Skip to main content

Advertisement

Log in

Update on the diagnosis and treatment of autoimmune pancreatitis

  • Published:
Current Gastroenterology Reports Aims and scope Submit manuscript

Abstract

Autoimmune pancreatitis (AIP) is the pancreatic manifestation of a systemic fibroinflammatory disease (IgG4-related systemic disease) in which affected organs demonstrate dense lymphoplasmacytic infiltration with abundant IgG4-positive cells. The diagnosis of AIP and its differentiation from pancreatic cancer, its main differential diagnosis, remains a clinical challenge. The five cardinal features of AIP are characteristic histology, imaging, and serology; other organ involvement; and response to steroid therapy. Recent advances in our understanding of these features have resulted in enhanced recognition and diagnosis of this benign disease. This in turn has resulted in the avoidance of unnecessary surgical procedures for suspected malignancy. This article reviews recent updates in the diagnosis and treatment of autoimmune pancreatitis.

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

Access this article

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Similar content being viewed by others

References and Recommended Reading

  1. Sarles H, Sarles JC, Muratore R, Guien C: Chronic inflammatory sclerosis of the pancreas: an autonomous pancreatic disease? Am J Dig Dis 1961, 6:688–698.

    Article  PubMed  CAS  Google Scholar 

  2. Yoshida K, Toki F, Takeuchi T, et al.: Chronic pancreatitis caused by an autoimmune abnormality. Proposal of the concept of autoimmune pancreatitis. Dig Dis Sci 1995, 40:1561–1568.

    Article  PubMed  CAS  Google Scholar 

  3. Hamano H, Kawa S, Horiuchi A, et al.: High serum IgG4 concentrations in patients with sclerosing pancreatitis. N Engl J Med 2001, 344:732–738.

    Article  PubMed  CAS  Google Scholar 

  4. Kamisawa T, Funata N, Hayashi Y, et al.: A new clinicopathological entity of IgG4-related autoimmune disease. J Gastroenterol 2003, 38:982–984.

    Article  PubMed  CAS  Google Scholar 

  5. Pickartz T, Mayerle J, Lerch MM: Autoimmune pancreatitis. Nat Clin Pract Gastroenterol Hepatol 2007, 4:314–323.

    Article  PubMed  CAS  Google Scholar 

  6. Krasinskas AM, Raina A, Khalid A, et al.: Autoimmune pancreatitis. Gastroenterol Clin North Am 2007, 36:239–257.

    Article  PubMed  Google Scholar 

  7. Toomey DP, Swan N, Torreggiani W, Conlon KC: Autoimmune pancreatitis. Br J Surg 2007, 94:1067–1074.

    Article  PubMed  CAS  Google Scholar 

  8. Finkelberg DL, Sahani D, Deshpande V, Brugge WR: Autoimmune pancreatitis. N Engl J Med 2006, 355:2670–2676.

    Article  PubMed  CAS  Google Scholar 

  9. Kwon S, Kim MH, Choi EK: The diagnostic criteria for autoimmune chronic pancreatitis: it is time to make a consensus. Pancreas 2007, 34:279–286.

    Article  PubMed  Google Scholar 

  10. Kloppel G, Luttges J, Lohr M, et al.: Autoimmune pancreatitis: pathological, clinical, and immunological features. Pancreas 2003, 27:14–19.

    Article  PubMed  Google Scholar 

  11. Weber SM, Cubukcu-Dimopulo O, Palesty JA, et al.: Lymphoplasmacytic sclerosing pancreatitis: inflammatory mimic of pancreatic carcinoma. J Gastrointest Surg 2003, 7:129–137.

    Article  PubMed  Google Scholar 

  12. Notohara K, Burgart LJ, Yadav D, et al.: Idiopathic chronic pancreatitis with periductal lymphoplasmacytic infiltration: clinicopathologic features of 35 cases. Am J Surg Pathol 2003, 27:1119–1127.

    Article  PubMed  Google Scholar 

  13. Chari ST, Smyrk TC, Levy MJ, et al.: Diagnosis of autoimmune pancreatitis: the mayo clinic experience. Clin Gastroenterol Hepatol 2006, 4:1010–1016.

    Article  PubMed  Google Scholar 

  14. Suda K, Takase M, Fukumura Y, et al.: Histopathologic characteristics of autoimmune pancreatitis based on comparison with chronic pancreatitis. Pancreas 2005, 30:355–358.

    Article  PubMed  Google Scholar 

  15. Kojima M, Sipos B, Klapper W, et al.: Autoimmune pancreatitis: frequency, IgG4 expression, and clonality of T and B cells. Am J Surg Pathol 2007, 31:521–528.

