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Primary Sclerosing Cholangitis
Siva P. Raman, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Pathology

        • Clinical Issues

          TERMINOLOGY

          • Abbreviations

            • Primary sclerosing cholangitis (PSC)
          • Definitions

            • Chronic, immune-mediated disease causing progressive inflammation, fibrosis, and stricturing of intrahepatic and extrahepatic ducts

          IMAGING

          • General Features

            • MR Findings

              • CT Findings

                • Radiographic Findings

                  • Ultrasonographic Findings

                    • Imaging Recommendations

                      DIFFERENTIAL DIAGNOSIS

                        PATHOLOGY

                        • General Features

                          • Gross Pathologic & Surgical Features

                            • Microscopic Features

                              CLINICAL ISSUES

                              • Presentation

                                • Demographics

                                  • Natural History & Prognosis

                                    • Treatment

                                      DIAGNOSTIC CHECKLIST

                                      • Consider

                                        • Image Interpretation Pearls

                                          Selected References

                                          1. Fung BM et al: Cancer risk in primary sclerosing cholangitis: epidemiology, prevention, and surveillance strategies. World J Gastroenterol. 25(6):659-71, 2019
                                          2. Selvaraj EA et al: Evolving role of magnetic resonance techniques in primary sclerosing cholangitis. World J Gastroenterol. 25(6):644-58, 2019
                                          3. Lee JJ et al: Cholangiocarcinoma and its mimickers in primary sclerosing cholangitis. Abdom Radiol (NY). 42(12):2898-2908, 2017
                                          4. Nakazawa T et al: The 2016 diagnostic criteria for primary sclerosing cholangitis. J Gastroenterol. 52(7):838-44, 2017
                                          5. Boyd S et al: Screening primary sclerosing cholangitis and biliary dysplasia with endoscopic retrograde cholangiography and brush cytology: risk factors for biliary neoplasia. Endoscopy. 48(5):432-9, 2016
                                          6. Folseraas T et al: Cancer risk and surveillance in primary sclerosing cholangitis. Clin Liver Dis. 20(1):79-98, 2016
                                          7. Costello JR et al: MR imaging of benign and malignant biliary conditions. Magn Reson Imaging Clin N Am. 22(3):467-88, 2014
                                          8. Williamson KD et al: Primary sclerosing cholangitis. Dig Dis. 32(4):438-45, 2014
                                          9. Arrivé L et al: MRI of cholangitis: traps and tips. Diagn Interv Imaging. 94(7-8):757-70, 2013
                                          10. Chundru S et al: MRI of diffuse liver disease: the common and uncommon etiologies. Diagn Interv Radiol. 19(6):479-87, 2013
                                          11. Hirschfield GM et al: Primary sclerosing cholangitis. Lancet. 382(9904):1587-99, 2013
                                          12. Kovač JD et al: MR imaging of primary sclerosing cholangitis: additional value of diffusion-weighted imaging and ADC measurement. Acta Radiol. 54(3):242-8, 2013
                                          13. Zein CO: Primary sclerosing cholangitis. Clin Liver Dis. 17(2):211-27, 2013
                                          14. Azizi L et al: MR Imaging of sclerosing cholangitis. Clin Res Hepatol Gastroenterol. 36(2):130-8, 2012
                                          15. Barnabas A et al: Primary sclerosing cholangitis: is any treatment worthwhile? Curr Gastroenterol Rep. 14(1):17-24, 2012
                                          16. Krones E et al: Evolving concepts in primary sclerosing cholangitis. Liver Int. 32(3):352-69, 2012
                                          17. Karlsen TH et al: Update on primary sclerosing cholangitis. Dig Liver Dis. 42(6):390-400, 2010
                                          18. Menias CO et al: Mimics of cholangiocarcinoma: spectrum of disease. Radiographics. 28(4):1115-29, 2008
                                          19. Elsayes KM et al: MR and MRCP in the evaluation of primary sclerosing cholangitis: current applications and imaging findings. J Comput Assist Tomogr. 30(3):398-404, 2006
                                          20. Bader TR et al: MR imaging features of primary sclerosing cholangitis: patterns of cirrhosis in relationship to clinical severity of disease. Radiology. 226(3):675-85, 2003
                                          21. Campbell WL et al: Using CT and cholangiography to diagnose biliary tract carcinoma complicating primary sclerosing cholangitis. AJR Am J Roentgenol. 177(5):1095-100, 2001
                                          22. Fulcher AS et al: Primary sclerosing cholangitis: evaluation with MR cholangiography-a case-control study. Radiology. 215(1):71-80, 2000
                                          23. Vitellas KM et al: Radiologic manifestations of sclerosing cholangitis with emphasis on MR cholangiopancreatography. Radiographics. 20(4):959-75; quiz 1108-9, 1112, 2000
                                          Related Anatomy
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                                          Related Differential Diagnoses
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                                          References
                                          Tables

