It is well known that some pathological conditions, especially of autoimmune etiology, are associated with the HLA (human leukocyte antigen) phenotype. Among these diseases, we include celiac disease, inflammatory bowel disease, autoimmune enteropathy, autoimmune hepatitis, primary sclerosing cholangitis and primary biliary cholangitis. Immunoglobulin G4-related diseases (IgG4-related diseases) constitute a second group of autoimmune gastrointestinal, hepatobiliary and pancreatic illnesses. IgG4-related diseases are systemic and rare autoimmune illnesses. They often are connected with chronic inflammation and fibrotic reaction that can occur in any organ of the body. The most typical feature of these diseases is a mononuclear infiltrate with IgG4-positive plasma cells and self-sustaining inflammatory response. In this review, we focus especially upon the hepatopancreatobiliary system, autoimmune pancreatitis and IgG4-related sclerosing cholangitis. The cooperation of the gastroenterologist, radiologist, surgeon and histopathologist is crucial for establishing correct diagnoses and appropriate treatment, especially in IgG4 hepatopancreatobiliary diseases.

Central European Institute of Technology Masaryk University 625 00 Brno Czech Republic

Department of Gastroenterology and Digestive Endoscopy Masaryk Memorial Cancer Institute 602 00 Brno Czech Republic

Department of Gastroenterology and Internal Medicine University Hospital Brno 625 00 Brno Czech Republic

Department of Gastroenterology and Internal Medicine University Hospital Ostrava 728 00 Ostrava Czech Republic

Department of Pediatrics University Hospital Brno 613 00 Brno Czech Republic

Department of Radiology and Nuclear Medicine University Hospital Brno 625 00 Brno Czech Republic

Department of Surgery University Hospital Brno 625 00 Brno Czech Republic

Faculty of Medicine Masaryk University 625 00 Brno Czech Republic

Faculty of Medicine University of Ostrava 703 00 Ostrava Czech Republic

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