Immunoglobulin G4-related cholangiopathy: clinical and experimental insights.
OBJECTIVE
The clinical spectrum of immunoglobulin G4-related disease (IgG4-RD) is diverse and the disease may affect multiple organ systems. The pancreatobiliary tract probably is the clinically most important localization of the disease but diagnosing IgG4-related cholangiopathy (synonym: IgG4-associated cholangitis) or autoimmune pancreatitis is often challenging. This review summarizes the current best practice in diagnosing and treating IgG4-related cholangiopathy and recent advances in our understanding of its cause.


RESULTS
The identification of IgG4-switched B-cell and plasma cell populations in patients with IgG4-related cholangiopathy and IgG4-RD and their disappearance upon successful treatment have established the role of these cells in the disorder. Ultimately, these findings may lead to the development of more sensitive diagnostic tests. The observation that many of the predominantly 50-70 years old male patients have been exposed lengthily to occupational hazardous compounds further supports the idea that chronic antigenic stimulation may be a pivotal etiological aspect of the disease. Immunosuppressive treatment remains the therapeutic cornerstone in IgG4-RD.


CONCLUSIONS
Currently available experimental evidence classifies IgG4-RD as an immune-mediated disorder, providing support to the use of immunosuppressants and possibly even more specific therapies targeting the B-cell and plasma cell lineages.
aDepartment of Gastroenterology and Hepatology and Tytgat Institute for Liver and Intestinal Research, Academic Medical Center, University of Amsterdam bDepartment of Ophthalmology, Vrije Universiteit Medical Center, Amsterdam, The Netherlands.
Lucas J
LJ
Maillette de Buy Wenniger
Ulrich
U
Beuers
D056704:Adaptive Immunity; D001327:Autoimmune Diseases / Q000175:diagnosis* / Q000276:immunology / Q000473:pathology; D002761:Cholangitis / Q000175:diagnosis* / Q000276:immunology / Q000473:pathology; D002908:Chronic Disease; D003937:Diagnosis, Differential; D006801:Humans; D007113:Immunity, Innate; D007074:Immunoglobulin G / Q000009:adverse effects / Q000378:metabolism*; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D008099:Liver / Q000473:pathology*; D008297:Male; D008875:Middle Aged; D016273:Occupational Exposure / Q000009:adverse effects*; D010950:Plasma Cells / Q000276:immunology; D011379:Prognosis
D007074:Immunoglobulin G; D007166:Immunosuppressive Agents