Skip to main content

Overlap Syndromes with Autoimmune Hepatitis

  • Chapter
Cholestatic Liver Disease

Part of the book series: Clinical Gastroenterology ((CG))

  • 920 Accesses

Abstract

Autoimmune hepatitis, or some features thereof, may co-exist with primary biliary cirrhosis and primary sclerosing cholangitis. However, definitions of what constitutes overlap syndrome are variable. Thus, the true prevalence of these entities is unknown, and the best management approach uncertain.

This is a preview of subscription content, log in via an institution to check access.

Access this chapter

Chapter
USD 29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD 109.00
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever

Tax calculation will be finalised at checkout

Purchases are for personal use only

Institutional subscriptions

Preview

Unable to display preview. Download preview PDF.

Unable to display preview. Download preview PDF.

References

  1. Czaja AJ. Overlap syndrome of primary biliary cirrhosis and autoimmune hepatitis: a foray across diagnostic boundaries (Editorial). J Hepatol 2006; 44: 251–252.

    Article  PubMed  Google Scholar 

  2. Levy C and Lindor KD. Primary sclerosing cholangitis: epidemiology, natural history, and prognosis (Review: 95 references). Semin. Liv Dis 2006; 26: 22–30.

    Article  Google Scholar 

  3. Czaja AJ. and Freese DK. Diagnosis and treatment of autoimmune hepatitis (Review: 216 references). Hepatology 2002; 36: 479–497.

    Article  PubMed  Google Scholar 

  4. Czaja AJ, Bianchi FB, Carpenter HA, et al. Treatment challenges and investigational opportunities in autoimmune hepatitis. Hepatology 2005; 41: 207–215.

    Article  PubMed  Google Scholar 

  5. Krawitt EL. Autoimmune hepatitis (Review: 100 references). N Engl J Med. 2006; 354: 54–66.

    Article  CAS  PubMed  Google Scholar 

  6. Heathcote EJ. Management of primary biliary cirrhosis (Review: 105 references). Hepatology 2000; 31: 1005–1013.

    Article  CAS  PubMed  Google Scholar 

  7. Kaplan MM and Gershwin ME. Primary biliary cirrhosis (Review: 98 references). N Engl J Med 2005; 353: 1261–1273.

    Article  CAS  PubMed  Google Scholar 

  8. Woodward J and Neuberger J. Autoimmune overlap syndromes (Review: 112 references). Hepatology 2001; 33: 994–1002.

    Article  CAS  PubMed  Google Scholar 

  9. Poupon R. Autoimmune overlapping syndromes (Review: 84 references). Clin. Liver Dis 2003; 7: 865–878.

    Article  PubMed  Google Scholar 

  10. Goodman ZD, McNally PR, Davis DR, and Ishak KG. Autoimmune cholangitis: a variant of primary biliary cirrhosis. Dig Dis Sci 1995; 40: 1232–1242.

    Article  CAS  PubMed  Google Scholar 

  11. Lacerda MA, Ludwig J, Dickson ER, Jorgensen RA, and Lindor KD. Antimitochondrial antibody-negative primary biliary cirrhosis. Am J Gastroenterol 1995; 90: 247–249.

    CAS  PubMed  Google Scholar 

  12. Brunner G and Klinge O. A cholangitis with antinuclear antibodies (immunocholangitis) resembling chronic non-suppurative destructive cholangitis. Deutsche. Med Wochenschr 1987; 112: 1454–1458.

    Article  CAS  Google Scholar 

  13. Taylor SL, Dean PJ, and Riely CA. Primary autoimmune cholangitis: an alternative to antimitochondrial antibody-negative primary biliary cirrhosis. Am J Surg Pathol 1994; 18: 91–99.

    CAS  PubMed  Google Scholar 

  14. Michieletti P, Wanless IR, Katz A, et al. Antimitochondrial antibody negative primary biliary cirrhosis: a distinct syndrome of autoimmune cholangitis. Gut 1994; 35: 260–265.

    Article  CAS  PubMed  Google Scholar 

  15. Invernizzi P, Crosignani A, Battezzan PM, et al. Comparison of the clinical features and clinical course of antimitochondrial antibody positive and negative biliary cirrhosis. Hepatology 1997; 25: 1090–1095.

