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IgG4 HPB Disease

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GI Surgery Annual

Part of the book series: GI Surgery Annual ((GISA,volume 25))

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Abstract

IgG4-related hepato-pancreato-biliary (IgG4 HPB) disease is a part of a multisystem disease called IgG4-related diseases (IgG4 RD). It may involve the liver, pancreas, or biliary tree in various combinations and termed as IgG4 hepatopathy, autoimmune pancreatitis type-1 (AIP-1), or IgG4 cholangiopathy, respectively. It is characterized by multi-organ fibroinflammatory condition with tumefactive lesions, dense lymphoplasmacytic infiltration, IgG4-rich plasma cells, storiform fibrosis, and elevated serum IgG4 levels. It can present with mass lesion, stricture, or features of end-organ damage. It mimics various malignancies, infectious etiologies, and vasculitis posing difficulties in diagnosis. Though the disease lacks any gold standard test for diagnosis, serum IgG4 levels, IgG4/IgG RNA ratio, and increased circulating plasmablast are helpful in diagnosing this disease. Steroid remains the main stay of therapy; however, some patients may require immunosuppressive treatment.

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Authors and Affiliations

  1. Department of Gastroenterology, P.G.I.M.E.R, Chandigarh, India

    Jimil Shah & Usha Dutta

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  1. Jimil Shah
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  2. Usha Dutta
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  1. Department of GI Surgery and Liver Transplantation, All India Institute of Medical Sciences, New Delhi, India

    Peush Sahni

  2. Department of GI Surgery and Liver Transplantation, All India Institute of Medical Sciences, New Delhi, India

    Sujoy Pal

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Shah, J., Dutta, U. (2019). IgG4 HPB Disease. In: Sahni, P., Pal, S. (eds) GI Surgery Annual. GI Surgery Annual, vol 25. Springer, Singapore. https://doi.org/10.1007/978-981-13-3227-2_6

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  • DOI: https://doi.org/10.1007/978-981-13-3227-2_6

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