Autoimmune Liver Diseases: Primary Sclerosing Cholangitis

2016 ◽  
pp. 289-305
Author(s):  
José Franco
Keyword(s):  
Primary Sclerosing Cholangitis ◽  
Liver Diseases ◽  
Sclerosing Cholangitis ◽  
Autoimmune Liver Diseases

scholarly journals Urisodeoxycholic Acid in Treatment of Liver Cirrhosis

2021 ◽  
Vol 9 (9) ◽  
pp. 1869-1873
Author(s):  
Sangale Mukta
Keyword(s):  
Liver Cirrhosis ◽  
Bile Acid ◽  
Primary Biliary Cirrhosis ◽  
Ursodeoxycholic Acid ◽  
Primary Sclerosing Cholangitis ◽  
Liver Diseases ◽  
Sclerosing Cholangitis ◽  
Chronic Liver ◽  
Biliary Cirrhosis ◽  
Chronic Liver Diseases

Abstract: Ursodeoxycholic acid is a dihy- droxy bile acid with a rapidly expanding spectrum of usage in acute and chronic liver diseases. The various mechanisms of action of this hydrophilic bile acid include direct cytoprotection, detergent action on dysfunctional microtubules, immunomodulation and induction of hypercholer- esis. Its efficacy in primary biliary cirrhosis and primary sclerosing cholangitis as an adjunct to medical therapy has been well established.Ursodeoxycholic acid prolongs survival in primary biliary cirrhosis and it improves biochemical parameters of cholestasis in various other cholestatic disorders including primary sclerosing cholangitis, intrahepatic cholestasis of pregnancy, cystic fibrosis and total parenteral nutritioninduced cholestasis. However, a positive effect on survival remains to be established in these diseases. Ursodeoxycholic acid is of unproven efficacy in non- cholestatic disorders such as acute rejection after liver transplantation, non-alcoholic steatohepatitis, alcoholic liver disease and chronic viral hepatitis. This review outlines the present knowledge of the Pharmacology of ursodeoxycholic acid, and presents data from clinical trials on its use in chronic liver diseases. Keywords: Liver cirrhosis, Urisodeoxycholic acid

scholarly journals Expression Analysis of ATP-Binding Cassette Transporters ABCB11 and ABCB4 in Primary Sclerosing Cholangitis and Variety of Pediatric and Adult Cholestatic and Noncholestatic Liver Diseases

2019 ◽  
Vol 2019 ◽  
pp. 1-10
Author(s):  
Cornelia Thoeni ◽  
Ruediger Waldherr ◽  
Jutta Scheuerer ◽  
Stefanie Schmitteckert ◽  
Ralph Roeth ◽  
...  
Keyword(s):  
Disease Progression ◽  
Primary Sclerosing Cholangitis ◽  
Liver Diseases ◽  
Sclerosing Cholangitis ◽  
Efflux Pump ◽  
Atp Binding ◽  
Cholestatic Liver Disease ◽  
Altered Expression ◽  
Outer Leaflet ◽  
Atp Binding Cassette

ATP-binding cassette (ABC) transporters are the members of the efflux pumps that are responsible for the removal of cytotoxic substances by active transport. ABCB11, the bile salt efflux pump of hepatocytes, coordinates cellular excretion of numerous conjugated bile salts into the bile canaliculi, whereas ABCB4 acts as an ATP-dependent floppase translocating phosphatidylcholine from the inner to the outer leaflet of the bile canalicular membrane. Loss of functional ABCB11 and ABCB4 proteins causes early-onset refractory cholestasis or cholangiopathy. In this study, we investigated the expression and localization pattern of ABCB11 and ABCB4 using immunohistochemistry and RNA profiling in liver samples from patients with different types and stages of chronic cholestatic liver disease, with emphasis on primary sclerosing cholangitis (PSC), compared to a variety of cholestatic and noncholestatic hepatopathies. Therefore, ABCB11 and ABCB4 expressions were investigated on formalin-fixed and paraffin-embedded (FFPE) material in a patient cohort of total 43 patients with or without cholestatic liver diseases, on protein level using immunohistochemistry and on RNA level using nanoString technology. Intriguingly, our results demonstrated increased expression of ABCB11 and ABCB4 on protein as well as RNA level in PSC, and the expression pattern correlated with disease progression. We concluded from our study that patients with PSC demonstrate altered expression levels and pattern of ABCB11 and ABCB4 which correlated with disease progression; thereby, ABCB11 and ABCB4 analysis may be a useful tool for assessment of disease stages in PSC.

Autoimmunity in liver disease

2019 ◽  
pp. 245-252
Author(s):  
Gavin Spickett
Keyword(s):  
Liver Disease ◽  
Primary Biliary Cirrhosis ◽  
Autoimmune Hepatitis ◽  
Primary Sclerosing Cholangitis ◽  
Liver Diseases ◽  
Sclerosing Cholangitis ◽  
Biliary Cirrhosis

This chapter covers the presentation, immunogenetics, immunopathology, diagnosis, treatment, and testing for a range of liver diseases. Primary biliary cirrhosis, autoimmune hepatitis, and primary sclerosing cholangitis are described.

scholarly journals Analysis of IL2/IL21 Gene Variants in Cholestatic Liver Diseases Reveals an Association with Primary Sclerosing Cholangitis

Digestion ◽  
2011 ◽  
Vol 84 (1) ◽  
pp. 29-35 ◽  
Author(s):  
Johannes Stallhofer ◽  
Gerald Ulrich Denk ◽  
Jürgen Glas ◽  
Rüdiger Paul Laubender ◽  
Burkhard Göke ◽  
...  
Keyword(s):  
Primary Sclerosing Cholangitis ◽  
Liver Diseases ◽  
Sclerosing Cholangitis ◽  
Gene Variants ◽  
I Gene
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