    Article  CAS  PubMed  Google Scholar 

  16. Ben-Ari Z, Dhillon AP, and Sherlock S. Autoimmune cholangiopathy: part of the spectrum of autoimmune chronic active hepatitis. Hepatology 1993; 18: 10–15.

    Article  CAS  PubMed  Google Scholar 

  17. Czaja AJ, Carpenter HA, Santrach PJ, and Moore SB. Autoimmune cholangitis within the spectrum of autoimmune liver disease. Hepatology 2000; 31: 1231–1238.

    Article  CAS  PubMed  Google Scholar 

  18. James OFW. Definition and epidemiology of primary biliary cirrhosis. In: Primary Biliary Cirrhosis (Neuberger, J., ed.) Eastbourne, West End Studios, 2000 pp. 53–59.

    Google Scholar 

  19. Weisner RH, LaRusso NF, Ludwig J, and Dickson ER. Comparison of the clinicopathological features of primary sclerosing cholangitis and primary biliary cirrhosis. Gastroenterology 1985; 88: 108–114.

    Google Scholar 

  20. Metcalf JV, Mitchison HC, Palmer JM, Jones DE, Bassendine MF, and James OFW. Natural history of early primary biliary cirrhosis. Lancet 1996; 348: 1399–1402.

    Article  CAS  PubMed  Google Scholar 

  21. Van de Water J, Cooper A, Surh CD, et al. Detection of autoantibodies to recombinant mitochondrial proteins in patients with primary biliary cirrhosis. N Engl J Med 1989; 320: 1377–1380.

    PubMed  Google Scholar 

  22. Nakanuma Y, Harada K, Kaji K, et al. Clinicopathological study of primary biliary cirrhosis negative for antimitochondrial antibodies. Liver 1997; 17, 281–287.

    Article  CAS  PubMed  Google Scholar 

  23. Kitami N, Komada T, Ishii H, et al. Immunological study of anti-M2 in antimitochondrial antibody negative primary biliary cirrhosis. Intern Med 1995; 34: 496–501.

    Article  CAS  PubMed  Google Scholar 

  24. Kinoshita H, Omagari K, Whittingham S, et al. Autoimmune cholangitis and primary biliary cirrhosis—an autoimmune enigma. Liver 1999; 19: 122–128.

    Article  CAS  PubMed  Google Scholar 

  25. Nakajima M, Shimizu H, Miyazaki A, Watanabe S, Kitami N, and Sato N. Detection of IgA, IgM and IgG subclasses of anti-M2 antibody by immunoblotting in autoimmune cholangitis: is autoimmune cholangitis an early stage of primary biliary cirrhosis? J Gastroenterol 1999; 34: 607–612.

    Article  CAS  PubMed  Google Scholar 

  26. Tsuneyama K, van de Water J, van Thiel D, et al. Abnormal expression of PDC-E2 on the apical surface of biliary epithelial cells in patients with antimitochondrial antibody-negative primary biliary cirrhosis. Hepatology 1995; 22: 1440–1446.

    CAS  PubMed  Google Scholar 

  27. Romero-Gómez M, Wichmann I, Crespo J, et al. Serum immunological profile in patients with chronic autoimmune cholestasis. Am J Gastroenterol 2004; 99: 2150–2157.

    Article  PubMed  CAS  Google Scholar 

  28. Davis PA, Leung P, Manns M, et al. M4 and M9 antibodies in the overlap syndrome of primary biliary cirrhosis and chronic active hepatitis: epitopes or epiphenomena? Hepatology 1992; 16: 1128–1136.

    Article  CAS  PubMed  Google Scholar 

  29. Gordon SC, Quattrociocchi-Longe TM, Khan BA, et al. Antibodies to carbonic anhydrase in patients with immune cholangiopathies. Gastroenterology 1995; 108: 1802–1809.

    Article  CAS  PubMed  Google Scholar 

  30. Invernizzi P, Battezzati PM, Crosignani A, et al. Antibody to carbonic anhydrase II is present in primary biliary cirrhosis irrespective of antimitochondrial antibody status. Clin Exp Immunol 1998; 114: 448–454.

    Article  CAS  PubMed  Google Scholar 

  31. Akisawa N, Nishimori I, Miyaji E, et al. The ability of carbonic anhydrase II antibody to distinguish autoimmune cholangitis from primary biliary cirrhosis in Japanese patients. J Gastroenterol 1999; 34: 366–371.

