Journal
HEPATOLOGY RESEARCH
Volume 38, Issue 8, Pages 745-761Publisher
WILEY
DOI: 10.1111/j.1872-034X.2008.00366.x
Keywords
autoimmune hepatitis; autoimmune liver disease; IgG4-associated sclerosing cholangitis; primary biliary cirrhosis; primary sclerosing cholangitis; secondary sclerosing cholangitis
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Autoinflammatory liver disease represents an important aspect of global hepatological practice. The three principal disease divisions recognized are autoimmune hepatitis, primary sclerosing cholangitis and primary biliary cirrhosis. Largely, but not exclusively, these diseases are considered to be autoimmune in origin. Increased recognition of outlier and overlap syndromes, changes in presentation and natural history, as well as the increased awareness of IgG4-associated sclerosing cholangitis, all highlight the limitations of the classic terminology. New insights continue to improve the care given to patients, and have arisen from carefully conducted clinical studies, therapeutic trials, as well as genetic and laboratory investigations. The challenges remain to treat patients before liver injury becomes permanent and to prevent the development of organ failure.
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