IgG4-Related Sclerosing Cholangitis in Europe

  • Nicolò de Pretis
  • Antonio Amodio
  • Giulia De Marchi
  • Luca FrulloniEmail author


IgG4-related sclerosing cholangitis (IgG4-RC) is a clinical condition described in the last 10 years, according to the growing knowledge on IgG4-related diseases (IgG4-RD). In Europe, few studies have been published on IgG4-RC and the information available are mainly derived from patients suffering from autoimmune pancreatitis (AIP). The chapter report the knowledge about the disease in Europe, and diagnostic algorytm and therapy currently applied for this rare but challenging disease.


  1. 1.
    Stone JH, Zen Y, Deshpande V. IgG4-related disease. N Engl J Med. 2012;366(6):539–51.CrossRefPubMedGoogle Scholar
  2. 2.
    Kamisawa T, Funata N, Hayashi Y, Eishi Y, Koike M, Tsuruta K, et al. A new clinicopathological entity of IgG4-related autoimmune disease. J Gastroenterol. 2003;38(10):982–4.CrossRefPubMedGoogle Scholar
  3. 3.
    Ikeura T, Manfredi R, Zamboni G, Negrelli R, Capelli P, Amodio A, et al. Application of international consensus diagnostic criteria to an Italian series of autoimmune pancreatitis. United European Gastroenterol J. 2013;1(4):276–84.CrossRefPubMedPubMedCentralGoogle Scholar
  4. 4.
    Maire F, Le Baleur Y, Rebours V, Vullierme MP, Couvelard A, Voitot H, et al. Outcome of patients with type 1 or 2 autoimmune pancreatitis. Am J Gastroenterol. 2011;106(1):151–6.CrossRefPubMedGoogle Scholar
  5. 5.
    Huggett MT, Culver EL, Kumar M, Hurst JM, Rodriguez-Justo M, Chapman MH, et al. Type 1 autoimmune pancreatitis and IgG4-related sclerosing cholangitis is associated with extrapancreatic organ failure, malignancy, and mortality in a prospective UK cohort. Am J Gastroenterol. 2014;109(10):1675–83.CrossRefPubMedPubMedCentralGoogle Scholar
  6. 6.
    van Heerde MJ, Buijs J, Rauws EA, de Buy Wenniger LJ, Hansen BE, Biermann K, et al. A comparative study of diagnostic scoring systems for autoimmune pancreatitis. Pancreas. 2014;43(4):559–64.CrossRefPubMedGoogle Scholar
  7. 7.
    Manfredi R, Frulloni L, Mantovani W, Bonatti M, Graziani R, Pozzi Mucelli R. Autoimmune pancreatitis: pancreatic and extrapancreatic MR imaging-MR cholangiopancreatography findings at diagnosis, after steroid therapy, and at recurrence. Radiology. 2011;260(2):428–36.CrossRefPubMedGoogle Scholar
  8. 8.
    Kamisawa T, Kim MH, Liao WC, Liu Q, Balakrishnan V, Okazaki K, et al. Clinical characteristics of 327 Asian patients with autoimmune pancreatitis based on Asian diagnostic criteria. Pancreas. 2011;40(2):200–5.CrossRefPubMedGoogle Scholar
  9. 9.
    Ghazale A, Chari ST, Zhang L, Smyrk TC, Takahashi N, Levy MJ, et al. Immunoglobulin G4-associated cholangitis: clinical profile and response to therapy. Gastroenterology. 2008;134(3):706–15.CrossRefPubMedGoogle Scholar
  10. 10.
    Bjornsson E, Chari ST, Smyrk TC, Lindor K. Immunoglobulin G4 associated cholangitis: description of an emerging clinical entity based on review of the literature. Hepatology. 2007;45(6):1547–54.CrossRefPubMedGoogle Scholar
  11. 11.
    Shimosegawa T, Chari ST, Frulloni L, Kamisawa T, Kawa S, Mino-Kenudson M, et al. International consensus diagnostic criteria for autoimmune pancreatitis: guidelines of the International Association of Pancreatology. Pancreas. 2011;40(3):352–8.CrossRefPubMedGoogle Scholar
  12. 12.
    Deshpande V, Zen Y, Chan JK, Yi EE, Sato Y, Yoshino T, et al. Consensus statement on the pathology of IgG4-related disease. Mod Pathol. 2012;25(9):1181–92.CrossRefPubMedGoogle Scholar
  13. 13.
    Okazaki K, Umehara H. Are classification criteria for IgG4-RD now possible? The concept of IgG4-related disease and proposal of comprehensive diagnostic criteria in Japan. Int J Rheumatol. 2012;2012:357071.CrossRefPubMedPubMedCentralGoogle Scholar
  14. 14.
    Khosroshahi A, Wallace ZS, Crowe JL, Akamizu T, Azumi A, Carruthers MN, et al. International consensus guidance statement on the management and treatment of IgG4-related disease. Arthritis Rheumatol. 2015;67(7):1688–99.CrossRefPubMedGoogle Scholar
  15. 15.
    Buijs J, van Heerde MJ, Rauws EA, de Buy Wenniger LJ, Hansen BE, Biermann K, et al. Comparable efficacy of low- versus high-dose induction corticosteroid treatment in autoimmune pancreatitis. Pancreas. 2014;43(2):261–7.CrossRefPubMedGoogle Scholar
  16. 16.
    Bi Y, Hart PA, Law R, Clain JE, Farnell MB, Gleeson FC, et al. Obstructive jaundice in autoimmune pancreatitis can be safely treated with corticosteroids alone without biliary stenting. Pancreatology. 2016;16(3):391–6.CrossRefPubMedGoogle Scholar
  17. 17.
    Masamune A, Nishimori I, Kikuta K, Tsuji I, Mizuno N, Iiyama T, et al. Randomised controlled trial of long-term maintenance corticosteroid therapy in patients with autoimmune pancreatitis. Gut. 2017;66(3):487–94.CrossRefPubMedGoogle Scholar
  18. 18.
    Hart PA, Topazian MD, Witzig TE, Clain JE, Gleeson FC, Klebig RR, et al. Treatment of relapsing autoimmune pancreatitis with immunomodulators and rituximab: the Mayo Clinic experience. Gut. 2013;62(11):1607–15.CrossRefPubMedGoogle Scholar
  19. 19.
    Carruthers MN, Topazian MD, Khosroshahi A, Witzig TE, Wallace ZS, Hart PA, et al. Rituximab for IgG4-related disease: a prospective, open-label trial. Ann Rheum Dis. 2015;74(6):1171–7.CrossRefPubMedGoogle Scholar
  20. 20.
    de Pretis N, Amodio A, Bernardoni L, Campagnola P, Capuano F, Chari ST, et al. Azathioprine maintenance therapy to prevent relapses in autoimmune pancreatitis. Clin Transl Gastroenterol. 2017;8(4):e90.CrossRefPubMedPubMedCentralGoogle Scholar

Copyright information

© Springer Science+Business Media Singapore 2019

Authors and Affiliations

  • Nicolò de Pretis
    • 1
  • Antonio Amodio
    • 1
  • Giulia De Marchi
    • 1
  • Luca Frulloni
    • 1
    Email author
  1. 1.University of VeronaVeronaItaly

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