Liver and intrahepatic bile ducts-nontumor
Biliary tract disease
Reviewers: Komal Arora, M.D. (see Reviewers page)
Revised: 19 May 2012, last major update May 2012
Copyright: (c) 2004-2012, PathologyOutlines.com, Inc.
● First described in 1994 (Am J Surg Pathol 1994;18:91)
● May be variant of antimitochondrial negative primary biliary cirrhosis (Dig Dis Sci 1995;40:1232, Clin Liver Dis 2004;8:177)
● May be due to IgG4 systemic disease (Clin Liver Dis 2004;8:177, J Med Case Rep 2011 Sep 28;5:485)
● More common in women
● Presents with pruritis, associated with arthralgias, sicca syndrome, Raynaud's phenomenon
● Laboratory: positive antinuclear antibody, negative antimitochondrial antibody
● Ductopenia with bile duct damage similar to primary biliary cirrhosis, mild chronic active hepatitis-like portal changes, bile ductular proliferation
● Variable granulomas
Florid duct lesion with granulomatous destruction of bile duct typical of primary biliary cirrhosis
IgG4 related case
End of Liver and intrahepatic bile ducts-nontumor > Biliary tract disease > Autoimmune cholangitis
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