Autoimmune pancreatitis

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Autoimmune pancreatitis is a rare systemic fibrotic inflammatory disorder that can affect organs such as the bile ducts, salivary glands, and retroperitoneal lymph nodes, in addition to the pancreas. Morphological characteristics of autoimmune pancreatitis include a diffusely enlarged 'sausage-shaped' pancreas and an irregularly narrowed duct of Wirsung. According to the revised Japan Pancreas Society criteria, the diagnosis of autoimmune pancreatitis requires that one or more secondary serologic or histologic criteria are also met: the presence of autoantibodies, elevated levels of γ-globulins, IgG or IgG4, a lymphoplasmacytic infiltrate, or pancreatic fibrosis. The presence in any affected organ of a lymphoplasmacytic inflammatory infiltrate containing greater than 10 IgG4-positive cells per high-power field is pathognomonic for autoimmune pancreatitis. Precise data on the incidence and prevalence of autoimmune pancreatitis are currently not available because most reports involve either limited patient series or resection specimen cohorts. New diagnostic tools and further studies of the underlying pathophysiology and prognosis of autoimmune pancreatitis are needed for adequate and effective treatment strategies to be developed. The most crucial issue when caring for patients with suspected autoimmune pancreatitis is to differentiate autoimmune pancreatitis from pancreatic carcinoma, because pancreatic carcinoma requires surgery, whereas autoimmune pancreatitis responds well to steroid treatment.

Key Points

  • The appearance of autoimmune pancreatitis (AIP) on imaging studies can mimic that of pancreatic cancer

  • At present, diagnostic criteria for AIP are constantly being updated to take new findings into account and are far from being firmly established

  • Data indicate that the autoantibody profile of AIP might be very different in patients from the West compared with those from Japan and Korea

  • Endoscopic ultrasound-guided Tru-cut® biopsy seems to be more effective than fine-needle aspiration cytology as an aid to the diagnosis of AIP, but it is not universally available

  • AIP responds well to conventional steroid therapy, but no controlled treatment trials are currently available to help predict whether a patient is suitable for, and likely respond to, therapy

  • International patient registries and further controlled studies are needed to raise awareness of AIP and to collect data on the course of the disease and its possible long-term complications

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Figure 1: Imaging and histologic features associated with autoimmune pancreatitis
Figure 2: An algorithm for the diagnosis and subsequent therapy of patients with suspected AIP used at Greifswald University Hospital


  1. 1

    Sarles H et al. (1961) Chronic inflammatory sclerosis of the pancreas an autonomous pancreatic disease? Am J Dig Dis 6: 688–689

  2. 2

    Yoshida K et al. (1995) Chronic pancreatitis caused by an autoimmune abnormality. Proposal of the concept of autoimmune pancreatitis. Dig Dis Sci 40: 1561–1568

  3. 3

    Members of the Criteria Committee for Autoimmune Pancreatitis of the Japan Pancreas Society (2002) Diagnostic criteria for autoimmune pancreatitis by the Japan Pancreas Society. J Jpn Pan Soc 17: 585–587

  4. 4

    Okazaki K et al. (2006) Clinical diagnostic criteria of autoimmune pancreatitis: revised proposal. J Gastroenterol 41: 626–631

  5. 5

    Choi EK et al. (2006) The Japanese diagnostic criteria for autoimmune chronic pancreatitis: is it completely satisfactory? Diagnostic criteria for autoimmune chronic pancreatitis revisited. Pancreas 33: 13–19

  6. 6

    Kim KP et al. (2006) Diagnostic criteria for autoimmune chronic pancreatitis revisited. World J Gastroenterol 12: 2487–2496

  7. 7

    Levy MJ et al. (2006) Chronic pancreatitis: focal pancreatitis or cancer? Is there a role for FNA/biopsy? Autoimmune pancreatitis. Endoscopy 38 (Suppl 1): S30–S35

  8. 8

    Kim KP et al. (2004) Autoimmune chronic pancreatitis. Am J Gastroenterol 99: 1605–1616

  9. 9

    Okazaki K et al. (2003) Autoimmune pancreatitis is increasing in Japan. Autoimmune related pancreatitis. Gastroenterology 125: 1557–1558

  10. 10

    Okazaki K and Chiba T (2002) Autoimmune related pancreatitis. Gut 51: 1–4

  11. 11

    Zamboni G et al. (2004) Histopathological features of diagnostic and clinical relevance in autoimmune pancreatitis: a study on 53 resection specimens and 9 biopsy specimens. Virchows Arch 445: 552–563

  12. 12

    Notohara K et al. (2003) Idiopathic chronic pancreatitis with periductal lymphoplasmacytic infiltration: clinicopathologic features of 35 cases. Am J Surg Pathol 27: 1119–1127

