Primary biliary cholangitis

Lindor KD, Bowlus CL, Boyer J, et al. Primary biliary cholangitis: 2018 practice guidance from the American Association for the Study of Liver Diseases. Hepatology. 2019 Jan;69(1):394-419.Full text  Abstract

European Association for the Study of the Liver. EASL clinical practice guidelines: the diagnosis and management of patients with primary biliary cholangitis. J Hepatol. 2017 Jul;67(1):145-72. Abstract

Nevens F, Andreone P, Mazzella G, et al. A placebo-controlled trial of obeticholic acid in primary biliary cholangitis. N Engl J Med. 2016 Aug 18;375(7):631-43. Abstract

1. Jones DE. Pathogenesis of primary biliary cirrhosis. Gut. 2007;56:1615-1624. Abstract

2. Hirschfield GM, Dyson JK, Alexander GJM, et al. The British Society of Gastroenterology/UK-PBC primary biliary cholangitis treatment and management guidelines. Gut. 2018 Sep;67(9):1568-94.Full text  Abstract

3. Kim WR, Lindor KD, Locke GR 3rd, et al. Epidemiology and natural history of primary biliary cirrhosis in a US community. Gastroenterology. 2000;119:1631-1636. Abstract

4. Lu M, Li J, Haller IV, et al. Factors associated with prevalence and treatment of primary biliary cholangitis in United States health systems. Clin Gastroenterol Hepatol. 2018 Aug;16(8):1333-41.e6.Full text  Abstract

5. Durazzo M, Belci P, Collo A, et al. Gender specific medicine in liver diseases: a point of view. World J Gastroenterol. 2014 Mar 7;20(9):2127-35.Full text  Abstract

6. Muratori P, Granito A, Pappas G, et al. Clinical and serological profile of primary biliary cirrhosis in men. QJM. 2007 Aug;100(8):534-5.Full text  Abstract

7. Boonstra K, Beuers U, Ponsioen CY. Epidemiology of primary sclerosing cholangitis and primary biliary cirrhosis: a systematic review. J Hepatol. 2012 May;56(5):1181-88.Full text  Abstract

8. Gazda J, Drazilova S, Janicko M, et al. The epidemiology of primary biliary cholangitis in European countries: a systematic review and meta-analysis. Can J Gastroenterol Hepatol. 2021 Jun 19;2021:9151525.Full text  Abstract

9. Lu M, Zhou Y, Haller IV, et al. Increasing prevalence of primary biliary cholangitis and reduced mortality with treatment. Clin Gastroenterol Hepatol. 2018 Aug;16(8):1342-50.e1.Full text  Abstract

10. Wang L, Gershwin ME, Wang FS. Primary biliary cholangitis in China. Curr Opin Gastroenterol. 2016 May;32(3):195-203. Abstract

11. Cheung KS, Seto WK, Fung J, et al. Epidemiology and natural history of primary biliary cholangitis in the Chinese: a territory-based study in Hong Kong between 2000 and 2015. Clin Transl Gastroenterol. 2017 Aug 31;8(8):e116.Full text  Abstract

12. Zeng N, Duan W, Chen S, et al. Epidemiology and clinical course of primary biliary cholangitis in the Asia-Pacific region: a systematic review and meta-analysis. Hepatol Int. 2019 Nov;13(6):788-99. Abstract

13. Gershwin ME, Selmi C, Worman HJ, et al. Risk factors and comorbidities in primary biliary cirrhosis: a controlled interview-based study of 1032 patients. Hepatology. 2005;42:1194-1202.Full text  Abstract

14. Lindor KD, Bowlus CL, Boyer J, et al. Primary biliary cholangitis: 2018 practice guidance from the American Association for the Study of Liver Diseases. Hepatology. 2019 Jan;69(1):394-419.Full text  Abstract

15. European Association for the Study of the Liver. EASL clinical practice guidelines: the diagnosis and management of patients with primary biliary cholangitis. J Hepatol. 2017 Jul;67(1):145-72. Abstract

