EUS Features

  • Michael J. Levy
  • William R. Brugge


Autoimmune pancreatitis (AIP) is a form of pancreatitis that often demonstrates distinct clinical, serological, histological, and imaging features. A hallmark of this disorder is a rapid and dramatic response to steroid therapy. Following the first description by Sarles [1] in 1961, subsequent reports included only patients with florid disease manifestations [2–4]. As initially described, patients presented with jaundice and a pancreatic head mass that was often incorrectly diagnosed as carcinoma, thereby leading to unnecessary operative intervention. Over time there has been a greater understanding of the diverse clinical, laboratory, and histological manifestations, leading to a more comprehensive set of diagnostic criteria that now allow diagnosis of a broader spectrum of AIP patients while maintaining diagnostic specificity. It is now recognized that AIP represents only one component of a systemic fibroinflammatory disease process termed IgG4-related diseases (IgG4-RD) that also afflicts other organs including the bile duct, lungs, thyroid, retroperitoneum, lymph nodes, salivary glands, and kidneys [5–11]}. These non-pancreatic sites of disease also demonstrate lymphoplasmacytic infiltration with IgG4-positive cells and often storiform fibrosis.

The purpose of this chapter is to review the breadth of endoscopic ultrasound (EUS) findings associated with AIP and to consider the EUS features in the context of clinical, imaging, histological, and laboratory data. We also suggest a role for EUS imaging and guided tissue sampling within the diagnostic algorithm.


Pancreatic Carcinoma Lymphoplasmacytic Infiltration Japan Pancreas Society Idiopathic Duct Centric Chronic Pancreatitis Storiform Fibrosis 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.


  1. 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: 688–98.PubMedCrossRefGoogle Scholar
  2. 2.
    Yoshida K, Toki F, Takeuchi T, Watanabe S, Shiratori K, Hayashi N. Chronic pancreatitis caused by an autoimmune abnormality. Proposal of the concept of autoimmune pancreatitis. Dig Dis Sci. 1995;40:1561–8.PubMedCrossRefGoogle Scholar
  3. 3.
    Ito T, Nakano I, Koyanagi S, Miyahara T, Migita Y, Ogoshi K, Sakai H, Matsunaga S, Yasuda O, Sumii T, Nawata H. Autoimmune pancreatitis as a new clinical entity. Three cases of autoimmune pancreatitis with effective steroid therapy. Dig Dis Sci. 1997;42:1458–68.PubMedCrossRefGoogle Scholar
  4. 4.
    Okazaki K, Chiba T. Autoimmune related pancreatitis. Gut. 2002;51:1–4.PubMedCrossRefGoogle Scholar
  5. 5.
    Zhang L, Smyrk TC. Autoimmune pancreatitis and IgG4-related systemic diseases. Int J Clin Exp Pathol. 2010;3:491–504.PubMedGoogle Scholar
  6. 6.
    Maire F, Le Baleur Y, Rebours V, Vullierme MP, Couvelard A, Voitot H, Sauvanet A, Hentic O, Levy P, Ruszniewski P, Hammel P. Outcome of patients with type 1 or 2 autoimmune pancreatitis. Am J Gastroenterol. 2010;106:151–6.PubMedCrossRefGoogle Scholar
  7. 7.
    Takuma K, Kamisawa T, Tabata T, Inaba Y, Egawa N, Igarashi Y. Short-term and long-term outcomes of autoimmune pancreatitis. Eur J Gastroenterol Hepatol. 2011;23:146–52.PubMedCrossRefGoogle Scholar
  8. 8.
    Takuma K, Kamisawa T, Igarashi Y. Autoimmune pancreatitis and IgG4-related sclerosing cholangitis. Curr Opin Rheumatol. 2011;23:80–7.PubMedCrossRefGoogle Scholar
  9. 9.
    Sah RP, Chari ST. Clinical hypothyroidism in autoimmune pancreatitis. Pancreas. 2010;39:1114–6.PubMedCrossRefGoogle Scholar
  10. 10.