  13. 13

    Wakabayashi T et al. (2005) Long-term prognosis of duct-narrowing chronic pancreatitis: strategy for steroid treatment. Pancreas 30: 31–39

  14. 14

    Kawa S et al. (2002) HLA DRB10405–DQB10401 haplotype is associated with autoimmune pancreatitis in the Japanese population. Gastroenterology 122: 1264–1269

  15. 15

    Umemura T et al. (2006) Genetic association of Fc receptor-like 3 polymorphisms with autoimmune pancreatitis in Japanese patients. Gut 55: 1367–1368

  16. 16

    Hamano H et al. (2001) High serum IgG4 concentrations in patients with sclerosing pancreatitis. N Engl J Med 344: 732–738

  17. 17

    Aoki S et al. (2005) Immunohistochemical study of autoimmune pancreatitis using anti-IgG4 antibody and patients' sera. Histopathology 47: 147–158

  18. 18

    Asada M et al. (2006) Identification of a novel autoantibody against pancreatic secretory trypsin inhibitor in patients with autoimmune pancreatitis. Pancreas 33: 20–26

  19. 19

    Okazaki K et al. (2000) Autoimmune-related pancreatitis is associated with autoantibodies and a TH1/TH2-type cellular immune response. Gastroenterology 118: 573–581

  20. 20

    Uchida K et al. (2002) Experimental immune-mediated pancreatitis in neonatally thymectomized mice immunized with carbonic anhydrase II and lactoferrin. Lab Invest 82: 411–424

  21. 21

    Davidson TS et al. (2005) An experimental model of autoimmune pancreatitis in the rat. Am J Pathol 166: 729–736

  22. 22

    Takayama M et al. (2004) Recurrent attacks of autoimmune pancreatitis result in pancreatic stone formation. Am J Gastroenterol 99: 932–937

  23. 23

    Pickartz T et al. (2004) Overlap syndrome of autoimmune pancreatitis and cholangitis associated with secondary Sjögren's syndrome. Eur J Gastroenterol Hepatol 16: 1295–1299

  24. 24

    Kamisawa T et al. (2003) Close relationship between autoimmune pancreatitis and multifocal fibrosclerosis. Gut 52: 683–687

  25. 25

    Tanaka S et al. (2000) Corticosteroid-responsive diabetes mellitus associated with autoimmune pancreatitis. Lancet 356: 910–911

  26. 26

    Tanaka S et al. (2001) Evidence of primary beta-cell destruction by T-cells and beta-cell differentiation from pancreatic ductal cells in diabetes associated with active autoimmune chronic pancreatitis. Diabetes Care 24: 1661–1667

  27. 27

    Kamisawa T et al. (2006) Digestion and absorption of patients with autoimmune pancreatitis. Hepatogastroenterology 53: 138–140

  28. 28

    Okazaki K et al. (2005) Autoimmune pancreatitis: etiology, pathogenesis, clinical findings and treatment. The Japanese experience. JOP 6 (1 Suppl): 89–96

  29. 29

    Kloppel G et al. (2003) Autoimmune pancreatitis: pathological, clinical, and immunological features. Pancreas 27: 14–19

  30. 30

    Kamisawa T et al. (2006) Autoimmune pancreatitis metachronously associated with retroperitoneal fibrosis with IgG4-positive plasma cell infiltration. World J Gastroenterol 12: 2955–2957

  31. 31

    Yoshimura Y et al. (2006) IgG4-associated prostatitis complicating autoimmune pancreatitis. Intern Med 45: 897–901

  32. 32

    Wakabayashi T et al. (1998) Chronic pancreatitis with diffuse irregular narrowing of the main pancreatic duct. Dig Dis Sci 43: 2415–2425

  33. 33

    Ozden I et al. (2005) Spontaneous regression of a pancreatic head mass and biliary obstruction due to autoimmune pancreatitis. Pancreatology 5: 300–303

  34. 34

    Inoue H et al. (2006) A case of pancreas cancer with autoimmune pancreatitis. Pancreas 33: 208–209

  35. 35

    Lowenfels AB et al. (1994) Prognosis of chronic pancreatitis: an international multicenter study. International Pancreatitis Study Group. Am J Gastroenterol 89: 1467–1471

  36. 36

    Lowenfels AB et al. (1999) Racial factors and the risk of chronic pancreatitis. Am J Gastroenterol 94: 790–794

  37. 37

    Sarles H et al. (1965) Observations on 205 confirmed cases of acute pancreatitis, recurring pancreatitis, and chronic pancreatitis. Gut 6: 545–559

  38. 38

    Sahani DV et al. (2004) Autoimmune pancreatitis: imaging features. Radiology 233: 345–352