16. Yeaman SJ, Kirby JA, Jones DE. Autoreactive responses to pyruvate dehydrogenase complex in the pathogenesis of primary biliary cirrhosis. Immunol Rev. 2000 Apr;174:238-49. Abstract

17. Invernizzi P, Selmi C, Ranftler C, et al. Antinuclear antibodies in primary biliary cirrhosis. Semin Liver Dis. 2005 Aug;25(3):298-310. Abstract

18. Gulamhusein AF, Hirschfield GM. Primary biliary cholangitis: pathogenesis and therapeutic opportunities. Nat Rev Gastroenterol Hepatol. 2020 Feb;17(2):93-110. Abstract

19. Jones DE, Donaldson PT. Genetic factors in the pathogenesis of primary biliary cirrhosis. Clin Liver Dis. 2003;7:841-864. Abstract

20. Lammert C, Nguyen DL, Juran BD, et al. Questionnaire based assessment of risk factors for primary biliary cirrhosis. Dig Liver Dis. 2013 Jul;45(7):589-94. Abstract

21. Prince MI, Ducker SJ, James OF. Case-control studies of risk factors for primary biliary cirrhosis in two United Kingdom populations. Gut. 2010 Apr;59(4):508-12. Abstract

22. Liang Y, Yang Z, Zhong R. Smoking, family history and urinary tract infection are associated with primary biliary cirrhosis: a meta-analysis. Hepatol Res. 2011 Jun;41(6):572-8. Abstract

23. Watt FE, James OF, Jones DE. Patterns of autoimmunity in primary biliary cirrhosis patients and their families: a population-based cohort study. QJM. 2004 Jul;97(7):397-406.Full text  Abstract

24. Jones DE, Watt FE, Metcalf JV, et al. Familial primary biliary cirrhosis reassessed: a geographically-based population study. J Hepatol. 1999 Mar;30(3):402-7. Abstract

25. Jones DE. Autoantigens in primary biliary cirrhosis. J Clin Pathol. 2000 Nov;53(11):813-21.Full text  Abstract

26. Garrido MC, Hubscher SG. Accuracy of staging in primary biliary cirrhosis. J Clin Pathol. 1996 Jul;49(7):556-9.Full text  Abstract

27. Lleo A, Wang GQ, Gershwin ME, et al. Primary biliary cholangitis. Lancet. 2020 Dec 12;396(10266):1915-26. Abstract

28. Sterling RK, Duarte-Rojo A, Patel K, et al. AASLD practice guideline on imaging-based non-invasive liver disease assessments of hepatic fibrosis and steatosis. Hepatology. 15 Mar 2024 [Epub ahead of print].Full text

29. Murillo Perez CF, Hirschfield GM, Corpechot C, et al. Fibrosis stage is an independent predictor of outcome in primary biliary cholangitis despite biochemical treatment response. Aliment Pharmacol Ther. 2019 Nov;50(10):1127-36. Abstract

30. European Association for the Study of the Liver. EASL Clinical Practice Guidelines on non-invasive tests for evaluation of liver disease severity and prognosis - 2021 update. J Hepatol. 2021 Sep;75(3):659-89.Full text  Abstract

31. Lammers WJ, Hirschfield GM, Corpechot C, et al. Development and validation of a scoring system to predict outcomes of patients with primary biliary cirrhosis receiving ursodeoxycholic acid therapy. Gastroenterology. 2015;149:1804-1812. Abstract

32. Carbone M, Sharp SJ, Flack S, et al. The UK-PBC risk scores: derivation and validation of a scoring system for long-term prediction of end-stage liver disease in primary biliary cholangitis. Hepatology. 2016;63:930-950. Abstract

33. Wesierska-Gadek J, Penner E, Battezzati PM, et al. Correlation of initial autoantibody profile and clinical outcome in primary biliary cirrhosis. Hepatology. 2006 May;43(5):1135-44. Abstract

34. Czaja AJ. Autoantibodies as prognostic markers in autoimmune liver disease. Dig Dis Sci. 2010 Aug;55(8):2144-61. Abstract

35. Granito A, Muratori P, Muratori L, et al. Antibodies to SS-A/Ro-52kD and centromere in autoimmune liver disease: a clue to diagnosis and prognosis of primary biliary cirrhosis. Aliment Pharmacol Ther. 2007 Sep 15;26(6):831-8. Abstract

