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Semin Diagn Pathol. 2012 Nov;29(4):205-11. doi: 10.1053/j.semdp.2012.07.005.

Immunoglobulin G4-related sclerosing cholangitis: pathologic features and histologic mimics.

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  • 1Histopathology Section, Institute of Liver Studies, King's College Hospital, Denmark Hill, London, UK. yoh.zen@kcl.ac.uk

Abstract

Since immunoglobulin G4 (IgG4)-related sclerosing cholangitis was first described in 2004, this condition has been recognized as a distinct entity characterized by unique pathologic features. Affected bile ducts show diffuse wall thickening, which represents transmural sclerosing inflammation composed of a dense lymphoplasmacytic infiltrate and fibrosis arranged at least focally in a storiform pattern. A diffuse IgG4-positive plasma cell infiltrate is demonstrable by immunohistochemistry. More than 90% of patients with IgG4-related sclerosing cholangitis have type 1 autoimmune pancreatitis, the remaining presenting as isolated cholangitis. The diagnosis requires a multidisciplinary approach, in which pathologic examination has a critical role. Tissue diagnosis on biopsy specimens (ie, bile duct biopsy, liver needle biopsies) is important, particularly for patients with isolated cholangitis, given the fact that radiological findings of IgG4-cholangitis are not reliable enough for establishing the diagnosis. Furthermore, serum IgG4 levels can be, albeit mildly, elevated in other biliary disorders. Histologic mimics of IgG4-related sclerosing cholangitis include primary sclerosing cholangitis, follicular cholangitis, and sclerosing cholangitis with granulocytic epithelial lesion, the latter 2 being recently described entities.

Copyright © 2012 Elsevier Inc. All rights reserved.

[PubMed - indexed for MEDLINE]
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