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Autoimmune (IgG4) Cholangitis
Siva P. Raman, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Pathology

        • Clinical Issues

          TERMINOLOGY

          • Synonyms

            • IgG4-related sclerosing disease (IRSD), IgG4-related sclerosing cholangitis (ISC)
          • Definitions

            • IRSD: Spectrum of disorders characterized by tumor-like lymphoplasmacytic infiltration and varying degrees of fibrosis in different organs
              • Pancreas is most commonly affected organ [autoimmune pancreatitis (AIP)] followed by salivary glands and biliary tree
            • ISC: Biliary manifestations of IRSD frequently associated with AIP
              • Manifested as wall thickening and biliary strictures

          IMAGING

          • General Features

            • Radiographic Findings

              • CT Findings

                • MR Findings

                  • Ultrasonographic Findings

                    • Imaging Recommendations

                      DIFFERENTIAL DIAGNOSIS

                        PATHOLOGY

                        • General Features

                          • Staging, Grading, & Classification

                            • Gross Pathologic & Surgical Features

                              • Microscopic Features

                                CLINICAL ISSUES

                                • Presentation

                                  • Demographics

                                    • Natural History & Prognosis

                                      • Treatment

                                        DIAGNOSTIC CHECKLIST

                                        • Image Interpretation Pearls

                                          Selected References

                                          1. Ollo D et al: Biliary involvement in type 2 autoimmune pancreatitis. Case Rep Gastroenterol. 13(1):200-6, 2019
                                          2. Swensson J et al: Differentiating IgG4-related sclerosing cholangiopathy from cholangiocarcinoma using CT and MRI: experience from a tertiary referring center. Abdom Radiol (NY). 44(6):2111-5, 2019
                                          3. Geary K et al: IgG4-related cholangiopathy and its mimickers: a case report and review highlighting the importance of early diagnosis. Int J Surg Pathol. 26(2):165-73, 2018
                                          4. Goodchild G et al: Immunoglobulin G4-related sclerosing cholangitis. Korean J Intern Med. 33(5):841-50, 2018
                                          5. Zen Y et al: IgG4-related sclerosing cholangitis: all we need to know. J Gastroenterol. 51(4):295-312, 2016
                                          6. Gardner CS et al: Diagnostic performance of imaging criteria for distinguishing autoimmune cholangiopathy from primary sclerosing cholangitis and bile duct malignancy. Abdom Imaging. 40(8):3052-61, 2015
                                          7. Joshi D et al: Review article: biliary and hepatic involvement in IgG4-related disease. Aliment Pharmacol Ther. 40(11-12):1251-61, 2014
                                          8. Zen Y et al: IgG4 Cholangiopathy. Int J Hepatol. 2012:472376, 2012
                                          9. Nakazawa T et al: Diagnostic procedures for IgG4-related sclerosing cholangitis. J Hepatobiliary Pancreat Sci. 18(2):127-36, 2011
                                          10. Okazaki K et al: Recent advances in the concept and diagnosis of autoimmune pancreatitis and IgG4-related disease. J Gastroenterol. 46(3):277-88, 2011
                                          11. Vlachou PA et al: IgG4-related sclerosing disease: autoimmune pancreatitis and extrapancreatic manifestations. Radiographics. 31(5):1379-402, 2011
                                          12. Naitoh I et al: Clinical significance of extrapancreatic lesions in autoimmune pancreatitis. Pancreas. 39(1):e1-5, 2010
                                          13. Alderlieste YA et al: Immunoglobulin G4-associated cholangitis: one variant of immunoglobulin G4-related systemic disease. Digestion. 79(4):220-8, 2009
                                          14. Itoh S et al: Lymphoplasmacytic sclerosing cholangitis: assessment of clinical, CT, and pathological findings. Clin Radiol. 64(11):1104-14, 2009
                                          15. Webster GJ et al: Autoimmune pancreatitis/IgG4-associated cholangitis and primary sclerosing cholangitis--overlapping or separate diseases? J Hepatol. 51(2):398-402, 2009
                                          16. Ghazale A et al: Immunoglobulin G4-associated cholangitis: clinical profile and response to therapy. Gastroenterology. 134(3):706-15, 2008
                                          17. Kamisawa T et al: IgG4-related sclerosing disease. World J Gastroenterol. 14(25):3948-55, 2008
                                          18. Chari ST et al: Diagnosis of autoimmune pancreatitis: the Mayo Clinic experience. Clin Gastroenterol Hepatol. 4(8):1010-6; quiz 934, 2006
                                          19. Kamisawa T et al: Autoimmune pancreatitis: proposal of IgG4-related sclerosing disease. J Gastroenterol. 41(7):613-25, 2006
                                          Related Anatomy
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                                          Related Differential Diagnoses
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                                          References
                                          Tables