36. Newton JL, Bhala N, Burt J, et al. Characterisation of the associations and impact of symptoms in primary biliary cirrhosis using a disease specific quality of life measure. J Hepatol. 2006 Apr;44(4):776-83. Abstract

37. Sorokin A, Brown JL, Thompson PD. Primary biliary cirrhosis, hyperlipidemia, and atherosclerotic risk: a systematic review. Atherosclerosis. 2007 Oct;194(2):293-9. Abstract

38. James O, Macklon AF, Watson AJ. Primary biliary cirrhosis: a revised clinical spectrum. Lancet. 1981 Jun 13;1(8233):1278-81. Abstract

39. Crowe J, Christensen E, Doniach D, et al. Early features of primary biliary cirrhosis: an analysis of 85 patients. Am J Gastroenterol. 1985 Jun;80(6):466-8. Abstract

40. Goldblatt J, Taylor PJ, Lipman T, et al. The true impact of fatigue in primary biliary cirrhosis: a population study. Gastroenterology. 2002 May;122(5):1235-41. Abstract

41. Newton JL, Gibson GJ, Tomlinson M, et al. Fatigue in primary biliary cirrhosis is associated with excessive daytime somnolence. Hepatology. 2006 Jul;44(1):91-8.Full text  Abstract

42. Newton JL, Hudson M, Tachtatzis P, et al. Population prevalence and symptom associations of autonomic dysfunction in primary biliary cirrhosis. Hepatology. 2007 Jun;45(6):1496-505.Full text  Abstract

43. Newton JL, Hollingsworth KG, Taylor R, et al. Cognitive impairment in primary biliary cirrhosis: symptom impact and potential aetiology. Hepatology. 2008 Aug;48(2):541-9. Abstract

44. Corpechot C, El Naggar A, Poujol-Robert A, et al. Assessment of biliary fibrosis by transient elastography in patients with PBC and PSC. Hepatology. 2006 May;43(5):1118-24.Full text  Abstract

45. Martin DM, Vroon DH, Nasrallah SM. Value of serum immunoglobulins in the diagnosis of liver disease. Liver. 1984 Jun;4(3):214-8. Abstract

46. Ben-Ari Z, Czaja AJ. Autoimmune hepatitis and its variant syndromes. Gut. 2001 Oct;49(4):589-94.Full text  Abstract

47. Lindor KD, Bowlus CL, Boyer J, et al. Primary biliary cholangitis: 2021 practice guidance update from the American Association for the Study of Liver Diseases. Hepatology. 2022 Apr;75(4):1012-3. Abstract

48. Carbone M, Mells G, Pells G, et al. Sex and age are determinants of the clinical phenotype of primary biliary cirrhosis and response to ursodeoxycholic acid. Gastroenterology. 2013 Mar;144(3):560-9.e7. Abstract

49. Shah RA, Kowdley KV. Current and potential treatments for primary biliary cholangitis. Lancet Gastroenterol Hepatol. 2020 Mar;5(3):306-15. Abstract

50. Nevens F, Andreone P, Mazzella G, et al. A placebo-controlled trial of obeticholic acid in primary biliary cholangitis. N Engl J Med. 2016 Aug 18;375(7):631-43. Abstract

51. Food and Drug Administration. Drug safety communication: Due to risk of serious liver injury, FDA restricts use of Ocaliva in primary biliary cholangitis (PBC) patients with advanced cirrhosis. May 2021 [internet publication].Full text

52. US Food and Drug Administration. FDA drug safety communication: FDA warns about serious liver injury with Ocaliva (obeticholic acid) for rare chronic liver disease. September 2017. https://www.fda.gov/ (last accessed 22 September 2017).Full text

53. Liermann Garcia RF, Evangalista Garcia C, McMaster P, et al. Transplantation for primary biliary cirrhosis: retrospective analysis of 400 patients in a single center. Hepatology. 2001 Jan;33(1):22-7. Abstract

