Which physical findings are characteristic of primary biliary cholangitis (PBC)?

1. American Liver Foundation. Primary biliary cholangitis (PBC, primary biliary cirrhosis). March 29, 2016. Available at http://www.liverfoundation.org/abouttheliver/info/pbc/. Accessed: June 3, 2016.

2. Ahrens EH Jr, Payne MA, Kunkel HG, Eisenmenger WJ, Blondheim SH. Primary biliary cirrhosis. 1950. Medicine (Baltimore). 1994 Sep. 73(5):264-78; discussion 278-80. [Medline].

3. Solis Herruzo JA, Solis Munoz P, Munoz Yague T. The pathogenesis of primary biliary cirrhosis. Rev Esp Enferm Dig. 2009 Jun. 101(6):413-23. [Medline].

4. McNally RJ, Ducker S, James OF. Are transient environmental agents involved in the cause of primary biliary cirrhosis? Evidence from space-time clustering analysis. Hepatology. 2009 Oct. 50(4):1169-74. [Medline].

5. Selmi C, Gershwin ME. The role of environmental factors in primary biliary cirrhosis. Trends Immunol. 2009 Aug. 30(8):415-20. [Medline].

6. Mendes FD, Kim WR, Pedersen R, et al. Mortality attributable to cholestatic liver disease in the United States. Hepatology. 2008 Apr. 47(4):1241-7. [Medline].

7. Niro GA, Poli F, Andriulli A, et al. TNF-alpha polymorphisms in primary biliary cirrhosis: a northern and southern Italian experience. Ann N Y Acad Sci. 2009 Sep. 1173:557-63. [Medline].

8. Drebber U, Mueller JJ, Klein E, et al. Liver biopsy in primary biliary cirrhosis: clinicopathological data and stage. Pathol Int. 2009 Aug. 59(8):546-54. [Medline].

9. Scheuer P. Primary biliary cirrhosis. Proc R Soc Med. 1967 Dec. 60(12):1257-60. [Medline]. [Full Text].

10. Charatcharoenwitthaya P, Pimentel S, Talwalkar JA, et al. Long-term survival and impact of ursodeoxycholic acid treatment for recurrent primary biliary cirrhosis after liver transplantation. Liver Transpl. 2007 Sep. 13(9):1236-45. [Medline].

11. Tsuda M, Moritoki Y, Lian ZX, et al. Biochemical and immunologic effects of rituximab in patients with primary biliary cirrhosis and an incomplete response to ursodeoxycholic acid. Hepatology. 2012 Feb. 55(2):512-21. [Medline].

12. US Food and Drug Administration. FDA approves Ocaliva for rare, chronic liver disease. May 31, 2016. Available at http://www.fda.gov/NewsEvents/Newsroom/PressAnnouncements/ucm503964.htm. Accessed: June 1, 2016.

13. Edwards JE, LaCerte C, Pheng LH, et al. Sa1576 Exposure-response relationship of obeticholic acid for alkaline phosphatase and total bilirubin in patients with primary biliary cirrhosis (PBC). Poster presented at Digestive Disease Week; San Diego, California; May 21, 2016.

14. Rudic JS, Giljaca V, Krstic MN, Bjelakovic G, Gluud C. Bisphosphonates for osteoporosis in primary biliary cirrhosis. Cochrane Database Syst Rev. 2011 Dec 7. 12:CD009144. [Medline].

15. Bjornsson E, Kalaitzakis E, Neuhauser M, et al. Fatigue measurements in patients with primary biliary cirrhosis and the risk of mortality during follow-up. Liver Int. 2010 Feb. 30(2):251-8. [Medline].

16. Balasubramaniam K, Grambsch PM, Wiesner RH, et al. Diminished survival in asymptomatic primary biliary cirrhosis. A prospective study. Gastroenterology. 1990 Jun. 98(6):1567-71. [Medline].

17. Ballardini G, Mirakian R, Bianchi FB, et al. Aberrant expression of HLA-DR antigens on bileduct epithelium in primary biliary cirrhosis: relevance to pathogenesis. Lancet. 1984 Nov 3. 2(8410):1009-13. [Medline].

18. Burroughs AK, Rosenstein IJ, Epstein O, et al. Bacteriuria and primary biliary cirrhosis. Gut. 1984 Feb. 25(2):133-7. [Medline].

19. Bush A, Mitchison H, Walt R, et al. Primary biliary cirrhosis and ulcerative colitis. Gastroenterology. 1987 Jun. 92(6):2009-13. [Medline].