    Goldsmith PJ, Cockbain AJ, Smith AM. Spontaneous resolution of pulmonary nodules in autoimmune pancreatitis. Jop: J Pancreas [Electronic Resource]. 2010; 11:630–2.Google Scholar
  11. 11.
    Triantopoulou C, Malachias G, Maniatis P, Anastopoulos J, Siafas I, Papailiou J. Renal lesions associated with autoimmune pancreatitis: CT findings. Acta Radiol. 2010;51:702–7.PubMedCrossRefGoogle Scholar
  12. 12.
    Ryu JK, Chung JB, Park SW, Lee JK, Lee KT, Lee WJ, Moon JH, Cho KB, Kang DW, Hwang J-H, Yoo K-S, Yoo BM, Lee DH, Kim HK, Moon YS, Lee J, Lee HS, Choi HS, Lee SK, Kim Y-T, Kim CD, Kim SJ, Hahm JS, Yoon YB. Review of 67 patients with autoimmune pancreatitis in Korea: a multicenter nationwide study. Pancreas. 2008;37:377–85.PubMedCrossRefGoogle Scholar
  13. 13.
    Okazaki K, Kawa S, Kamisawa T, Naruse S, Tanaka S, Nishimori I, Ohara H, Ito T, Kiriyama S, Inui K, Shimosegawa T, Koizumi M, Suda K, Shiratori K, Yamaguchi K, Yamaguchi T, Sugiyama M, Otsuki M, Research Committee of Intractable Diseases of the P. Clinical diagnostic criteria of autoimmune pancreatitis: revised proposal. J Gastroenterol. 2006;41:626–31.PubMedCrossRefGoogle Scholar
  14. 14.
    Chari ST, Smyrk TC, Levy MJ, Topazian MD, Takahashi N, Zhang L, Clain JE, Pearson RK, Petersen BT, Vege SS, Farnell MB. Diagnosis of autoimmune pancreatitis: the Mayo Clinic experience. Clin Gastroenterol Hepatol. 2006;4:1010–6. quiz 934.PubMedCrossRefGoogle Scholar
  15. 15.
    Pearson RK, Longnecker DS, Chari ST, Smyrk TC, Okazaki K, Frulloni L, Cavallini G. Controversies in clinical pancreatology: autoimmune pancreatitis: does it exist? Pancreas. 2003;27:1–13.PubMedCrossRefGoogle Scholar
  16. 16.
    Society JP. Diagnostic criteria for autoimmune pancreatitis by the Japan Pancreas Society. J Jpn Pancreas Soc. 2002;17:585–7.Google Scholar
  17. 17.
    Kloppel G, Luttges J, Lohr M, Zamboni G, Longnecker D. Autoimmune pancreatitis: pathological, clinical, and immunological features. Pancreas. 2003;27:14–9.PubMedCrossRefGoogle Scholar
  18. 18.
    Chari ST, Smyrk TC, Levy MJ, Topazian MD, Takahashi N, Clain JE, Pearson RK, Petersen BT, Swaroop VS, Farnell MB. Autoimmune pancreatitis: diagnosis using histology, imaging, serology, other organ involvement and response to steroids. 2006: Pending publication.Google Scholar
  19. 19.
    Ghazale A, Chari ST, Smyrk TC, Levy MJ, Topazian MD, Takahashi N, Clain JE, Pearson RK, Pelaez-Luna M, Petersen BT, Vege SS, Farnell MB. Value of serum IgG4 in the diagnosis of autoimmune pancreatitis and in distinguishing it from pancreatic cancer. Am J Gastroenterol. 2007;102:1646–53.PubMedCrossRefGoogle Scholar
  20. 20.
    Notohara K, Burgart LJ, Yadav D, Chari S, Smyrk TC. Idiopathic chronic pancreatitis with periductal lymphoplasmacytic infiltration: clinicopathologic features of 35 cases.[see comment]. Am J Surg Pathol. 2003;27:1119–27.PubMedCrossRefGoogle Scholar
  21. 21.