    Article  PubMed  Google Scholar 

  16. Zhang L, Notohara K, Levy MJ, et al.: IgG4-positive plasma cell infiltration in the diagnosis of autoimmune pancreatitis. Mod Pathol 2007, 20:23–28.

    Article  PubMed  CAS  Google Scholar 

  17. Deshpande V, Chicano S, Finkelberg D, et al.: Autoimmune pancreatitis: a systemic immune complex mediated disease. Am J Surg Pathol 2006, 30:1537–1545.

    Article  PubMed  Google Scholar 

  18. Deshpande V, Mino-Kenudson M, Brugge WR, et al.: Endoscopic ultrasound guided fine needle aspiration biopsy of autoimmune pancreatitis: diagnostic criteria and pitfalls. Am J Surg Pathol 2005, 29:1464–1471.

    Article  PubMed  Google Scholar 

  19. Levy MJ, Reddy RP, Wiersema MJ, et al.: EUS-guided trucut biopsy in establishing autoimmune pancreatitis as the cause of obstructive jaundice. Gastrointest Endosc 2005, 61:467–472.

    Article  PubMed  Google Scholar 

  20. Levy MJ, Wiersema MJ, Chari ST: Chronic pancreatitis: focal pancreatitis or cancer? Is there a role for FNA/biopsy? Auto-immune pancreatitis. Endoscopy 2006, 38(Suppl 1):S30–S35.

    Article  PubMed  Google Scholar 

  21. Deheragoda MG, Church NI, Rodriguez-Justo M, et al.: The use of immunoglobulin g4 immunostaining in diagnosing pancreatic and extrapancreatic involvement in autoimmune pancreatitis. Clin Gastroenterol Hepatol 2007, 5:1229–1234.

    Article  PubMed  Google Scholar 

  22. Aoki S, Nakazawa T, Ohara H, et al.: Immunohistochemical study of autoimmune pancreatitis using anti-IgG4 antibody and patients’ sera. Histopathology 2005, 47:147–158.

    Article  PubMed  CAS  Google Scholar 

  23. Kamisawa T, Okamoto A, Funata N: Clinicopathological features of autoimmune pancreatitis in relation to elevation of serum IgG4. Pancreas 2005, 31:28–31.

    Article  PubMed  CAS  Google Scholar 

  24. Takahashi N, Fletcher JG, Fidler JL, et al.: Dual-phase CT of autoimmune pancreatitis: multi-reader study. AJR Am J Roentgenology 2008, In press.

  25. Sahani DV, Kalva SP, Farrell J, et al.: Autoimmune pancreatitis: imaging features. Radiology 2004, 233:345–352.

    Article  PubMed  Google Scholar 

  26. Kamisawa T, Chen PY, Tu Y, et al.: MRCP and MRI findings in 9 patients with autoimmune pancreatitis. World J Gastroenterol 2006, 12:2919–2922.

    PubMed  Google Scholar 

  27. Farrell JJ, Garber J, Sahani D, Brugge WR: EUS findings in patients with autoimmune pancreatitis. Gastrointest Endosc 2004, 60:927–936.

    Article  PubMed  Google Scholar 

  28. Aparisi L, Farre A, Gomez-Cambronero L, et al.: Antibodies to carbonic anhydrase and IgG4 levels in idiopathic chronic pancreatitis: relevance for diagnosis of autoimmune pancreatitis. Gut 2005, 54:703–709.

    Article  PubMed  CAS  Google Scholar 

  29. Okazaki K, Uchida K, Ohana M, et al.: Autoimmune-related pancreatitis is associated with autoantibodies and a Th1/Th2-type cellular immune response. Gastroenterology 2000, 118:573–581.

    Article  PubMed  CAS  Google Scholar 

  30. Ghazale A, Chari ST, Smyrk TC, et al.: Value of serum IgG4 in the diagnosis of autoimmune pancreatitis and in distinguishing it from pancreatic cancer. Am J Gastroenterol 2007, 102:1646–1653.

    Article  PubMed  CAS  Google Scholar 

  31. Choi EK, Kim MH, Lee TY, et al.: The sensitivity and specificity of serum immunoglobulin G and immunoglobulin G4 levels in the diagnosis of autoimmune chronic pancreatitis: Korean experience. Pancreas 2007, 35:156–161.

    Article  PubMed  Google Scholar 

  32. Kamisawa T, Nakajima H, Egawa N, et al.: IgG4-related sclerosing disease incorporating sclerosing pancreatitis, cholangitis, sialadenitis and retroperitoneal fibrosis with lymphadenopathy. Pancreatology 2006, 6:132–137.