                                          Tables

                                          KEY FACTS

                                          • Terminology

                                            • Imaging

                                              • Pathology

                                                • Clinical Issues

                                                  TERMINOLOGY

                                                  • Synonyms

                                                    • IgG4-related sclerosing disease (IRSD), IgG4-related sclerosing cholangitis (ISC)
                                                  • Definitions

                                                    • IRSD: Spectrum of disorders characterized by tumor-like lymphoplasmacytic infiltration and varying degrees of fibrosis in different organs
                                                      • Pancreas is most commonly affected organ [autoimmune pancreatitis (AIP)] followed by salivary glands and biliary tree
                                                    • ISC: Biliary manifestations of IRSD frequently associated with AIP
                                                      • Manifested as wall thickening and biliary strictures

                                                  IMAGING

                                                  • General Features

                                                    • Radiographic Findings

                                                      • CT Findings

                                                        • MR Findings

                                                          • Ultrasonographic Findings

                                                            • Imaging Recommendations

                                                              DIFFERENTIAL DIAGNOSIS

                                                                PATHOLOGY

                                                                • General Features

                                                                  • Staging, Grading, & Classification

                                                                    • Gross Pathologic & Surgical Features

                                                                      • Microscopic Features

                                                                        CLINICAL ISSUES

                                                                        • Presentation

                                                                          • Demographics

                                                                            • Natural History & Prognosis

                                                                              • Treatment

                                                                                DIAGNOSTIC CHECKLIST

                                                                                • Image Interpretation Pearls

                                                                                  Selected References

                                                                                  1. Ollo D et al: Biliary involvement in type 2 autoimmune pancreatitis. Case Rep Gastroenterol. 13(1):200-6, 2019
                                                                                  2. Swensson J et al: Differentiating IgG4-related sclerosing cholangiopathy from cholangiocarcinoma using CT and MRI: experience from a tertiary referring center. Abdom Radiol (NY). 44(6):2111-5, 2019
                                                                                  3. Geary K et al: IgG4-related cholangiopathy and its mimickers: a case report and review highlighting the importance of early diagnosis. Int J Surg Pathol. 26(2):165-73, 2018
                                                                                  4. Goodchild G et al: Immunoglobulin G4-related sclerosing cholangitis. Korean J Intern Med. 33(5):841-50, 2018
                                                                                  5. Zen Y et al: IgG4-related sclerosing cholangitis: all we need to know. J Gastroenterol. 51(4):295-312, 2016
                                                                                  6. Gardner CS et al: Diagnostic performance of imaging criteria for distinguishing autoimmune cholangiopathy from primary sclerosing cholangitis and bile duct malignancy. Abdom Imaging. 40(8):3052-61, 2015
                                                                                  7. Joshi D et al: Review article: biliary and hepatic involvement in IgG4-related disease. Aliment Pharmacol Ther. 40(11-12):1251-61, 2014
                                                                                  8. Zen Y et al: IgG4 Cholangiopathy. Int J Hepatol. 2012:472376, 2012
                                                                                  9. Nakazawa T et al: Diagnostic procedures for IgG4-related sclerosing cholangitis. J Hepatobiliary Pancreat Sci. 18(2):127-36, 2011
                                                                                  10. Okazaki K et al: Recent advances in the concept and diagnosis of autoimmune pancreatitis and IgG4-related disease. J Gastroenterol. 46(3):277-88, 2011
                                                                                  11. Vlachou PA et al: IgG4-related sclerosing disease: autoimmune pancreatitis and extrapancreatic manifestations. Radiographics. 31(5):1379-402, 2011
                                                                                  12. Naitoh I et al: Clinical significance of extrapancreatic lesions in autoimmune pancreatitis. Pancreas. 39(1):e1-5, 2010
                                                                                  13. Alderlieste YA et al: Immunoglobulin G4-associated cholangitis: one variant of immunoglobulin G4-related systemic disease. Digestion. 79(4):220-8, 2009
                                                                                  14. Itoh S et al: Lymphoplasmacytic sclerosing cholangitis: assessment of clinical, CT, and pathological findings. Clin Radiol. 64(11):1104-14, 2009
                                                                                  15. Webster GJ et al: Autoimmune pancreatitis/IgG4-associated cholangitis and primary sclerosing cholangitis--overlapping or separate diseases? J Hepatol. 51(2):398-402, 2009
                                                                                  16. Ghazale A et al: Immunoglobulin G4-associated cholangitis: clinical profile and response to therapy. Gastroenterology. 134(3):706-15, 2008
                                                                                  17. Kamisawa T et al: IgG4-related sclerosing disease. World J Gastroenterol. 14(25):3948-55, 2008
                                                                                  18. Chari ST et al: Diagnosis of autoimmune pancreatitis: the Mayo Clinic experience. Clin Gastroenterol Hepatol. 4(8):1010-6; quiz 934, 2006
                                                                                  19. Kamisawa T et al: Autoimmune pancreatitis: proposal of IgG4-related sclerosing disease. J Gastroenterol. 41(7):613-25, 2006