    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.PubMedCrossRefGoogle Scholar
  22. 22.
    Zhang L, Notohara K, Levy MJ, Chari ST, Smyrk TC. IgG4-positive plasma cell infiltration in the diagnosis of autoimmune pancreatitis. Mod Pathol. 2007;20: 23–8.PubMedCrossRefGoogle Scholar
  23. 23.
    Ghazale A, Chari ST. Optimising corticosteroid treatment for autoimmune pancreatitis. Gut. 2007;56: 1650–2.PubMedCrossRefGoogle Scholar
  24. 24.
    Chari ST, Takahashi N, Levy MJ, Smyrk TC, Clain JE, Pearson RK, Petersen BT, Topazian MA, Vege SS. A diagnostic strategy to distinguish autoimmune pancreatitis from pancreatic cancer. Clin Gastroenterol Hepatol. 2009;7:1097–103.PubMedCrossRefGoogle Scholar
  25. 25.
    Yadav D, Notahara K, Smyrk TC, Clain JE, Pearson RK, Farnell MB, Chari ST. Idiopathic tumefactive chronic pancreatitis: clinical profile, histology, and natural history after resection. Clin Gastroenterol Hepatol. 2003;1:129–35.PubMedCrossRefGoogle Scholar
  26. 26.
    Kamisawa T, Egawa N, Nakajima H, Tsuruta K, Okamoto A, Kamata N. Clinical difficulties in the differentiation of autoimmune pancreatitis and pancreatic carcinoma. Am J Gastroenterol. 2003;98: 2694–9.PubMedCrossRefGoogle Scholar
  27. 27.
    Taniguchi T, Tanio H, Seko S, Nishida O, Inoue F, Okamoto M, Ishigami S, Kobayashi H. Autoimmune pancreatitis detected as a mass in the head of the pancreas without hypergammaglobulinemia, which relapsed after surgery: case report and review of the literature. Dig Dis Sci. 2003;48:1465–71.PubMedCrossRefGoogle Scholar
  28. 28.
    Kojima E, Kimura K, Noda Y, Kobayashi G, Itoh K, Fujita N. Autoimmune pancreatitis and multiple bile duct strictures treated effectively with steroid. J Gastroenterol. 2003;38:603–7.PubMedCrossRefGoogle Scholar
  29. 29.
    Chari ST, Smyrk TC, Takahashi N, Clain JE, Farnell MB, Levy MJ, Pearson RK, Petersen BT, Topazian M, Vege SS. Autoimmune pancreatitis: diagnosis using histology, imaging, serology, other organ involvement and response to steroids. Pancreas. 2005;31:435–6.CrossRefGoogle Scholar
  30. 30.
    Sadler R, Chapman RW, Simpson D, Soonawalla ZF, Waldegrave EL, Burden JM, Misbah SA, Ferry BL. The diagnostic significance of serum IgG4 levels in patients with autoimmune pancreatitis: a UK study. Eur J Gastroenterol Hepatol. 2010;23:139–45.CrossRefGoogle Scholar
  31. 31.
    Park SH, Kim M-H, Kim SY, Kim HJ, Moon S-H, Lee SS, Byun JH, Lee SK, Seo DW, Lee M-G. Magnetic resonance cholangiopancreatography for the diagnostic evaluation of autoimmune pancreatitis. Pancreas. 2010;39:1191–8.PubMedCrossRefGoogle Scholar
  32. 32.
    Ishigami K, Tajima T, Nishie A, Ushijima Y, Fujita N, Asayama Y, Kakihara D, Irie H, Ito T, Igarashi H, Nakamura M, Honda H. MRI findings of pancreatic lymphoma and autoimmune pancreatitis: a comparative study. Eur J Radiol. 2010;74:e22–8.PubMedCrossRefGoogle Scholar
  33. 33.
    Nishino T, Oyama H, Toki F, Shiratori K. Differentiation between autoimmune pancreatitis and pancreatic carcinoma based on endoscopic retrograde cholangiopancreatography findings. J Gastroenterol. 2010;45:988–96.PubMedCrossRefGoogle Scholar
  34. 34.
