Skip to main content

Advertisement

Log in

Autoimmune pancreatitis: an update

  • Special Section: Pancreatitis
  • Published:
Abdominal Radiology Aims and scope Submit manuscript

Abstract

Objective

Since the time of inception of autoimmune pancreatitis (AIP), our knowledge of autoimmune pancreatitis has expanded significantly. The aim of this review is to provide an update on clinical manifestations, diagnosis, imaging features, and treatment of AIP.

Background and clinical significance

Type 1 AIP is the pancreatic manifestation of IgG4-related systemic disease, which can be diagnosed using a combination of clinical, histopathological, pancreatic imaging findings in conjunction with manifestation in other organs, as well of responsiveness to steroid treatment. It is vital to differentiate AIP from pancreatic cancer since both can mimic each other clinically and radiologically. Type 2 AIP is a rare but distinct subtype of AIP which occurs mostly in the younger patient.

Conclusion

AIP is steroid-responsive chronic pancreatitis with distinct manifestations on imaging.

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.

Fig. 1
Fig. 2
Fig. 3
Fig. 4
Fig. 5
Fig. 6
Fig. 7
Fig. 8
Fig. 9
Fig. 10
Fig. 11
Fig. 12
Fig. 13

Similar content being viewed by others

References

  1. Sarles H, Sarles JC, Muratore R, Guien C (1961) Chronic inflammatory sclerosis of the pancreas--an autonomous pancreatic disease? The American journal of digestive diseases 6:688-698

    Article  CAS  Google Scholar 

  2. Yoshida K, Toki F, Takeuchi T, Watanabe S, Shiratori K, Hayashi N (1995) Chronic pancreatitis caused by an autoimmune abnormality. Proposal of the concept of autoimmune pancreatitis. Digestive diseases and sciences 40 (7):1561-1568

  3. KKhandelwal A, Shanbhogue AK, Takahashi N, Sandrasegaran K, Prasad SR (2014) Recent advances in the diagnosis and management of autoimmune pancreatitis. AJR American journal of roentgenology 202 (5):1007-1021. https://doi.org/10.2214/ajr.13.11247

    Article  PubMed  Google Scholar 

  4. Nagpal SJS, Sharma A, Chari ST (2018) Autoimmune Pancreatitis. The American journal of gastroenterology 113 (9):1301.https://doi.org/10.1038/s41395-018-0146-0

    Article  PubMed  Google Scholar 

  5. Chari ST, Takahashi N, Levy MJ, Smyrk TC, Clain JE, Pearson RK, Petersen BT, Topazian MA, Vege SS (2009) A diagnostic strategy to distinguish autoimmune pancreatitis from pancreatic cancer. Clinical gastroenterology and hepatology : the official clinical practice journal of the American Gastroenterological Association 7 (10):1097-1103. https://doi.org/10.1016/j.cgh.2009.04.020

    Article  CAS  Google Scholar 

  6. Nishimori I, Tamakoshi A, Otsuki M (2007) Prevalence of autoimmune pancreatitis in Japan from a nationwide survey in 2002. Journal of gastroenterology 42 Suppl 18:6-8. https://doi.org/10.1007/s00535-007-2043-y

    Article  PubMed  Google Scholar 

  7. Kanno A, Masamune A, Okazaki K, Kamisawa T, Kawa S, Nishimori I, Tsuji I, Shimosegawa T (2015) Nationwide epidemiological survey of autoimmune pancreatitis in Japan in 2011. Pancreas 44 (4):535-539. https://doi.org/10.1097/mpa.0000000000000325

    Article  PubMed  Google Scholar 

  8. de Castro SM, de Nes LC, Nio CY, Velseboer DC, ten Kate FJ, Busch OR, van Gulik TM, Gouma DJ (2010) Incidence and characteristics of chronic and lymphoplasmacytic sclerosing pancreatitis in patients scheduled to undergo a pancreatoduodenectomy. HPB : the official journal of the International Hepato Pancreato Biliary Association 12 (1):15-21. https://doi.org/10.1111/j.1477-2574.2009.00112.x

