IgG4 HPB Disease

  • Jimil Shah
  • Usha Dutta
Part of the GI Surgery Annual book series (GISA, volume 25)


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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Copyright information

© Indian Association of Surgical Gastroenterology 2019

Authors and Affiliations

  • Jimil Shah
    • 1
  • Usha Dutta
    • 1
  1. 1.Department of GastroenterologyP.G.I.M.E.RChandigarhIndia

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