Skip to main content
SpringerLink
Log in

Liver Involvement in Sjögren’s Syndrome

  • Chapter
  • First Online:
Sjögren’s Syndrome

  • 1245 Accesses

Abstract

Liver involvement in Sjögren’s syndrome (SS) is commonly subclinical and almost never appears as the first manifestation of the disease. The prevalence of liver involvement in SS varies among series and depends on the diagnostic criteria used. Studies conducted in the 1960s, based on clinical (i.e., liver enlargement) and serological (i.e., elevated alkaline phosphatase) findings estimated the SS liver involvement as high as 20% [1]. A somewhat lower prevalence of liver disease has been reported in more recent studies, with estimates ranging from 2% to 20% of SS patients [2–6].

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

Access this chapter

Log in via an institution
Chapter
USD 29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD 84.99
Price excludes VAT (USA)
  • Available as EPUB and PDF
  • Read on any device
  • Instant download
  • Own it forever
Softcover Book
USD 139.00
Price excludes VAT (USA)
  • Compact, lightweight edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info
Hardcover Book
USD 109.99
Price excludes VAT (USA)
  • Durable hardcover edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info

Tax calculation will be finalised at checkout

Purchases are for personal use only

Institutional subscriptions

References

  1. Bloch KJ, Buchanan WW, Wohl MJ, et al. Sjoegren’s syndrome. A clinical, pathological and serological study of sixty-two cases. Medicine (Baltimore). 1965;44:187–231.

    Article  CAS  Google Scholar 

  2. Skopouli FN, Barbatis C, Moutsopoulos HM. Liver involvement in primary Sjögren’s ­syndrome. Br J Rheumatol. 1994;33(8):745–8.

    Article  PubMed  CAS  Google Scholar 

  3. Csepregi A, Szodoray P, Zeher M. Do autoantibodies predict autoimmune liver disease in primary Sjögren’s syndrome? Data of 180 patients upon a 5 year follow-up. Scand J Immunol. 2002;56(6):623–9.

    Article  PubMed  CAS  Google Scholar 

  4. Kaplan MJ, Ike RW. The liver is a common non-exocrine target in primary Sjögren’s ­syndrome: a retrospective review. BMC Gastroenterol. 2002;2:21.

    Article  PubMed  Google Scholar 

  5. Lindgren S, Manthorpe R, Eriksson S. Autoimmune liver disease in patients with primary Sjögren’s syndrome. J Hepatol. 1994;20(3):354–8.

    Article  PubMed  CAS  Google Scholar 

  6. Hatzis GS, Fragoulis GE, Karatzaferis A, et al. Prevalence and longterm course of primary biliary cirrhosis in primary Sjögren’s syndrome. J Rheumatol. 2008;35(10):2012–6.

    PubMed  Google Scholar 

  7. Whaley K, Goudie RB, Williamson J, et al. Liver disease in Sjögren’s syndrome and rheumatoid arthritis. Lancet. 1970;1(7652):861–3.

    Article  PubMed  CAS  Google Scholar 

  8. Zeher M, Szodoray P. Sjogren’s syndrome and associated disorders. Published by Research Signpost, 2009.

    Google Scholar 

  9. Kaplan MM, Gershwin ME. Primary biliary cirrhosis. N Engl J Med. 2005;353(12):1261–73.

    Article  PubMed  CAS  Google Scholar 

  10. Invernizzi P, Crosignani A, Battezzati PM, et al. Comparison of the clinical features and clinical course of antimitochondrial antibody-positive and -negative primary biliary cirrhosis. Hepatology. 1997;25(5):1090–5.

    Article  PubMed  CAS  Google Scholar 

  11. Tsianos EV, Hoofnagle JH, Fox PC, et al. Sjögren’s syndrome in patients with primary biliary cirrhosis. Hepatology. 1990;11(5):730–4.

    Article  PubMed  CAS  Google Scholar 

  12. Uddenfeldt P, Danielsson A, Forssell A, et al. Features of Sjögren’s syndrome in patients with primary biliary cirrhosis. J Intern Med. 1991;230(5):443–8.

    Article  PubMed  CAS  Google Scholar 

  13. Andonopoulos AP, Drosos AA, Skopouli FN, et al. Secondary Sjögren’s syndrome in rheumatoid arthritis. J Rheumatol. 1987;14(6):1098–103.

    PubMed  CAS  Google Scholar 

  14. Rigamonti C, Shand LM, Feudjo M, et al. Clinical features and prognosis of primary biliary cirrhosis associated with systemic sclerosis. Gut. 2006;55(3):388–94.

    Article  PubMed  CAS  Google Scholar 

  15. Moutsopoulos HM, Kordossis T. Sjögren’s syndrome revisited: autoimmune epithelitis. Br J Rheumatol. 1996;35(3):204–6.

    Article  PubMed  CAS  Google Scholar 

  16. Shimoda S, Harada K, Niiro H, et al. Biliary epithelial cells and primary biliary cirrhosis: the role of liver-infiltrating mononuclear cells. Hepatology. 2008;47:958–65.

