期刊
SEMINARS IN DIAGNOSTIC PATHOLOGY
卷 29, 期 4, 页码 205-211出版社
W B SAUNDERS CO-ELSEVIER INC
DOI: 10.1053/j.semdp.2012.07.005
关键词
Sclerosing cholangitis; Autoimmune pancreatitis; IgG4; Follicular cholangitis; Granulocytic epithelial lesion
Since immunoglobulin 04 (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. (C) 2012 Elsevier Inc. All rights reserved.
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