Immunoglobulin G4-related cholangiopathy: clinical and experimental insights

Current Opinion in Gastroenterology
Lucas Maillette de Buy Wenniger, Ulrich Beuers

Abstract

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. 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. Currently available experimental evidence classifies IgG4-RD as an immun...Continue Reading

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Citations

Oct 4, 2015·Gastrointestinal Endoscopy Clinics of North America·Jacques Devière
Jan 20, 2018·Langenbeck's Archives of Surgery·F RassamT M van Gulik

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Methods Mentioned

BETA
biopsy
biopsies
flow cytometry

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