    Levy MJ, Wiersema MJ, Chari ST. Chronic pancreatitis: focal pancreatitis or cancer? Is there a role for FNA/biopsy? Autoimmune pancreatitis. Endoscopy. 2006;38 Suppl 1:S30–5.PubMedCrossRefGoogle Scholar
  35. 35.
    Finkelberg DL, Sahani D, Deshpande V, Brugge WR. Autoimmune pancreatitis. N Eng J Med. 2006;355: 2670–6.CrossRefGoogle Scholar
  36. 36.
    Farrell JJ, Garber J, Sahani D, Brugge WR. EUS findings in patients with autoimmune pancreatitis. Gastrointest Endosc. 2004;60:927–36.PubMedCrossRefGoogle Scholar
  37. 37.
    Levy MJ, Reddy RP, Wiersema MJ, Smyrk TC, Clain JE, Harewood GC, Pearson RK, Rajan E, Topazian MD, Yusuf TE, Chari ST, Petersen BT. EUS-guided trucut biopsy in establishing autoimmune pancreatitis as the cause of obstructive jaundice. Gastrointest Endosc. 2005;61:467–72.PubMedCrossRefGoogle Scholar
  38. 38.
    Zhang L, Chari ST, Levy MJ, SMyrk TC. Pancreatic IgG4 stain for diagnosing autoimmune pancreatitis (AIP) and for distinguishing AIP subtypes. Gastroenterology. 2005;128:A474.Google Scholar
  39. 39.
    Hocke M, Ignee A, Dietrich CF. Contrast-enhanced endoscopic ultrasound in the diagnosis of autoimmune pancreatitis. Endoscopy. 2011;43:163–5.PubMedCrossRefGoogle Scholar
  40. 40.
    Levy MJ, Smyrk TC, Takahashi N, Zhang L, Chari ST. Idiopathic duct-centric pancreatitis: disease description and endoscopic ultrasonography-guided trucut biopsy diagnosis. Pancreatology. 2011;11: 76–80.PubMedCrossRefGoogle Scholar
  41. 41.
    Mizuno N, Bhatia V, Hosoda W, Sawaki A, Hoki N, Hara K, Takagi T, Ko SBH, Yatabe Y, Goto H, Yamao K. Histological diagnosis of autoimmune pancreatitis using EUS-guided trucut biopsy: a comparison study with EUS-FNA. J Gastroenterol. 2009;44:742–50.PubMedCrossRefGoogle Scholar
  42. 42.
    Deshpande V, Mino-Kenudson M, Brugge WR, Pitman MB, Fernandez-del Castillo C, Warshaw AL, Lauwers GY. Endoscopic ultrasound guided fine needle aspiration biopsy of autoimmune pancreatitis: diagnostic criteria and pitfalls. Am J Surg Pathol. 2005;29:1464–71.PubMedCrossRefGoogle Scholar
  43. 43.
    Chari ST, Kloeppel G, Zhang L, Notohara K, Lerch MM, Shimosegawa T, Autoimmune Pancreatitis International Cooperative Study G. Histopathologic and clinical subtypes of autoimmune pancreatitis: the Honolulu consensus document. Pancreas. 2010;39: 549–54.PubMedCrossRefGoogle Scholar
  44. 44.
    Voss M, Hammel P, Molas G, Palazzo L, Dancour A, O’Toole D, Terris B, Degott C, Bernades P, Ruszniewski P. Value of endoscopic ultrasound guided fine needle aspiration biopsy in the diagnosis of solid pancreatic masses. Gut. 2000;46:244–9.PubMedCrossRefGoogle Scholar
  45. 45.
    Eloubeidi MA, Tamhane A. EUS-guided FNA of solid pancreatic masses: a learning curve with 300 consecutive procedures. Gastrointest Endosc. 2005; 61:700–8.PubMedCrossRefGoogle Scholar
  46. 46.