    Article  PubMed  CAS  Google Scholar 

  33. Hirano K, Shiratori Y, Komatsu Y, et al.: Involvement of the biliary system in autoimmune pancreatitis: a follow-up study. Clin Gastroenterol Hepatol 2003, 1:453–464.

    Article  PubMed  Google Scholar 

  34. Zen Y, Harada K, Sasaki M, et al.: IgG4-related sclerosing cholangitis with and without hepatic inflammatory pseudotumor, and sclerosing pancreatitis-associated sclerosing cholangitis: do they belong to a spectrum of sclerosing pancreatitis? Am J Surg Pathol 2004, 28:1193–1203.

    Article  PubMed  Google Scholar 

  35. Nakazawa T, Ohara H, Sano H, et al.: Cholangiography can discriminate sclerosing cholangitis with autoimmune pancreatitis from primary sclerosing cholangitis. Gastrointest Endosc 2004, 60:937–944.

    Article  PubMed  Google Scholar 

  36. 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, 45:1547–1554.

    Article  PubMed  Google Scholar 

  37. Takahashi N, Kawashima A, Fletcher JG, Chari ST: Renal involvement in patients with autoimmune pancreatitis: CT and MR imaging findings. Radiology 2007, 242:791–801.

    Article  PubMed  Google Scholar 

  38. Saeki T, Saito A, Yamazaki H, et al.: Tubulointerstitial nephritis associated with IgG4-related systemic disease. Clin Exp Nephrol 2007, 11:168–173.

    Article  PubMed  Google Scholar 

  39. Umemura T, Zen Y, Hamano H, et al.: Immunoglobin G4-hepatopathy: association of immunoglobin G4-bearing plasma cells in liver with autoimmune pancreatitis. Hepatology 2007, 46:463–471.

    Article  PubMed  CAS  Google Scholar 

  40. Ghazale A, Chari S: Is autoimmune pancreatitis a risk factor for pancreatic cancer? Pancreas 2007, 35:376.

    Article  PubMed  Google Scholar 

  41. Yoneda K, Murata K, Katayama K, et al.: Tubulointerstitial nephritis associated with IgG4-related autoimmune disease. Am J Kidney Dis 2007, 50:455–462.

    Article  PubMed  Google Scholar 

  42. Pearson RK, Longnecker DS, Chari ST, et al.: Controversies in clinical pancreatology: autoimmune pancreatitis: does it exist? Pancreas 2003, 27:1–13.

    Article  PubMed  Google Scholar 

  43. Kamisawa T, Egawa N, Nakajima H, et al.: Morphological changes after steroid therapy in autoimmune pancreatitis. Scand J Gastroenterol 2004, 39:1154–1158.

    Article  PubMed  CAS  Google Scholar 

  44. Kamisawa T, Okamoto A: Prognosis of autoimmune pancreatitis. J Gastroenterol 2007, 42(Suppl 18):59–62.

    Article  PubMed  CAS  Google Scholar 

  45. Wakabayashi T, Kawaura Y, Satomura Y, et al.: Long-term prognosis of duct-narrowing chronic pancreatitis: strategy for steroid treatment. Pancreas 2005, 30:31–39.

    PubMed  CAS  Google Scholar 

  46. Zamboni G, Luttges J, Capelli P, et al.: Histopathological features of diagnostic and clinical relevance in autoimmune pancreatitis: a study on 53 resection specimens and 9 biopsy specimens. Virchows Arch 2004, 445:552–563.

    Article  PubMed  Google Scholar 

  47. Ghazale A, Chari ST: Optimising corticosteroid treatment for autoimmune pancreatitis. Gut 2007, 56:1650–1652.

    Article  PubMed  CAS  Google Scholar 

  48. Kamisawa T, Yoshiike M, Egawa N, et al.: Treating patients with autoimmune pancreatitis: results from a long-term follow-up study. Pancreatology 2005, 5:234–238; discussion 238–240.

    Article  PubMed  Google Scholar 

  49. Hirano K, Tada M, Isayama H, et al.: Long-term prognosis of autoimmune pancreatitis without and with corticosteroid treatment. Gut 2007, 56:1719–1724.

    Article  PubMed  CAS  Google Scholar 

  50. Ghazale A, Chari S, Zhang L, et al.: IgG4-associated cholangitis: clinical profile and response to therapy. Gastroenterology 2008, In press.

Download references

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to Santhi Swaroop Vege.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Ghazale, A.H., Chari, S.T. & Vege, S.S. Update on the diagnosis and treatment of autoimmune pancreatitis. Curr Gastroenterol Rep 10, 115–121 (2008). https://doi.org/10.1007/s11894-008-0031-x

Download citation

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s11894-008-0031-x

Keywords

Navigation