                                          Tables

                                          KEY FACTS

                                          • Terminology

                                            • Imaging

                                              • Pathology

                                                • Clinical Issues

                                                  TERMINOLOGY

                                                  • Abbreviations

                                                    • Primary sclerosing cholangitis (PSC)
                                                  • Definitions

                                                    • Chronic, immune-mediated disease causing progressive inflammation, fibrosis, and stricturing of intrahepatic and extrahepatic ducts

                                                  IMAGING

                                                  • General Features

                                                    • MR Findings

                                                      • CT Findings

                                                        • Radiographic Findings

                                                          • Ultrasonographic Findings

                                                            • Imaging Recommendations

                                                              DIFFERENTIAL DIAGNOSIS

                                                                PATHOLOGY

                                                                • General Features

                                                                  • Gross Pathologic & Surgical Features

                                                                    • Microscopic Features

                                                                      CLINICAL ISSUES

                                                                      • Presentation

                                                                        • Demographics

                                                                          • Natural History & Prognosis

                                                                            • Treatment

                                                                              DIAGNOSTIC CHECKLIST

                                                                              • Consider

                                                                                • Image Interpretation Pearls

                                                                                  Selected References

                                                                                  1. Fung BM et al: Cancer risk in primary sclerosing cholangitis: epidemiology, prevention, and surveillance strategies. World J Gastroenterol. 25(6):659-71, 2019
                                                                                  2. Selvaraj EA et al: Evolving role of magnetic resonance techniques in primary sclerosing cholangitis. World J Gastroenterol. 25(6):644-58, 2019
                                                                                  3. Lee JJ et al: Cholangiocarcinoma and its mimickers in primary sclerosing cholangitis. Abdom Radiol (NY). 42(12):2898-2908, 2017
                                                                                  4. Nakazawa T et al: The 2016 diagnostic criteria for primary sclerosing cholangitis. J Gastroenterol. 52(7):838-44, 2017
                                                                                  5. Boyd S et al: Screening primary sclerosing cholangitis and biliary dysplasia with endoscopic retrograde cholangiography and brush cytology: risk factors for biliary neoplasia. Endoscopy. 48(5):432-9, 2016
                                                                                  6. Folseraas T et al: Cancer risk and surveillance in primary sclerosing cholangitis. Clin Liver Dis. 20(1):79-98, 2016
                                                                                  7. Costello JR et al: MR imaging of benign and malignant biliary conditions. Magn Reson Imaging Clin N Am. 22(3):467-88, 2014
                                                                                  8. Williamson KD et al: Primary sclerosing cholangitis. Dig Dis. 32(4):438-45, 2014
                                                                                  9. Arrivé L et al: MRI of cholangitis: traps and tips. Diagn Interv Imaging. 94(7-8):757-70, 2013
                                                                                  10. Chundru S et al: MRI of diffuse liver disease: the common and uncommon etiologies. Diagn Interv Radiol. 19(6):479-87, 2013
                                                                                  11. Hirschfield GM et al: Primary sclerosing cholangitis. Lancet. 382(9904):1587-99, 2013
                                                                                  12. Kovač JD et al: MR imaging of primary sclerosing cholangitis: additional value of diffusion-weighted imaging and ADC measurement. Acta Radiol. 54(3):242-8, 2013
                                                                                  13. Zein CO: Primary sclerosing cholangitis. Clin Liver Dis. 17(2):211-27, 2013
                                                                                  14. Azizi L et al: MR Imaging of sclerosing cholangitis. Clin Res Hepatol Gastroenterol. 36(2):130-8, 2012
                                                                                  15. Barnabas A et al: Primary sclerosing cholangitis: is any treatment worthwhile? Curr Gastroenterol Rep. 14(1):17-24, 2012
                                                                                  16. Krones E et al: Evolving concepts in primary sclerosing cholangitis. Liver Int. 32(3):352-69, 2012
                                                                                  17. Karlsen TH et al: Update on primary sclerosing cholangitis. Dig Liver Dis. 42(6):390-400, 2010
                                                                                  18. Menias CO et al: Mimics of cholangiocarcinoma: spectrum of disease. Radiographics. 28(4):1115-29, 2008
                                                                                  19. Elsayes KM et al: MR and MRCP in the evaluation of primary sclerosing cholangitis: current applications and imaging findings. J Comput Assist Tomogr. 30(3):398-404, 2006
                                                                                  20. Bader TR et al: MR imaging features of primary sclerosing cholangitis: patterns of cirrhosis in relationship to clinical severity of disease. Radiology. 226(3):675-85, 2003
                                                                                  21. Campbell WL et al: Using CT and cholangiography to diagnose biliary tract carcinoma complicating primary sclerosing cholangitis. AJR Am J Roentgenol. 177(5):1095-100, 2001
                                                                                  22. Fulcher AS et al: Primary sclerosing cholangitis: evaluation with MR cholangiography-a case-control study. Radiology. 215(1):71-80, 2000
                                                                                  23. Vitellas KM et al: Radiologic manifestations of sclerosing cholangitis with emphasis on MR cholangiopancreatography. Radiographics. 20(4):959-75; quiz 1108-9, 1112, 2000