    Mitsuhashi T, Ghafari S, Chang CY, Gu M. Endoscopic ultrasound-guided fine needle aspiration of the pancreas: cytomorphological evaluation with emphasis on adequacy assessment, diagnostic criteria and contamination from the gastrointestinal tract. Cytopathology. 2006;17:34–41.PubMedCrossRefGoogle Scholar
  47. 47.
    Turner BG, Cizginer S, Agarwal D, Yang J, Pitman MB, Brugge WR. Diagnosis of pancreatic neoplasia with EUS and FNA: a report of accuracy. Gastrointest Endosc. 2009;71:91–8.CrossRefGoogle Scholar
  48. 48.
    Piccinino F, Sagnelli E, Pasquale G, Giusti G. Complications following percutaneous liver biopsy. A multicentre retrospective study on 68,276 biopsies. J Hepatol. 1986;2:165–73.PubMedCrossRefGoogle Scholar
  49. 49.
    Kovalik EC, Schwab SJ, Gunnells JC, Bowie D, Smith SR. No change in complication rate using spring-loaded gun compared to traditional percutaneous renal allograft biopsy techniques. Clin Nephrol. 1996;45:383–5.PubMedGoogle Scholar
  50. 50.
    Brandt KR, Charboneau JW, Stephens DH, Welch TJ, Goellner JR. CT- and US-guided biopsy of the pancreas. Radiology. 1993;187:99–104.PubMedGoogle Scholar
  51. 51.
    Welch TJ, Sheedy 2nd PF, Johnson CD, Johnson CM, Stephens DH. CT-guided biopsy: prospective analysis of 1,000 procedures. Radiology. 1989;171:493–6.PubMedGoogle Scholar
  52. 52.
    Harrison BD, Thorpe RS, Kitchener PG, McCann BG, Pilling JR. Percutaneous Trucut lung biopsy in the diagnosis of localised pulmonary lesions. Thorax. 1984;39:493–9.PubMedCrossRefGoogle Scholar
  53. 53.
    Ingram DM, Sheiner HJ, Shilkin KB. Operative biopsy of the pancreas using the Trucut needle. Aust N Z J Surg. 1978;48:203–6.PubMedCrossRefGoogle Scholar
  54. 54.
    Lavelle MA, O’Toole A. Trucut biopsy of the prostate. Br J Urol. 1994;73:600.PubMedGoogle Scholar
  55. 55.
    Ball AB, Fisher C, Pittam M, Watkins RM, Westbury G. Diagnosis of soft tissue tumours by Tru-Cut biopsy. Br J Surg. 1990;77:756–8.PubMedCrossRefGoogle Scholar
  56. 56.
    Wiersema MJ, Levy MJ, Harewood GC, Vazquez-Sequeiros E, Jondal ML, Wiersema LM. Initial experience with EUS-guided trucut needle biopsies of perigastric organs. Gastrointest Endosc. 2002;56: 275–8.PubMedCrossRefGoogle Scholar
  57. 57.
    Levy MJ, Jondal ML, Clain J, Wiersema MJ. Preliminary experience with an EUS-guided trucut biopsy needle compared with EUS-guided FNA. Gastrointest Endosc. 2003;57:101–6.PubMedCrossRefGoogle Scholar
  58. 58.
    Levy MJ, Wiersema MJ. EUS-guided trucut biopsy. Gastrointest Endosc. 2005;62:417–26.PubMedCrossRefGoogle Scholar
  59. 59.
    Levy MJ, Smyrk TC, Reddy RP, Clain JE, Harewood GC, Kendrick ML, Pearson RK, Petersen BT, Rajan E, Topazian MD, Wang KK, Wiersema MJ, Yusuf TE, Chari ST. Endoscopic ultrasound-guided trucut biopsy of the cyst wall for diagnosing cystic pancreatic tumors. Clin Gastroenterol Hepatol. 2005;3: 974–9.PubMedCrossRefGoogle Scholar
  60. 60.
