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IgG4-Associated Cholangitis: A Comprehensive Review

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Abstract

IgG4-associated cholangitis (IAC) is a major manifestation of immunoglobulin G4-related disease (IgG4-RD), an inflammatory multiorgan disorder of unknown cause. IAC and autoimmune pancreatitis (AIP) may mimic sclerosing cholangitis, cholangiocarcinoma, or pancreatic carcinoma. Typically, elderly male patients present with abdominal discomfort, weight loss, jaundice, and itch. At present, no accurate diagnostic test for IAC and IgG4-RD is at hand, often causing significant diagnostic delay. Serum IgG4 is only diagnostic when markedly raised (>4× ULN). Imaging in IAC discloses mass-forming lesions and/or strictures in the biliary tract. Histology may show tissue infiltration of IgG4-expressing plasma cells. Diagnostic criteria for histologic and imaging findings, serum tests, organ manifestation pattern, and response to immunosuppressive therapy (HISORt) criteria are used for the diagnosis of IgG4-RD. Still, considering the difficulty in diagnosing IAC and AIP, unnecessary hepatic or pancreatic resections for presumed malignancies occur. The good response to corticosteroid therapy in IAC and other manifestations of IgG4-RD suggests an immune-mediated inflammatory disease. Maintenance immunosuppression after induction of remission is needed in the majority of patients to avoid relapse. The pathogenesis of IAC and IgG4-RD remains poorly understood. Unresolved questions include: (i) Does IgG4 have a pro- or anti-inflammatory role in IAC? (ii) Is IAC a B cell- and/or T cell-mediated disease? (iii) Which are the molecular targets attacked by the immune system in IgG4-RD? Here, we review the diagnostic and therapeutic management of the disease and discuss recent pathophysiological findings, which might help to better understand the molecular mechanisms contributing to IAC and other manifestations of IgG4-RD.

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Conflict of Interest

Lowiek M. Hubers, Lucas J. Maillette de Buy Wenniger, Marieke E. Doorenspleet, Paul L. Klarenbeek, Joanne Verheij, Erik A. Rauws, Thomas M. van Gulik, Ronald P.J. Oude Elferink, Stan F.J. van de Graaf, Niek de Vries, and Ulrich Beuers declare that they have no conflict of interest.

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  1. Department of Gastroenterology and Hepatology and Tytgat Institute of Liver and Intestinal Research, Academic Medical Center, University of Amsterdam, Meibergdreef 9, room G4-216, 1105 AZ, Amsterdam, The Netherlands

    Lowiek M. Hubers, Lucas J. Maillette de Buy Wenniger, Erik A. Rauws, Ronald P. J. Oude Elferink, Stan F. J. van de Graaf & Ulrich Beuers

  2. Department of Clinical Immunology and Rheumatology and Laboratory of Experimental Immunology, Academic Medical Center, University of Amsterdam, Amsterdam, The Netherlands

    Marieke E. Doorenspleet, Paul L. Klarenbeek & Niek de Vries

  3. Department of Pathology, Academic Medical Center, University of Amsterdam, Amsterdam, The Netherlands

    Joanne Verheij

  4. Department of Surgery, Academic Medical Center, University of Amsterdam, Amsterdam, The Netherlands

    Thomas M. van Gulik

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  1. Lowiek M. Hubers
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Hubers, L.M., Maillette de Buy Wenniger, L.J., Doorenspleet, M.E. et al. IgG4-Associated Cholangitis: A Comprehensive Review. Clinic Rev Allerg Immunol 48, 198–206 (2015). https://doi.org/10.1007/s12016-014-8430-2

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