    Article  CAS  PubMed  Google Scholar 

  32. Kim WR, Poterucha JJ, Jorgensen RA, et al. Does antimitochondrial antibody status affect response to treatment in patients with primary biliary cirrhosis? Outcomes of ursodeoxycholic acid therapy and liver transplantation. Hepatology 1997; 26: 22–26.

    Article  CAS  PubMed  Google Scholar 

  33. Chazouillères O, Wendum D, Serfaty L, Montembault S, Rosmorduc O, and Poupon R. Primary biliary cirrhosis—autoimmune hepatitis overlap syndrome: clinical features and response to therapy. Hepatology 1998; 28: 296–301.

    Article  PubMed  Google Scholar 

  34. Lohse AW, zum Bűschenfelde KH, Franz B, Kanzler S, and Gerken G. Characterization of the overlap syndrome of primary biliary cirrhosis (PBC) and autoimmune hepatitis: evidence for it being a hepatitic form of PBC in genetically susceptible individuals. Hepatology 1999; 29: 1078–1084.

    Article  CAS  PubMed  Google Scholar 

  35. Colombato LA, Alvarez F, Cote J, and Huet P-M. Autoimmune cholangiopathy: the result of consecutive primary biliary cirrhosis and autoimmune hepatitis? Gastroenterology 1994; 107: 1839–1843.

    CAS  PubMed  Google Scholar 

  36. Horsmans Y, Piet A, Brenard R, Rahier J, and Geubel AP. Autoimmune chronic active hepatitis responsive to immunosuppressive therapy evolving into typical primary biliary cirrhosis syndrome: a case report. J Hepatol 1994; 21: 194–198.

    Article  CAS  PubMed  Google Scholar 

  37. Chazouillères O, Wendum D, Serfaty L, Rosmorduc O, and Poupon R. Long term outcome and response to therapy of primary biliary cirrhosis—autoimmune hepatitis overlap syndrome. J Hepatol 2006; 44: 400–406.

    Article  PubMed  CAS  Google Scholar 

  38. Johnson PJ and McFarlane IG. Meeting report: International Autoimmune Hepatitis Group. Hepatology 1993; 18: 998–1005.

    Article  CAS  PubMed  Google Scholar 

  39. Alvarez F, Berg PA, Bianchi FB, et al. International Autoimmune Hepatitis Group Report: review of criteria for diagnosis of autoimmune hepatitis. J Hepatol 1999; 31: 929–938.

    Article  CAS  PubMed  Google Scholar 

  40. Talwalkar JA, Keach JC, Angulo P, and Lindor KD. Overlap of autoimmune hepatitis and primary biliary cirrhosis: an evaluation of a modified scoring system. Am J Gastroenterol 2002; 97: 1191–1197.

    Article  PubMed  Google Scholar 

  41. Joshi S, Cauch-Dudek K, Wanless IR, et al. Primary biliary cirrhosis with additional features of autoimmune hepatitis: response to therapy with ursodeoxycholic acid. Hepatology 2002; 35: 409–413.

    Article  CAS  PubMed  Google Scholar 

  42. Yamamoto K, Terada R, Okamoto R, et al. A scoring system for primary biliary cirrhosis and its application for variant forms of autoimmune liver disease. J Gastroenterol 2003; 38: 52–59.

    Article  CAS  PubMed  Google Scholar 

  43. Gheorghe L, Iacob S, Gheorghe C, et al. Frequency and predictive factors for overlap syndrome between autoimmune hepatitis and primary cholestatic liver disease. Eur J Gastroenterol Hepatol 2004; 16: 585–592.

    Article  PubMed  Google Scholar 

  44. Günsar F, Akara US, Ersöz G, Karasu Z, Yüce G, and Batur Y Clinical and biochemical features and therapy responses in primary biliary cirrhosis and primary biliary cirrhosis-autoimmune hepatitis overlap syndrome. Hepato-Gastroenterology 2002; 49: 1195–2000.

    PubMed  Google Scholar 

  45. Silveira M, Talwalkar JA, and Lindor KD. Overlap of autoimmune hepatitis and primary biliary cirrhosis: long-term outcomes (Abstract). Gastroenterology 2006; 130(Suppl 2), A–801.