    Gines A, Wiersema MJ, Clain JE, Pochron NL, Rajan E, Levy MJ. Prospective study of a Trucut needle for performing EUS-guided biopsy with EUS-guided FNA rescue. Gastrointest Endosc;62.Google Scholar
  61. 61.
    Abraham SC, Wilentz RE, Yeo CJ, Sohn TA, Cameron JL, Boitnott JK, Hruban RH. Pancreaticoduodenectomy (Whipple resection) in patients without malignancy: are they all chronic pancreatitis? Am J Surg Pathol. 2003;27:110–20.PubMedCrossRefGoogle Scholar
  62. 62.
    Kloppel G, Luttges J, Sipos B, Capelli P, Zamboni G. Autoimmune pancreatitis: pathological findings. Jop: J Pancreas [Electronic Resource]. 2005;6:97–101.Google Scholar
  63. 63.
    Zamboni G, Luttges J, Capelli P, Frulloni L, Cavallini G, Pederzoli P, Leins A, Longnecker D, Kloppel G. Histopathological features of diagnostic and clinical relevance in autoimmune pancreatitis: a study on 53 resection specimens and 9 biopsy specimens. Virchows Arch. 2004;445:552–63.PubMedCrossRefGoogle Scholar
  64. 64.
    Suda K, Takase M, Fukumura Y, Ogura K, Ueda A, Matsuda T, Suzuki F. Histopathologic characteristics of autoimmune pancreatitis based on comparison with chronic pancreatitis. Pancreas. 2005;30:355–8.PubMedCrossRefGoogle Scholar
  65. 65.
    Kamisawa T. IgG4-positive plasma cells specifically infiltrate various organs in autoimmune pancreatitis. Pancreas. 2004;29:167–8.PubMedCrossRefGoogle Scholar
  66. 66.
    Kawaguchi K, Koike M, Tsuruta K, Okamoto A, Tabata I, Fujita N. Lymphoplasmacytic sclerosing pancreatitis with cholangitis: a variant of primary sclerosing cholangitis extensively involving pancreas. Hum Pathol. 1991;22:387–95.PubMedCrossRefGoogle Scholar
  67. 67.
    Kloppel G. Chronic pancreatitis, pseudotumors and other tumor-like lesions. Mod Pathol. 2007;20 Suppl 1:S113–31.PubMedCrossRefGoogle Scholar
  68. 68.
    Kamisawa T, Takuma K, Tabata T, Inaba Y, Egawa N, Tsuruta K, Hishima T, Sasaki T, Itoi T. Serum IgG4-negative autoimmune pancreatitis. J Gastroenterol. 2010;46:108–16.PubMedCrossRefGoogle Scholar
  69. 69.
    Naitoh I, Nakazawa T, Ohara H, Ando T, Hayashi K, Okumura F, Miyabe K, Yoshida M, Sano H, Takada H, Kanematsu T, Joh T. Comparative evaluation of the Japanese diagnostic criteria for autoimmune pancreatitis. Pancreas. 2010;39:1173–9.PubMedCrossRefGoogle Scholar
  70. 70.
    Kloppel G, Detlefsen S, Chari ST, Longnecker DS, Zamboni G. Autoimmune pancreatitis: the clinicopathological characteristics of the subtype with granulocytic epithelial lesions. J Gastroenterol. 2010;45: 787–93.PubMedCrossRefGoogle Scholar
  71. 71.
    Deshpande V, Gupta R, Sainani N, Sahani DV, Virk R, Ferrone C, Khosroshahi A, Stone JH, Lauwers GY. Subclassification of autoimmune pancreatitis: a histologic classification with clinical significance. Am J Surg Pathol. 2010;35:26–35.CrossRefGoogle Scholar

Copyright information

© Springer Science+Business Media New York 2013

Authors and Affiliations

  1. 1.Division of Gastroenterology and HepatologyMayo ClinicRochesterUSA
  2. 2.Harvard Medical SchoolMassachusetts General HospitalBostonUSA

Personalised recommendations