Primary biliary cholangitis

References

Key articles

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

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

Reference articles

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

2. 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

3. James OF, Bhopal R, Howel D, et al. Primary biliary cirrhosis once rare, now common in the United Kingdom? Hepatology. 1999;30:390-394.Full text  Abstract

4. 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

5. Lucey MR, Neuberger JM, Williams R. Primary biliary cirrhosis in men. Gut. 1986;27:1373-1376.Full text  Abstract

6. Jones DE. Pathogenesis of primary biliary cirrhosis. Gut. 2007 Nov;56(11):1615-24. Abstract

7. 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

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

9. 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

10. Jones DE, Donaldson PT. Genetic factors in the pathogenesis of primary biliary cirrhosis. Clin Liver Dis. 2003 Nov;7(4):841-64. Abstract

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

12. Kim WR, Lindor KD, Locke GR 3rd, et al. Epidemiology and natural history of primary biliary cirrhosis in a US community. Gastroenterology. 2000 Dec;119(6):1631-6. Abstract

13. James OF, Bhopal R, Howel D, et al. Primary biliary cirrhosis once rare, now common in the United Kingdom? Hepatology. 1999 Aug;30(2):390-4.Full text  Abstract

14. 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 Nov;42(5):1194-202.Full text  Abstract

15. 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

16. 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

17. 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

18. 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

19. Howel D, Fischbacher CM, Bhopal RS, et al. An exploratory population-based case-control study of primary biliary cirrhosis. Hepatology. 2000 May;31(5):1055-60.Full text  Abstract

20. Lucey MR, Neuberger JM, Williams R. Primary biliary cirrhosis in men. Gut. 1986 Nov;27(11):1373-6.Full text  Abstract

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

22. Metcalf JV, Bhopal RS, Gray J, et al. Incidence and prevalence of primary biliary cirrhosis in the city of Newcastle-upon-Tyne, England. Int J Epidemiol. 1997 Aug;26(4):830-6.Full text  Abstract

23. Zein CO, Angulo P, Lindor KD. When is liver biopsy needed in the diagnosis of primary biliary cirrhosis? Clin Gastroenterol Hepatol. 2003 Mar;1(2):89-95. Abstract

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

25. 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 Dec;149(7):1804-1812.e4. Abstract

26. 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 Mar;63(3):930-50. Abstract

27. 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

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

29. 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

30. 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

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

32. Sokol RJ, Kim YS, Hoofnagle JH, et al. Intestinal malabsorption of vitamin E in primary biliary cirrhosis. Gastroenterology. 1989 Feb;96(2 Pt 1):479-86. Abstract

33. 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

34. 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

35. 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

36. 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

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

38. 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

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

40. 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

41. Pauli-Magnus C, Meier PJ. Hepatobiliary transporters and drug-induced cholestasis. Hepatology. 2006 Oct;44(4):778-87.Full text  Abstract

42. Arrese M. Cholestasis during pregnancy: rare hepatic diseases unmasked by pregnancy. Ann Hepatol. 2006 Jul-Sep;5(3):216-8. Abstract

43. Jones DE, Sutcliffe K, Pairman J, et al. An integrated care pathway improves quality of life in primary biliary cirrhosis. QJM. 2008 Jul;101(7):535-43.Full text  Abstract

44. 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):560-569.e7. Abstract

45. 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

46. 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 [internet publication].Full text

47. 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

48. 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.Full text  Abstract

49. Bressler B, Pinto R, El-Ashry D, et al. Which patients with primary biliary cirrhosis or primary sclerosing cholangitis should undergo endoscopic screening for oesophageal varices detection? Gut. 2005 Mar;54(3):407-10.Full text  Abstract

50. 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.Full text  Abstract

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

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

53. 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

54. 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

55. Jones EA, Neuberger JM, Bergasa NV. Opiate antagonist therapy for the pruritus of cholestasis: the avoidance of opioid withdrawal-like reactions. QJM. 2002 Aug;95(8):547-52.Full text  Abstract

56. McRae CA, Prince MI, Hudson M, et al. Pain as a complication of use of opiate antagonists for symptom control in cholestasis. Gastroenterology. 2003 Aug;125(2):591-6. Abstract

57. Schworer H, Hartmann H, Ramadori G. Relief of cholestatic pruritus by a novel class of drugs: 5-hydroxytryptamine type 3 (5-HT3) receptor antagonists: effectiveness of ondansetron. Pain. 1995 Apr;61(1):33-7. Abstract

58. Mayo MJ, Handem I, Saldana S, et al. Sertraline as a first-line treatment for cholestatic pruritus. Hepatology. 2007 Mar;45(3):666-74. Abstract

59. Hegade VS, Kendrick SF, Dobbins RL, et al. Effect of ileal bile acid transporter inhibitor GSK2330672 on pruritus in primary biliary cholangitis: a double-blind, randomised, placebo-controlled, crossover, phase 2a study. Lancet. 2017 Mar 18;389(10074):1114-23. Abstract

60. 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.Full text  Abstract

61. Kremer AE, Martens JJ, Kulik W, et al. Lysophosphatidic acid is a potential mediator of cholestatic pruritus. Gastroenterology. 2010 Sep;139(3):1008-18, 1018.e1. Abstract

62. Rudic JS, Poropat G, Krstic MN, et al. Hormone replacement for osteoporosis in women with primary biliary cirrhosis. Cochrane Database Syst Rev. 2011 Dec 7;(12):CD009146.Full text  Abstract

63. Rudic JS, Giljaca V, Krstic MN, et al. Bisphosphonates for osteoporosis in primary biliary cirrhosis. Cochrane Database Syst Rev. 2011 Dec 7;(12):CD009144.Full text  Abstract

64. 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

65. 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

66. Gong Y, Christensen E, Gluud C. Azathioprine for primary biliary cirrhosis. Cochrane Database Syst Rev. 2007 Jul 18;(3):CD006000. Abstract

67. 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

68. 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

69. 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

70. 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

71. 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

72. 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

73. 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

74. Corpechot C, Abenavoli L, Rabahi N, et al. Biochemical response to ursodeoxycholic acid and long-term prognosis in primary biliary cirrhosis. Hepatology. 2008 Sep;48(3):871-7.Full text  Abstract

75. Pares A, Caballeria L, Rodes J, et al. Long-term effects of ursodeoxycholic acid in primary biliary cirrhosis: results of a double-blind controlled multicentric trial. J Hepatol. 2000 Apr;32(4):561-6. Abstract

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