    Google Scholar 

  46. Keaveny AP, Gordon FD, and Khettry U. Post-liver transplantation de novo hepatitis with overlap features. Pathology International 2005; 55: 660–664.

    Article  PubMed  Google Scholar 

  47. Minuk GY, Sutherland LR, Pappas SC, et al. Autoimmune chronic active hepatitis (lupoid hepatitis) and primary sclerosing cholangitis in two young adult females. Can J Gastroenterol 1988; 2: 22–27.

    Google Scholar 

  48. Rabinovitz M, Demetris AJ, Bou-Abboud CF, and Van Thiel DH. Simultaneous occurrence of primary sclerosing cholangitis and autoimmune chronic active hepatitis in a patient with ulcerative colitis. Dig Dis Sci 1992; 37: 1606–1611.

    Article  CAS  PubMed  Google Scholar 

  49. Perdigoto R, Carpenter HA, and Czaja AJ. Frequency and significance of chronic ulcerative colitis in severe corticosteroid-treated autoimmune hepatitis. J Hepatol 1992; 14: 325–331.

    Article  CAS  PubMed  Google Scholar 

  50. Lawrence SP, Sherman KE, Lawson JM, and Goodman ZD. A 39 year old man with chronic hepatitis. Semin Liver Dis 1994; 14: 97–105.

    Article  CAS  PubMed  Google Scholar 

  51. Wurbs D, Klein R, Terraccciano LM, Berg PA, and Bianchi LA. (1995) 28-year-old woman with a combined hepatitic/cholestatic syndrome. Hepatology 1995; 22: 1598–1605.

    Article  CAS  PubMed  Google Scholar 

  52. Gohlke F, Lohse AW, Dienes HP, et al. Evidence for an overlap syndrome of autoimmune hepatitis and primary sclerosing cholangitis. J Hepatol 1996; 24: 699–705.

    Article  CAS  PubMed  Google Scholar 

  53. Protzer U, Dienes HP, Bianchi L, et al. Post-infantile giant cell hepatitis in patients with primary sclerosing cholangitis and autoimmune hepatitis. Liver 1996; 16: 27–282.

    Google Scholar 

  54. Boberg KM, Fausa O, Haaland T, et al. Features of autoimmune hepatitis in primary sclerosing cholangitis: an evaluation of 114 primary sclerosing cholangitis patients to a scoring system for the diagnosis of autoimmune hepatitis. Hepatology 1996; 23: 1369–1376.

    Article  CAS  PubMed  Google Scholar 

  55. Luketic VAC, Gomez DA, Sanyal AJ, and Shiffman ML. An atypical presentation for primary sclerosing cholangitis. Dig Dis Sci 1997; 42: 2009–2016.

    Article  CAS  PubMed  Google Scholar 

  56. McNair ANB, Moloney M, Portmann BC, Williams R, and McFarlane IG. Autoimmune hepatitis overlapping with primary sclerosing cholangitis in five cases. Am J Gastroenterol 1998; 93: 777–784.

    Article  CAS  PubMed  Google Scholar 

  57. Czaja AJ. Frequency and nature of the variant syndromes of autoimmune liver disease. Hepatology 1998; 28: 360–365.

    Article  CAS  PubMed  Google Scholar 

  58. Koskinas J, Raptis I, Manika Z, and Hadziyannis S. Overlapping syndrome of autoimmune hepatitis and primary sclerosing cholangitis associated with pyoderma gangrenosum and ulcerative colitis. Eur J Gastroenterol Hepatol 1999; 11: 1421–1424.

    Article  CAS  PubMed  Google Scholar 

  59. van Buuren HR, van Hoogstraten HJF, Terkivatan T, Schalm SW, and Vleggaar FP. High prevalence of autoimmune hepatitis among patients with primary sclerosing cholangitis. J Hepatol 2000; 33: 543–548.

    Article  PubMed  Google Scholar 

  60. Kaya M, Angulo P, and Lindor KD. Overlap of autoimmune hepatitis and primary sclerosing cholangitis: an evaluation of a modified scoring system. J Hepatol 2000; 33: 537–542.

