Elsevier

Journal of Hepatology

Volume 61, Issue 3, September 2014, Pages 690-695
Journal of Hepatology

Clinical Application of Basic Science
IgG4 cholangiopathy – Current concept, diagnosis, and pathogenesis

https://doi.org/10.1016/j.jhep.2014.04.016Get rights and content
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Summary

IgG4 related cholangiopathy, a distinctive type of cholangitis of unknown origin, is characterized by increased serum levels of IgG4, massive infiltration of IgG4-positive plasma cells with storiform fibrosis and/or obliterative phlebitis in the thickened bile duct wall, and good response to steroids. Patients with IgG4-cholangiopathy are frequently associated with autoimmune pancreatitis; IgG4-cholangiopathy is recognized as a biliary manifestation of IgG4-related disease. This condition can be diagnosed by a combination of imaging, serology, histopathology, and steroid responsiveness; however, cholangiographic features are often difficult to differentiate from primary sclerosing cholangitis, pancreatic cancer, or cholangiocarcinoma. The Japanese clinical diagnostic criteria for IgG4-related sclerosing cholangitis established in 2012 are useful in the diagnosis of IgG4-cholangiopathy. Although the precise pathogenic mechanism remains unclear, the development of IgG4-cholangiopathy may involve: susceptible genetic factors, abnormal innate and acquired immunity, decreased naïve regulatory T cells, and specific B cell responses.

Further studies on genetic backgrounds, disease specific antigens, and the role of IgG4 are necessary to clarify the pathogenesis.

Abbreviations

AIP
autoimmune pancreatitis
ANA
anti-nuclear antibody
CA-II
carbonic anhydrase-II
CBD
common bile duct
CTLA-4
cytotoxic T lymphocyte antigen-4
ERCP
endoscopic retrograde cholangio-pancreatography
FCRL
Fc-receptor-like
IFN-γ
interferon-γ
IgG4-RD
IgG4-related disease
IgG4-SC
IgG4-related sclerosing cholangitis
IL-4
interleukin-4
LF
lactoferrin
LPSP
lymphoplasmacytic sclerosing pancreatitis
PSC
primary sclerosing cholangitis

Keywords

IgG4-related disease
IgG4-cholangiopathy
IgG4-related sclerosing cholangitis
Autoimmune pancreatitis

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