    Article  PubMed  CAS  Google Scholar 

  17. Lleo A, Selmi C, Invernizzi P, et al. The consequences of apoptosis in autoimmunity. J Autoimmun. 2008;31:257–62.

    Article  PubMed  CAS  Google Scholar 

  18. Odin JA, Huebert RC, Casciola-Rosen L, et al. Bcl-2-dependent oxidation of pyruvate ­dehydrogenase-e2, a primary biliary cirrhosis autoantigen, during apoptosis. J Clin Invest. 2001;108:223–32.

    PubMed  CAS  Google Scholar 

  19. Lleo A, Selmi C, Invernizzi P, et al. Apotopes and the biliary specificity of primary biliary ­cirrhosis. Hepatology. 2009;49:871–9.

    Article  PubMed  CAS  Google Scholar 

  20. Wakabayashi K, Yoshida K, Leung PS, et al. Induction of autoimmune cholangitis in non-obese diabetic (NOD).1101 mice following a chemical xenobiotic immunization. Clin Exp Immunol. 2009;155(3):577–86.

    Article  PubMed  CAS  Google Scholar 

  21. Cha S, Nagashima H, Brown VB, et al. Two NOD Idd-associated intervals contribute synergistically to the development of autoimmune exocrinopathy (Sjögren’s syndrome) on a healthy murine background. Arthritis Rheum. 2002;46(5):1390–8.

    Article  PubMed  CAS  Google Scholar 

  22. Oertelt S, Lian ZX, Cheng CM, et al. Anti-mitochondrial antibodies and primary biliary ­cirrhosis in tgf-beta receptor ii dominant-negative mice. J Immunol. 2006;177:1655–60.

    PubMed  CAS  Google Scholar 

  23. Moritoki Y, Zhang W, Tsuneyama K, et al. B cells suppress the inflammatory response in a mouse model of primary biliary cirrhosis. Gastroenterology. 2009;136:1037–47.

    Article  PubMed  CAS  Google Scholar 

  24. Wakabayashi K, Lian ZX, Moritoki Y, et al. Il-2 receptor alpha(−/−) mice and the development of primary biliary cirrhosis. Hepatology. 2006;44:1240–9.

    Article  PubMed  CAS  Google Scholar 

  25. Wakabayashi K, Lian ZX, Leung PS, et al. Loss of tolerance in c57bl/6 mice to the autoantigen e2 subunit of pyruvate dehydrogenase by a xenobiotic with ensuing biliary ductular disease. Hepatology. 2008;48:531–40.

    Article  PubMed  CAS  Google Scholar 

  26. Mattner J, Savage PB, Leung P, et al. Liver autoimmunity triggered by microbial activation of natural killer T cells. Cell Host Microbe. 2008;3:304–15.

    Article  PubMed  CAS  Google Scholar 

  27. Abraham S, Begum S, Isenberg D. Hepatic manifestations of autoimmune rheumatic diseases. Ann Rheum Dis. 2004;63(2):123–9.

    Article  PubMed  CAS  Google Scholar 

  28. Christodoulou MI, Kapsogeorgou EK, Moutsopoulos HM. Characteristics of the minor ­salivary gland infiltrates in Sjögren’s syndrome. J Autoimmun. 2010;34(4):400–7.

    Article  PubMed  CAS  Google Scholar 

  29. Christodoulou MI, Kapsogeorgou EK, Moutsopoulos NM, et al. Foxp3+ T-regulatory cells in Sjogren’s syndrome: correlation with the grade of the autoimmune lesion and certain adverse prognostic factors. Am J Pathol. 2008;173(5):1389–96.

    Article  PubMed  Google Scholar 

  30. Tishler M, Alosachie I, Barka N, et al. Primary Sjögren’s syndrome and primary biliary cirrhosis: differences and similarities in the autoantibody profile. Clin Exp Rheumatol. 1995;13(4):497–500.

    PubMed  CAS  Google Scholar 

  31. Invernizzi P, Selmi C, Ranftler C, et al. Antinuclear antibodies in primary biliary cirrhosis. Semin Liver Dis. 2005;25:298–310.

    Article  PubMed  Google Scholar 

  32. Zurgil N, Bakimer R, Moutsopoulos HM, et al. Antimitochondrial (pyruvate dehydrogenase) autoantibodies in autoimmune rheumatic diseases. J Clin Immunol. 1992;12(3):201–9.

    Article  PubMed  CAS  Google Scholar 

  33. Vergani D, Longhi MS, Bogdanos DP, et al. Autoimmune hepatitis. Semin Immunopathol. 2009;31(3):421–35. Epub 2009 Jun 17.

    Article  PubMed  CAS  Google Scholar 

  34. Krawitt EL. Clinical features and management of autoimmune hepatitis. World J Gastroenterol. 2008;14(21):3301–5.

    Article  PubMed  CAS  Google Scholar 

  35. Ishibashi H, Komori A, Shimoda S, et al. Guidelines for therapy of autoimmune liver disease. Semin Liver Dis. 2007;27(2):214–26.

