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Primary Biliary Cholangitis
Atif Zaheer, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Pathology

          • Clinical Issues

            TERMINOLOGY

            • Abbreviations

              • Primary biliary cholangitis (PBC)
            • Synonyms

              • Primary biliary cirrhosis
            • Definitions

              • Chronic progressive cholestatic liver disease characterized by nonsuppurative destruction of interlobular bile ducts
                • Leads to advanced fibrosis, cirrhosis, and liver failure

            IMAGING

            • General Features

              • CT Findings

                • MR Findings

                  • Ultrasonographic Findings

                    • Nuclear Medicine Findings

                      • Other Modality 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. Idilman IS et al: Magnetic resonance imaging features in 283 patients with primary biliary cholangitis. Eur Radiol. 30(9):5139-48, 2020
                                              2. Mané F et al: Treatment response in primary biliary cholangitis: the role of autoimmune hepatitis features. Ann Hepatol. 18(3):488-93, 2019
                                              3. Czaja AJ et al: Autoimmune hepatitis overlap syndromes and liver pathology. Gastroenterol Clin North Am. 46(2):345-64, 2017
                                              4. Kovač JD et al: Primary biliary cirrhosis and primary sclerosing cholangitis: an update on MR imaging findings with recent developments. J Gastrointestin Liver Dis. 25(4):517-24, 2016
                                              5. Meng Y et al: The value of MRI in the diagnosis of primary biliary cirrhosis and assessment of liver fibrosis. PLoS One. 10(3):e0120110, 2015
                                              6. Karlsen TH et al: Review article: controversies in the management of primary biliary cirrhosis and primary sclerosing cholangitis. Aliment Pharmacol Ther. 39(3):282-301, 2014
                                              7. Granito A et al: Antinuclear antibodies as ancillary markers in primary biliary cirrhosis. Expert Rev Mol Diagn. 12(1):65-74, 2012
                                              8. Chuang N et al: Update on the epidemiology of primary biliary cirrhosis. Expert Rev Gastroenterol Hepatol. 5(5):583-90, 2011
                                              9. Grattagliano I et al: Long-term ursodeoxycholate improves circulating redox changes in primary biliary cirrhotic patients. Clin Biochem. 44(17-18):1400-4, 2011
                                              10. Kim KA et al: The diagnosis and treatment of primary biliary cirrhosis. Korean J Hepatol. 17(3):173-9, 2011
                                              11. Mendes F et al: Recurrent and de novo autoimmune liver diseases. Clin Liver Dis. 15(4):859-78, 2011
                                              12. Gonzales E et al: Liver diseases related to MDR3 (ABCB4) gene deficiency. Front Biosci. 14:4242-56, 2009
                                              13. Kuiper EM et al: Improved prognosis of patients with primary biliary cirrhosis that have a biochemical response to ursodeoxycholic acid. Gastroenterology. 136(4):1281-7, 2009
                                              14. Watanabe T et al: Features of hepatocellular carcinoma in cases with autoimmune hepatitis and primary biliary cirrhosis. World J Gastroenterol. 15(2):231-9, 2009
                                              15. Kita H et al: Pathogenesis of primary biliary cirrhosis. Clin Liver Dis. 7(4):821-39, 2003
                                              16. MacQuillan GC et al: Liver transplantation for primary biliary cirrhosis. Clin Liver Dis. 7(4):941-56, ix, 2003
                                              17. Blachar A et al: Primary biliary cirrhosis: clinical, pathologic, and helical CT findings in 53 patients. Radiology. 220(2):329-36, 2001
                                              Related Anatomy
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                                              Related Differential Diagnoses
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                                              References
                                              Tables

                                              Tables

                                              KEY FACTS

                                              • Terminology

                                                • Imaging

                                                  • Top Differential Diagnoses

                                                    • Pathology

                                                      • Clinical Issues

                                                        TERMINOLOGY

                                                        • Abbreviations

                                                          • Primary biliary cholangitis (PBC)
                                                        • Synonyms

                                                          • Primary biliary cirrhosis
                                                        • Definitions

                                                          • Chronic progressive cholestatic liver disease characterized by nonsuppurative destruction of interlobular bile ducts
                                                            • Leads to advanced fibrosis, cirrhosis, and liver failure

                                                        IMAGING

                                                        • General Features

                                                          • CT Findings

                                                            • MR Findings

                                                              • Ultrasonographic Findings

                                                                • Nuclear Medicine Findings

                                                                  • Other Modality 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. Idilman IS et al: Magnetic resonance imaging features in 283 patients with primary biliary cholangitis. Eur Radiol. 30(9):5139-48, 2020
                                                                                          2. Mané F et al: Treatment response in primary biliary cholangitis: the role of autoimmune hepatitis features. Ann Hepatol. 18(3):488-93, 2019
                                                                                          3. Czaja AJ et al: Autoimmune hepatitis overlap syndromes and liver pathology. Gastroenterol Clin North Am. 46(2):345-64, 2017
                                                                                          4. Kovač JD et al: Primary biliary cirrhosis and primary sclerosing cholangitis: an update on MR imaging findings with recent developments. J Gastrointestin Liver Dis. 25(4):517-24, 2016
                                                                                          5. Meng Y et al: The value of MRI in the diagnosis of primary biliary cirrhosis and assessment of liver fibrosis. PLoS One. 10(3):e0120110, 2015
                                                                                          6. Karlsen TH et al: Review article: controversies in the management of primary biliary cirrhosis and primary sclerosing cholangitis. Aliment Pharmacol Ther. 39(3):282-301, 2014
                                                                                          7. Granito A et al: Antinuclear antibodies as ancillary markers in primary biliary cirrhosis. Expert Rev Mol Diagn. 12(1):65-74, 2012
                                                                                          8. Chuang N et al: Update on the epidemiology of primary biliary cirrhosis. Expert Rev Gastroenterol Hepatol. 5(5):583-90, 2011
                                                                                          9. Grattagliano I et al: Long-term ursodeoxycholate improves circulating redox changes in primary biliary cirrhotic patients. Clin Biochem. 44(17-18):1400-4, 2011
                                                                                          10. Kim KA et al: The diagnosis and treatment of primary biliary cirrhosis. Korean J Hepatol. 17(3):173-9, 2011
                                                                                          11. Mendes F et al: Recurrent and de novo autoimmune liver diseases. Clin Liver Dis. 15(4):859-78, 2011
                                                                                          12. Gonzales E et al: Liver diseases related to MDR3 (ABCB4) gene deficiency. Front Biosci. 14:4242-56, 2009
                                                                                          13. Kuiper EM et al: Improved prognosis of patients with primary biliary cirrhosis that have a biochemical response to ursodeoxycholic acid. Gastroenterology. 136(4):1281-7, 2009
                                                                                          14. Watanabe T et al: Features of hepatocellular carcinoma in cases with autoimmune hepatitis and primary biliary cirrhosis. World J Gastroenterol. 15(2):231-9, 2009
                                                                                          15. Kita H et al: Pathogenesis of primary biliary cirrhosis. Clin Liver Dis. 7(4):821-39, 2003
                                                                                          16. MacQuillan GC et al: Liver transplantation for primary biliary cirrhosis. Clin Liver Dis. 7(4):941-56, ix, 2003
                                                                                          17. Blachar A et al: Primary biliary cirrhosis: clinical, pathologic, and helical CT findings in 53 patients. Radiology. 220(2):329-36, 2001