  39. 39

    Shin HJ et al. (2002) Endoscopic ultrasound-guided fine-needle aspiration in 179 cases: the MD Anderson Cancer Center experience. Cancer 96: 174–180

  40. 40

    Weber SM et al. (2003) Lymphoplasmacytic sclerosing pancreatitis: inflammatory mimic of pancreatic carcinoma. J Gastrointest Surg 7: 129–139

  41. 41

    Farrell JJ et al. (2004) EUS findings in patients with autoimmune pancreatitis. Gastrointest Endosc 60: 927–936

  42. 42

    Hamano H et al. (2002) Hydronephrosis associated with retroperitoneal fibrosis and sclerosing pancreatitis. Lancet 359: 1403–1404

  43. 43

    Zhang L et al. (2007) IgG4-positive plasma cell infiltration in the diagnosis of autoimmune pancreatitis. Mod Pathol 20: 23–28

  44. 44

    Deshpande V et al. (2005) Autoimmune pancreatitis: more than just a pancreatic disease? A contemporary review of its pathology. Arch Pathol Lab Med 129: 1148–1154

  45. 45

    Ectors N et al. (1997) Non-alcoholic duct destructive chronic pancreatitis. Gut 41: 263–268

  46. 46

    Scully KA et al. (2000) The characteristic appearance of non-alcoholic duct destructive chronic pancreatitis: a report of 2 cases. Arch Pathol Lab Med 124: 1535–1538

  47. 47

    Uchida K et al. (2003) Case of chronic pancreatitis involving an autoimmune mechanism that extended to retroperitoneal fibrosis. Pancreas 26: 92–94

  48. 48

    Kamisawa T et al. (2003) Clinical difficulties in the differentiation of autoimmune pancreatitis and pancreatic carcinoma. Am J Gastroenterol 98: 2694–2699

  49. 49

    Aparisi L et al. (2005) Antibodies to carbonic anhydrase and IgG4 levels in idiopathic chronic pancreatitis: relevance for diagnosis of autoimmune pancreatitis. Gut 54: 703–709

  50. 50

    Nahon Uzan K et al. (2005) Is idiopathic chronic pancreatitis an autoimmune disease. Clin Gastroenterol Hepatol 3: 903–909

  51. 51

    Sahin P et al. (2004) Autoimmune pancreatitis associated with immune-mediated inflammation of the papilla of Vater: report on two cases. Pancreas 29: 162–166

  52. 52

    Kino-Ohsaki J et al. (1996) Serum antibodies to carbonic anhydrase I and II in patients with idiopathic chronic pancreatitis and Sjögren's syndrome. Gastroenterology 110: 1579–1586

  53. 53

    Horiuchi A et al. (1998) Characteristic pancreatic duct appearance in autoimmune chronic pancreatitis: a case report and review of the Japanese literature. Am J Gastroenterol 93: 260–263

  54. 54

    Ito T et al. (1997) Autoimmune pancreatitis as a new clinical entity. Three cases of autoimmune pancreatitis with effective steroid therapy. Dig Dis Sci 42: 1458–1468

  55. 55

    Irie H et al. (1998) Autoimmune pancreatitis: CT and MR characteristics. AJR Am J Roentgenol 170: 1323–1327

  56. 56

    Nakamura H et al. (2006) A case of IgG4-related autoimmune disease with multiple organ involvement. Scand J Rheumatol 35: 69–71

  57. 57

    Chari ST et al. (2006) Diagnosis of autoimmune pancreatitis: the Mayo Clinic experience. Clin Gastroenterol Hepatol 4: 1010–1016

  58. 58

    Kamisawa T and Okamoto A (2006) Autoimmune pancreatitis: proposal of IgG4-related sclerosing disease. J Gastroenterol 41: 613–625

  59. 59

    Abraham SC et al. (2003) Eosinophilic pancreatitis and increased eosinophils in the pancreas. Am J Surg Pathol 27: 334–342

  60. 60

    Abraham SC et al. (2003) Pancreaticoduodenectomy (Whipple resections) in patients without malignancy: are they all 'chronic pancreatitis'? Am J Surg Pathol 27: 110–120

  61. 61

    Hardacre JM et al. (2003) Results of pancreaticoduodenectomy for lymphoplasmacytic sclerosing pancreatitis. Ann Surg 237: 853–859

  62. 62

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

  63. 63

    Kamisawa T et al. (2006) Usefulness of biopsying the major duodenal papilla to diagnose autoimmune pancreatitis: a prospective study using IgG4-immunostaining. World J Gastroenterol 12: 2031–2033

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We are most grateful to Helmut Friess and Jörg Kleeff for providing us with radiographic images of autoimmune pancreatitis and to Heinz Pickartz for providing us with histology micrographs of autoimmune pancreatitis.

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Correspondence to Markus M Lerch.

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