    Article  PubMed  CAS  Google Scholar 

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

    Article  PubMed  CAS  Google Scholar 

  37. Haddad J, Deny P, Munz-Gotheil C, et al. Lymphocytic sialadenitis of Sjögren’s syndrome associated with chronic hepatitis C virus liver disease. Lancet. 1992;339(8789):321–3.

    Article  PubMed  CAS  Google Scholar 

  38. Pawlotsky JM, Ben Yahia M, Andre C, et al. Immunological disorders in C virus chronic active hepatitis: a prospective case-control study. Hepatology. 1994;19(4):841–8.

    Article  PubMed  CAS  Google Scholar 

  39. Scott CA, Avellini C, Desinan L, et al. Chronic lymphocytic sialoadenitis in HCV-related chronic liver disease: comparison of Sjögren’s syndrome. Histopathology. 1997;30(1):41–8.

    Article  PubMed  CAS  Google Scholar 

  40. Koike K, Moriya K, Ishibashi K, et al. Sialadenitis histologically resembling Sjogren syndrome in mice transgenic for hepatitis C virus envelope genes. Proc Natl Acad Sci USA. 1997;94(1):233–6.

    Article  PubMed  CAS  Google Scholar 

  41. Loustaud-Ratti V, Riche A, Liozon E, et al. Prevalence and characteristics of Sjögren’s ­syndrome or Sicca syndrome in chronic hepatitis C virus infection: a prospective study. J Rheumatol. 2001;28(10):2245–51.

    PubMed  CAS  Google Scholar 

  42. García-Carrasco M, Ramos M, Cervera R, et al. Hepatitis C virus infection in ‘primary’ Sjögren’s syndrome: prevalence and clinical significance in a series of 90 patients. Ann Rheum Dis. 1997;56(3):173–5.

    Article  PubMed  Google Scholar 

  43. Jorgensen C, Legouffe MC, Perney P, et al. Sicca syndrome associated with hepatitis C virus infection. Arthritis Rheum. 1996;39(7):1166–71.

    Article  PubMed  CAS  Google Scholar 

  44. Ramos-Casals M, García-Carrasco M, Cervera R, et al. Hepatitis C virus infection mimicking primary Sjögren syndrome. A clinical and immunologic description of 35 cases. Medicine (Baltimore). 2001;80(1):1–8.

    Article  CAS  Google Scholar 

  45. Ramos-Casals M, Sánchez-Tapias JM, Parés A, et al. Characterization and differentiation of autoimmune versus viral liver involvement in patients with Sjögren’s syndrome. J Rheumatol. 2006;33(8):1593–9.

    PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Department of Pathophysiology, School of Medicine, National University of Athens, Athens, Greece

    George E. Fragoulis

  2. Department of Dietetics and Nutritional Science, Harokopio University of Athens, Athens, Greece

    Fotini N. Skopouli

  3. Division of Rheumatology, Allergy and Clinical Immunology, University of California at Davis, Davis, CA, USA

    Carlo Selmi

  4. Department of Translational Medicine, IRCCS Istituto Clinico Humanitas, University of Milan, Rozzano (MI), Italy

    Carlo Selmi

  5. Division of Rheumatology, Allergy and Clinical Immunology, Genome and Biomedical Sciences Facility, University of California at Davis, Davis, CA, USA

    M. Eric Gershwin

Authors
  1. George E. Fragoulis
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Fotini N. Skopouli
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Carlo Selmi
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. M. Eric Gershwin
    View author publications

    You can also search for this author in PubMed Google Scholar

Editor information

Editors and Affiliations

  1. Barcelona, Surgey, Hospital Clinic, Calle Villarroel, 170, Barcelona, 08036, Spain

    Manuel Ramos-Casals

  2. Massachusetts General Hospital, Boston, 02114, Massachusetts, USA

    John H. Stone

  3. National University of Athens, Athens, Greece

    Haralampos M. Moutsopoulos

Rights and permissions

Reprints and permissions

Copyright information

© 2011 Springer-Verlag London Limited

About this chapter

Cite this chapter

Fragoulis, G.E., Skopouli, F.N., Selmi, C., Gershwin, M.E. (2011). Liver Involvement in Sjögren’s Syndrome. In: Ramos-Casals, M., Stone, J., Moutsopoulos, H. (eds) Sjögren’s Syndrome. Springer, London. https://doi.org/10.1007/978-0-85729-947-5_17

Download citation

  • DOI: https://doi.org/10.1007/978-0-85729-947-5_17

  • Published:

  • Publisher Name: Springer, London

  • Print ISBN: 978-0-85729-946-8

  • Online ISBN: 978-0-85729-947-5

  • eBook Packages: MedicineMedicine (R0)

Publish with us

Policies and ethics

Search

Navigation