IgG4-related hepatobiliary disease: an overview.
IgG4-related hepatobiliary diseases are part of a multiorgan fibroinflammatory condition termed IgG4-related disease, and include IgG4-related sclerosing cholangitis (IgG4-SC) and IgG4-related hepatopathy. These diseases can present with biliary strictures and/or mass lesions, making them difficult to differentiate from primary sclerosing cholangitis (PSC) or other hepatobiliary malignancies. Diagnosis is based on a combination of clinical, biochemical, radiological and histological findings. However, a gold standard diagnostic test is lacking, warranting the identification of more specific disease markers. Novel assays - such as the serum IgG4:IgG1 ratio and IgG4:IgG RNA ratio (which distinguish IgG4-SC from PSC with high serum IgG4 levels), and plasmablast expansion to recognize IgG4-SC with normal serum IgG4 levels - require further validation. Steroids and other immunosuppressive therapies can lead to clinical and radiological improvement when given in the inflammatory phase of the disease, but evidence for the efficacy of treatment regimens is limited. Progressive fibrosclerotic disease, liver cirrhosis and an increased risk of malignancy are now recognized outcomes. Insights into the genetic and immunological features of the disease have increased over the past decade, with an emphasis on HLAs, T cells, circulating memory B cells and plasmablasts, chemokine-mediated trafficking, as well as the role of the innate immune system.
Translational Gastroenterology Unit, John Radcliffe Hospital, Headley Way, Headington, Oxford, OX3 9DU, UK.|Nuffield Department of Medicine, Oxford University, Old Road Campus, Headington, Oxford, OX3 7BN, UK.|Liver Transplant Unit, Royal Free Hospital, Pond Street, London, NW3 2QG, UK.
Emma L
EL
Culver
Translational Gastroenterology Unit, John Radcliffe Hospital, Headley Way, Headington, Oxford, OX3 9DU, UK.|Nuffield Department of Medicine, Oxford University, Old Road Campus, Headington, Oxford, OX3 7BN, UK.
Roger W
RW
Chapman
D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D001327:Autoimmune Diseases / Q000175:diagnosis / Q000188:drug therapy; D001402:B-Lymphocytes / Q000276:immunology; D001660:Biliary Tract Diseases / Q000175:diagnosis* / Q000188:drug therapy / Q000276:immunology; D015496:CD4-Positive T-Lymphocytes / Q000276:immunology; D002760:Cholangiopancreatography, Endoscopic Retrograde; D015209:Cholangitis, Sclerosing / Q000175:diagnosis* / Q000188:drug therapy; D003937:Diagnosis, Differential; D005544:Forecasting; D019693:Hepatitis, Autoimmune / Q000175:diagnosis* / Q000188:drug therapy / Q000276:immunology; D006801:Humans; D007113:Immunity, Innate; D007156:Immunologic Memory / Q000276:immunology; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D010195:Pancreatitis / Q000175:diagnosis / Q000188:drug therapy / Q000276:immunology; D010265:Paraproteinemias / Q000175:diagnosis* / Q000188:drug therapy / Q000276:immunology; D012008:Recurrence; D012307:Risk Factors; D009626:Terminology as Topic; D016896:Treatment Outcome
D000305:Adrenal Cortex Hormones; D007166:Immunosuppressive Agents