20. Calmus Y, Gane P, Rouger P, et al. Hepatic expression of class I and class II major histocompatibility complex molecules in primary biliary cirrhosis: effect of ursodeoxycholic acid. Hepatology. 1990 Jan. 11(1):12-5. [Medline].

21. Christensen E, Crowe J, Doniach D, et al. Clinical pattern and course of disease in primary biliary cirrhosis based on an analysis of 236 patients. Gastroenterology. 1980 Feb. 78(2):236-46. [Medline].

22. Culp KS, Fleming CR, Duffy J, et al. Autoimmune associations in primary biliary cirrhosis. Mayo Clin Proc. 1982 Jun. 57(6):365-70. [Medline].

23. Dickson ER, Grambsch PM, Fleming TR, et al. Prognosis in primary biliary cirrhosis: model for decision making. Hepatology. 1989 Jul. 10(1):1-7. [Medline].

24. Elta GH, Sepersky RA, Goldberg MJ, et al. Increased incidence of hypothyroidism in primary biliary cirrhosis. Dig Dis Sci. 1983 Nov. 28(11):971-5. [Medline].

25. 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. [Medline].

26. Hoffmann RM, Pape GR, Spengler U, et al. Clonal analysis of liver-derived T cells of patients with primary biliary cirrhosis. Clin Exp Immunol. 1989 May. 76(2):210-5. [Medline].

27. Janes CH, Dickson ER, Okazaki R, et al. Role of hyperbilirubinemia in the impairment of osteoblast proliferation associated with cholestatic jaundice. J Clin Invest. 1995 Jun. 95(6):2581-6. [Medline].

28. 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. [Medline].

29. Kaplan MM, Elta GH, Furie B, et al. Fat-soluble vitamin nutriture in primary biliary cirrhosis. Gastroenterology. 1988 Sep. 95(3):787-92. [Medline].

30. Keller DM. Beta blockers ameliorate GI permeability in cirrhosis. Medscape Medical News from WebMD. May 2, 2013. Available at http://www.medscape.com/viewarticle/803542. Accessed: May 14, 2013.

31. 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. [Medline].

32. Klein R, Huizenga JR, Gips CH, et al. Antimitochondrial antibody profiles in patients with primary biliary cirrhosis before orthotopic liver transplantation and titres of antimitochondrial antibody-subtypes after transplantation. J Hepatol. 1994 Feb. 20(2):181-9. [Medline].

33. Lacerda MA, Ludwig J, Dickson ER, et al. Antimitochondrial antibody-negative primary biliary cirrhosis. Am J Gastroenterol. 1995 Feb. 90(2):247-9. [Medline].

34. Liermann Garcia RF, Evangelista Garcia C, et al. Transplantation for primary biliary cirrhosis: retrospective analysis of 400 patients in a single center. Hepatology. 2001 Jan. 33(1):22-7. [Medline].

35. Lindor K, Dickson R. Primary biliary cirrhosis. Schiff ER, Sorrell MF, Maddrey WC, eds. Schiff's Diseases of the Liver. 8th ed. Lippincott-Raven: Philadelphia; 1999. Vol 1: 679-92.

36. Mahl TC, Shockcor W, Boyer JL. Primary biliary cirrhosis: survival of a large cohort of symptomatic and asymptomatic patients followed for 24 years. J Hepatol. 1994 Jun. 20(6):707-13. [Medline].

37. Mang FW, Michieletti P, O'Rourke K, et al. Primary biliary cirrhosis, sicca complex, and dysphagia. Dysphagia. 1997 Summer. 12(3):167-70. [Medline].

38. Marx WJ, O'Connell DJ. Arthritis of primary biliary cirrhosis. Arch Intern Med. 1979 Feb. 139(2):213-6. [Medline].

39. Nakanuma Y, Tsuneyama K, Kono N, et al. Biliary epithelial expression of pyruvate dehydrogenase complex in primary biliary cirrhosis: an immunohistochemical and immunoelectron microscopic study. Hum Pathol. 1995 Jan. 26(1):92-8. [Medline].

40. Newton JL, Gibson GJ, Tomlinson M, Wilton K, Jones D. Fatigue in primary biliary cirrhosis is associated with excessive daytime somnolence. Hepatology. 2006 Jul. 44(1):91-8. [Medline].

41. Pares A, Rimola A, Bruguera M, et al. Renal tubular acidosis in primary biliary cirrhosis. Gastroenterology. 1981 Apr. 80(4):681-6. [Medline].

