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Semin Liver Dis 2016; 36(03): 242-256
DOI: 10.1055/s-0036-1584319
Review Article
Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.

The Pathology of IgG4-Related Disease in the Bile Duct and Pancreas

Yoh Zen
1   Department of Diagnostic Pathology, Kobe University Graduate School of Medicine, Kobe, Japan
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Publication History

Publication Date:
28 July 2016 (online)

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Abstract

Immunoglobulin G4-related disease (IgG4-RD) in the pancreatobiliary system manifests as sclerosing cholangitis (SC), hepatic inflammatory pseudotumors, and type 1 autoimmune pancreatitis (AIP). The pathology of IgG4-RD involves an inflammatory process and fibrogenic pathway, the combination of which damages the affected organs. Fibroinflammatory injury is characterized by three microscopic findings: a diffuse lymphoplasmacytic infiltrate rich in IgG4-positive plasma cells, obliterative phlebitis, and storiform fibrosis. Although the diagnosis of IgG4-related pancreatocholangitis is relatively straightforward in surgical specimens, the current clinical requirement is to diagnose patients using biopsy samples, which remains challenging. Histological differential diagnoses include primary SC, follicular cholangitis/pancreatitis, SC with granulocytic epithelial lesions, and type 2 AIP. Although the massive infiltration of IgG4-positive plasma cells is a histological hallmark of IgG4-RD, many other immune cells (e.g., Th2 lymphocytes, regulatory T cells, and M2 macrophages) appear to be strongly involved in orchestral immune reactions.

Keywords

immunoglobulin G4 - sclerosing cholangitis - autoimmune pancreatitis
 

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