in clinical practice. Modified from ref. 96. *Surveillance and management options reviewed on cancer surveillance section. **Referral to cholestatic liver disease specialist and/or tertiary care center for consultation may be advisable. CA 19-9, carbohydrate antigen 19-9; MRCP, magnetic resonance cholangiopancreatography.

Mentions: Adding to the UDCA controversy, a recent study evaluating the effect of UDCA withdrawal showed that discontinuation of UDCA in patients with PSC caused significant deterioration in liver biochemistry.94 The accompanying editorial by Tabibian and Lindor95 suggested that perhaps until a safe and more effective treatment becomes available there may be a role for judicious use of UDCA in patients with well-compensated disease. The authors propose an individualized stepwise approach, exemplified in Figure 3.


Current Concepts in Primary Biliary Cirrhosis and Primary Sclerosing Cholangitis.

Sclair SN, Little E, Levy C - Clin Transl Gastroenterol (2015)

Proposed algorithm for UDCA use in clinical practice. Modified from ref. 96. *Surveillance and management options reviewed on cancer surveillance section. **Referral to cholestatic liver disease specialist and/or tertiary care center for consultation may be advisable. CA 19-9, carbohydrate antigen 19-9; MRCP, magnetic resonance cholangiopancreatography.
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4816277&req=5

fig3: Proposed algorithm for UDCA use in clinical practice. Modified from ref. 96. *Surveillance and management options reviewed on cancer surveillance section. **Referral to cholestatic liver disease specialist and/or tertiary care center for consultation may be advisable. CA 19-9, carbohydrate antigen 19-9; MRCP, magnetic resonance cholangiopancreatography.
Mentions: Adding to the UDCA controversy, a recent study evaluating the effect of UDCA withdrawal showed that discontinuation of UDCA in patients with PSC caused significant deterioration in liver biochemistry.94 The accompanying editorial by Tabibian and Lindor95 suggested that perhaps until a safe and more effective treatment becomes available there may be a role for judicious use of UDCA in patients with well-compensated disease. The authors propose an individualized stepwise approach, exemplified in Figure 3.

Bottom Line: Ursodeoxycholic acid (UDCA) is the recommended treatment for PBC, and recent studies with obeticholic acid showed promising results for UDCA non-responders.Unlike PBC, no therapy has been shown to alter the natural history of PSC.In addition to the lack of therapy, PSC is a pre-malignant condition and close surveillance is indicated.

View Article: PubMed Central - PubMed

Affiliation: Schiff Center for Liver Diseases, University of Miami Miller School of Medicine, Miami, Florida, USA.

ABSTRACT
Primary biliary cirrhosis (PBC) and primary sclerosing cholangitis (PSC) are chronic, cholestatic diseases of the liver with common clinical manifestations. Early diagnosis and treatment of PBC slows progression and decreases the need for transplant. However, one-third of patients will progress regardless of treatment. Bilirubin <1.0 and alkaline phosphatase <2.0 x the upper limit of normal at 1 year after treatment appear to predict 10-year survival. Ursodeoxycholic acid (UDCA) is the recommended treatment for PBC, and recent studies with obeticholic acid showed promising results for UDCA non-responders. Unlike PBC, no therapy has been shown to alter the natural history of PSC. The recommended initial diagnostic test for PSC is magnetic resonance cholangiopancreatography, typically showing bile duct wall thickening, focal bile duct dilatation, and saccular dilatation of the intra- and/or extrahepatic bile ducts. Immunoglobulin 4-associated cholangitis must be excluded when considering the diagnosis of PSC, to allow for proper treatment, and monitoring of disease progression. In addition to the lack of therapy, PSC is a pre-malignant condition and close surveillance is indicated.

No MeSH data available.


Related in: MedlinePlus