    Article  CAS  PubMed  Google Scholar 

  61. Griga T, Tromm A, Muller KM, and May B. Overlap syndrome between autoimmune hepatitis and primary sclerosing cholangitis in two cases. Eur J Gastroenterol Hepatol 2000; 12: 559–564.

    Article  CAS  PubMed  Google Scholar 

  62. Hatzis GS, Vassilou VA, and Delladetsima JK. Overlap syndrome of primary sclerosing cholangitis and autoimmune hepatitis. Eur J Gastroenterol Hepatol 2000; 13: 203–206.

    Article  Google Scholar 

  63. Abdo AA, Bain VG, Kichian K, and Lee SS. Evolution of autoimmune hepatitis to primary sclerosing cholangitis: a sequential syndrome. Hepatology 2002; 36: 1393–1399.

    PubMed  Google Scholar 

  64. Takiguchi J, Ohira H, and Shishido S. Autoimmune hepatitis overlapping with primary sclerosing cholangitis. Intern Med 2002; 41: 696–700.

    Article  PubMed  Google Scholar 

  65. Hong-Curtis J, Yeh MM, Jain D, and Lee JH. Rapid progression of autoimmune hepatitis in the background of primary sclerosing cholangitis. J Clin Gastroenterol 2004; 38: 906–909.

    Article  PubMed  Google Scholar 

  66. Floreani A, Rizzott ER, Ferrara F, et al. Clinical course and outcome of autoimmune hepatitis/primary sclerosing cholangitis syndrome. Am J Gastroenterol 2005; 100: 1516–1522.

    Article  PubMed  Google Scholar 

  67. Rabinovitz M. Evolution of autoimmune hepatitis to primary sclerosing cholangitis (Letter). Hepatology 2003; 33: 946–947.

    Article  Google Scholar 

  68. El-Shabrawi M, Wilkinson ML, Portmann BC, et al. Primary sclerosing cholangitis in childhood. Gastroenterology 1987; 92: 1226–1235.

    CAS  PubMed  Google Scholar 

  69. Debray D, Pariente D, Urvoas E, Hadchouel M, and Bernard O. Sclerosing cholangitis in children. J Pediatr 1994; 124: 49–56.

    Article  CAS  PubMed  Google Scholar 

  70. Wilschanski M, Chait P, Wade JA, et al. Primary sclerosing cholangitis in 32 children: clinical, laboratory, and radiographic features, with survival analysis. Hepatology 1995; 22: 1415–1422.

    CAS  PubMed  Google Scholar 

  71. Gregorio GV, Portmann B, Reid F, et al. Autoimmune hepatitis in childhood: a 20-year experience. Hepatology 1997; 25: 541–547.

    Article  CAS  PubMed  Google Scholar 

  72. Gregorio GV, Portmann B, Karani J, et al. Autoimmune hepatitis/ sclerosing cholangitis overlap syndrome in childhood: a 16-year prospective study. Hepatology 2001; 33: 544–553.

    Article  CAS  PubMed  Google Scholar 

  73. Chazouillères O. Diagnosis of primary sclerosing cholangitis—autoimmune hepatitis overlap syndrome: to score or not to score? (Editorial: 15 references). J Hepatol 2000; 33: 661–663.

    Article  PubMed  Google Scholar 

  74. Targan SR, Landers C, Vidrich A, and Czaja AJ. High-titer antineutrophil cytoplasmic antibodies in type-1 autoimmune hepatitis. Gastroenterology 1995; 108: 1159–1166.

    Article  CAS  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Editor information

Editors and Affiliations

Rights and permissions

Reprints and permissions

Copyright information

© 2008 Humana Press Inc., Totowa, NJ

About this chapter

Cite this chapter

Smith, A.D. (2008). Overlap Syndromes with Autoimmune Hepatitis. In: Lindor, K.D., Talwalkar, J.A. (eds) Cholestatic Liver Disease. Clinical Gastroenterology. Humana Press. https://doi.org/10.1007/978-1-59745-118-5_5

Download citation

  • DOI: https://doi.org/10.1007/978-1-59745-118-5_5

  • Publisher Name: Humana Press

  • Print ISBN: 978-1-58829-838-6

  • Online ISBN: 978-1-59745-118-5

  • eBook Packages: MedicineMedicine (R0)

Publish with us

Policies and ethics