Recent Advances in the Concept and Pathogenesis of IgG4-Related Disease in the Hepato-Bilio-Pancreatic System
Gut and Liver
;
: 462-470, 2014.
Article
in English
| WPRIM
| ID: wpr-108136
ABSTRACT
Recent studies have proposed nomenclatures of type 1 autoimmune pancreatitis (AIP) (IgG4-related pancreatitis), IgG4-related sclerosing cholangitis (IgG4-SC), IgG4-related cholecystitis, and IgG4-related hepatopathy as IgG4-related disease (IgG4-RD) in the hepato-bilio-pancreatic system. In IgG4-related hepatopathy, a novel concept of IgG4-related autoimmune hepatitis (AIH) with the same histopathological features as AIH has been proposed. Among organs involved in IgG4-RD, associations with pancreatic and biliary lesions are most frequently observed, supporting the novel concept of "biliary diseases with pancreatic counterparts." Targets of type 1 AIP and IgG4-SC may be periductal glands around the bile and pancreatic ducts. Based on genetic backgrounds, innate and acquired immunity, Th2-dominant immune status, regulatory T (Treg) or B cells, and complement activation via a classical pathway may be involved in the development of IgG4-RD. Although the role of IgG4 remains unclear in IgG4-RD, IgG4-production is upregulated by interleukin 10 from Treg cells and by B cell activating factor from monocytes/basophils with stimulation of toll-like receptors/nucleotide-binding oligomerization domain-like receptors. Based on these findings, we have proposed a hypothesis for the development of IgG4-RD in the hepato-bilio-pancreatic system. Further studies are necessary to clarify the pathogenic mechanism of IgG4-RD.
Full text:
Available
Index:
WPRIM (Western Pacific)
Main subject:
Pancreatitis
/
Autoimmune Diseases
/
Immunoglobulin G
/
Cholangitis, Sclerosing
/
Cholecystitis
/
Interleukin-10
/
T-Lymphocytes, Regulatory
/
B-Cell Activating Factor
/
Adaptive Immunity
/
Liver Diseases
Type of study:
Etiology study
Limits:
Humans
Language:
English
Journal:
Gut and Liver
Year:
2014
Type:
Article
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