42. Parikh-Patel A, Gold E, Mackay IR, et al. The geoepidemiology of primary biliary cirrhosis: contrasts and comparisons with the spectrum of autoimmune diseases. Clin Immunol. 1999 May. 91(2):206-18. [Medline].

43. Pares A, Caballeria L, Rodes J. Excellent long-term survival in patients with primary biliary cirrhosis and biochemical response to ursodeoxycholic Acid. Gastroenterology. 2006 Mar. 130(3):715-20. [Medline].

44. Reynolds TB, Denison EK, Frankl HD, Lieberman FL, Peters RL. Primary biliary cirrhosis with scleroderma, Raynaud's phenomenon and telangiectasia. New syndrome. Am J Med. 1971 Mar. 50(3):302-12. [Medline].

45. Ricci P, Therneau TM, Malinchoc M, et al. A prognostic model for the outcome of liver transplantation in patients with cholestatic liver disease. Hepatology. 1997 Mar. 25(3):672-7. [Medline].

46. Roll J, Boyer JL, Barry D, et al. The prognostic importance of clinical and histologic features in asymptomatic and symptomatic primary biliary cirrhosis. N Engl J Med. 1983 Jan 6. 308(1):1-7. [Medline].

47. Ros E, Garcia-Puges A, Reixach M, et al. Fat digestion and exocrine pancreatic function in primary biliary cirrhosis. Gastroenterology. 1984 Jul. 87(1):180-7. [Medline].

48. Rutan G, Martinez AJ, Fieshko JT, et al. Primary biliary cirrhosis, Sjogren's syndrome, and transverse myelitis. Gastroenterology. 1986 Jan. 90(1):206-10. [Medline].

49. Selinger S, Tsai J, Pulini M, et al. Autoimmune thrombocytopenia and primary biliary cirrhosis with hypoglycemia and insulin receptor autoantibodies. A case report. Ann Intern Med. 1987 Nov. 107(5):686-8. [Medline].

50. Shapiro JM, Smith H, Schaffner F. Serum bilirubin: a prognostic factor in primary biliary cirrhosis. Gut. 1979 Feb. 20(2):137-40. [Medline].

51. Thomas PK, Walker JG. Xanthomatous neuropathy in primary biliary cirrhosis. Brain. 1965 Dec. 88(5):1079-88. [Medline].

52. Yamada G, Hyodo I, Tobe K, et al. Ultrastructural immunocytochemical analysis of lymphocytes infiltrating bile duct epithelia in primary biliary cirrhosis. Hepatology. 1986 May-Jun. 6(3):385-91. [Medline].

Nikolaos T Pyrsopoulos, MD, PhD, MBA, FACP, AGAF Chief of Gastroenterology and Hepatology, Medical Director of Liver Transplantation, Division of Gastroenterology and Hepatology, Rutgers New Jersey Medical School

Nikolaos T Pyrsopoulos, MD, PhD, MBA, FACP, AGAF is a member of the following medical societies: American Association for the Study of Liver Diseases, American College of Gastroenterology, American College of Physicians, American Society for Gastrointestinal Endoscopy, American Society of Transplantation, American Liver Foundation, International Liver Transplantation Society, Transplantation Society, American Gastroenterological Association, American Medical Association

Disclosure: Received consulting fee from Gilead Sciences for consulting.

K Rajender Reddy, MD, FACP, FACG Professor of Medicine, Director of Hepatology, Medical Director of Liver Transplantation, Hospital of the University of Pennsylvania

K Rajender Reddy, MD, FACP, FACG is a member of the following medical societies: American Association for the Study of Liver Diseases, American College of Gastroenterology, American College of Physicians, American Gastroenterological Association, American Society for Gastrointestinal Endoscopy, Florida Medical Association

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Received salary from Medscape for employment. for: Medscape.

BS Anand, MD Professor, Department of Internal Medicine, Division of Gastroenterology, Baylor College of Medicine

BS Anand, MD is a member of the following medical societies: American Association for the Study of Liver Diseases, American College of Gastroenterology, American Gastroenterological Association, American Society for Gastrointestinal Endoscopy

Disclosure: Nothing to disclose.

George Y Wu, MD, PhD Professor, Department of Medicine, Director, Hepatology Section, Herman Lopata Chair in Hepatitis Research, University of Connecticut School of Medicine

George Y Wu, MD, PhD is a member of the following medical societies: American Association for the Study of Liver Diseases, American Gastroenterological Association, American Medical Association, American Society for Clinical Investigation, Association of American Physicians

Disclosure: Nothing to disclose.