54. Montano-Loza AJ, Hansen BE, Corpechot C, et al. Factors associated with recurrence of primary biliary cholangitis after liver transplantation and effects on graft and patient survival. Gastroenterology. 2019 Jan;156(1):96-107. Abstract

55. Bosch A, Dumortier J, Maucort-Boulch D, et al. Preventive administration of UDCA after liver transplantation for primary biliary cirrhosis is associated with a lower risk of disease recurrence. J Hepatol. 2015 Dec;63(6):1449-58. Abstract

56. Jones DEJ. Complications of cholestasis. Medicine. 2002;30:67-68.

57. Datta DV, Sherlock S. Cholestyramine for long term relief of the pruritus complicating intrahepatic cholestasis. Gastroenterology. 1966 Mar;50(3):323-32. Abstract

58. Tandon P, Rowe BH, Vandermeer B, et al. The efficacy and safety of bile acid binding agents, opioid antagonists, or rifampin in the treatment of cholestasis-associated pruritus. Am J Gastroenterol. 2007 Jul;102(7):1528-36. Abstract

59. Khurana S, Singh P. Rifampin is safe for treatment of pruritus due to chronic cholestasis: a meta-analysis of prospective randomized-controlled trials. Liver Int. 2006 Oct;26(8):943-8. Abstract

60. Prince MI, Burt AD, Jones DE. Hepatitis and liver dysfunction with rifampicin therapy for pruritus in primary biliary cirrhosis. Gut. 2002 Mar;50(3):436-9.Full text  Abstract

61. Gross CR, Malinchoc M, Kim WR, et al. Quality of life before and after liver transplantation for cholestatic liver disease. Hepatology. 1999 Feb;29(2):356-64. Abstract

62. Lee JY, Danford CJ, Trivedi HD, et al. Treatment of fatigue in primary biliary cholangitis: a systematic review and meta-analysis. Dig Dis Sci. 2019 Aug;64(8):2338-50. Abstract

63. Siegel JL, Jorgensen R, Angulo P, et al. Treatment of ursodeoxycholic acid is associated with weight gain in patients with primary biliary cirrhosis. J Clin Gastroenterol. 2003 Aug;37(2):183-5. Abstract

64. Rudic JS, Poropat G, Krstic MN, et al. Bezafibrate for primary biliary cirrhosis. Cochrane Database Syst Rev. 2012 Jan 18;1:CD009145.Full text  Abstract

65. Corpechot C, Chazouillères O, Rousseau A, et al. A placebo-controlled trial of bezafibrate in primary biliary cholangitis. N Engl J Med. 2018 Jun 7;378(23):2171-81.Full text  Abstract

66. Study to evaluate the efficacy and safety of elafibranor in patients with primary biliary cholangitis (PBC) and inadequate response to ursodeoxycholic acid. NCT03124108. September 2019 [internet publication].Full text

67. Prince M, Chetwynd A, Newman W, et al. Survival and symptom progression in a geographically based cohort of patients with primary biliary cirrhosis: follow-up for up to 28 years. Gastroenterology. 2002 Oct;123(4):1044-51. Abstract

68. Gores GJ, Wiesner RH, Dickson ER, et al. Prospective evaluation of esophageal varices in primary biliary cirrhosis: development, natural history, and influence on survival. Gastroenterology. 1989 Jun;96(6):1552-9. Abstract

69. Jones DE, Metcalf JV, Collier JD, et al. Hepatocellular carcinoma in primary biliary cirrhosis and its impact on outcomes. Hepatology. 1997 Nov;26(5):1138-42. Abstract

70. Jacoby A, Rannard A, Buck D, et al. Development, validation, and evaluation of the PBC-40, a disease specific health related quality of life measure for primary biliary cirrhosis. Gut. 2005 Nov;54(11):1622-9. Abstract

71. Poupon RE, Chretien Y, Chazouilleres O, et al. Quality of life in patients with primary biliary cirrhosis. Hepatology. 2004 Aug;40(2):489-94.Full text  Abstract

72. Centers for Disease Control and Prevention. Adult immunization schedule by age: recommendations for ages 19 years or older, United States, 2024. Nov 2023 [internet publication].Full text