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How to Diagnose Immunoglobulin G4-Associated Cholangitis: The Jack-of-All-Trades in the Biliary Tract

BACKGROUND: Immunoglobulin (Ig) G4-associated cholangitis (IAC) is an inflammatory disorder of the biliary tract displaying characteristic features of IgG4-related disease (IgG4-RD): elevation of IgG4 serum levels, infiltration of IgG4+ plasma cells in the affected tissue, and good response to immun...

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Detalles Bibliográficos
Autores principales: Hubers, Lowiek M., Beuers, Ulrich
Formato: Online Artículo Texto
Lenguaje:English
Publicado: S. Karger Verlag für Medizin und Naturwissenschaften GmbH 2015
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4569201/
https://www.ncbi.nlm.nih.gov/pubmed/26468313
http://dx.doi.org/10.1159/000431028
Descripción
Sumario:BACKGROUND: Immunoglobulin (Ig) G4-associated cholangitis (IAC) is an inflammatory disorder of the biliary tract displaying characteristic features of IgG4-related disease (IgG4-RD): elevation of IgG4 serum levels, infiltration of IgG4+ plasma cells in the affected tissue, and good response to immunosuppressive treatment. METHODS AND RESULTS: The clinical presentation of IAC is often misleading, mimicking other diseases of the biliary tract such as cholangiocarcinoma or primary and secondary sclerosing cholangitis. The HISORt criteria form the cornerstone in the diagnosis of IAC, combining histopathological (H), imaging (I), and serological (S) features including serum IgG4, other organ manifestations (O) of IgG4-RD and response to treatment (Rt). The accuracy of the HISORt criteria is limited. Novel diagnostic approaches are under evaluation. CONCLUSION: More accurate biomarkers are needed to correctly diagnose IgG4-RD and prevent misdiagnoses and unnecessary therapeutic interventions.