    Article  Google Scholar 

  9. Yadav D, Notahara K, Smyrk TC, Clain JE, Pearson RK, Farnell MB, Chari ST (2003) Idiopathic tumefactive chronic pancreatitis: clinical profile, histology, and natural history after resection. Clinical gastroenterology and hepatology : the official clinical practice journal of the American Gastroenterological Association 1 (2):129-135. https://doi.org/10.1053/cgh.2003.50016

    Article  Google Scholar 

  10. Khandelwal A, Saboo SS (2013) Re: Autoimmune pancreatitis: an illustrated guide to diagnosis. Clinical radiology 68 (9):e518. https://doi.org/10.1016/j.crad.2013.03.013

    Article  CAS  PubMed  Google Scholar 

  11. Kamisawa T, Chari ST, Giday SA, Kim MH, Chung JB, Lee KT, Werner J, Bergmann F, Lerch MM, Mayerle J, Pickartz T, Lohr M, Schneider A, Frulloni L, Webster GJ, Reddy DN, Liao WC, Wang HP, Okazaki K, Shimosegawa T, Kloeppel G, Go VL (2011) Clinical profile of autoimmune pancreatitis and its histological subtypes: an international multicenter survey. Pancreas 40 (6):809-814. https://doi.org/10.1097/MPA.0b013e3182258a15

    Article  CAS  PubMed  Google Scholar 

  12. Ito T, Nakamura T, Fujimori N, Niina Y, Igarashi H, Oono T, Uchida M, Kawabe K, Takayanagi R, Nishimori I, Otsuki M, Shimosegawa T (2011) Characteristics of pancreatic diabetes in patients with autoimmune pancreatitis. Journal of digestive diseases 12 (3):210-216. https://doi.org/10.1111/j.1751-2980.2011.00498.x

    Article  PubMed  Google Scholar 

  13. Nishimori I, Tamakoshi A, Kawa S, Tanaka S, Takeuchi K, Kamisawa T, Saisho H, Hirano K, Okamura K, Yanagawa N, Otsuki M (2006) Influence of steroid therapy on the course of diabetes mellitus in patients with autoimmune pancreatitis: findings from a nationwide survey in Japan. Pancreas 32 (3):244-248. https://doi.org/10.1097/01.mpa.0000202950.02988.07

    Article  CAS  PubMed  Google Scholar 

  14. Sandrasegaran K, Menias CO (2018) Imaging in Autoimmune Pancreatitis and Immunoglobulin G4-Related Disease of the Abdomen. Gastroenterology clinics of North America 47 (3):603-619. https://doi.org/10.1016/j.gtc.2018.04.007

    Article  PubMed  Google Scholar 

  15. Chari ST, Kloeppel G, Zhang L, Notohara K, Lerch MM, Shimosegawa T (2010) Histopathologic and clinical subtypes of autoimmune pancreatitis: the Honolulu consensus document. Pancreas 39 (5):549-554. https://doi.org/10.1097/MPA.0b013e3181e4d9e5

    Article  CAS  PubMed  Google Scholar 

  16. Sah RP, Chari ST, Pannala R, Sugumar A, Clain JE, Levy MJ, Pearson RK, Smyrk TC, Petersen BT, Topazian MD, Takahashi N, Farnell MB, Vege SS (2010) Differences in clinical profile and relapse rate of type 1 versus type 2 autoimmune pancreatitis. Gastroenterology 139 (1):140-148; quiz e112-143. https://doi.org/10.1053/j.gastro.2010.03.054

  17. Shinagare S, Shinagare AB, Deshpande V (2012) Autoimmune pancreatitis: a guide for the histopathologist. Seminars in diagnostic pathology 29 (4):197-204. https://doi.org/10.1053/j.semdp.2012.07.007

    Article  PubMed  Google Scholar 

  18. 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 (2006) Clinical diagnostic criteria of autoimmune pancreatitis: revised proposal. Journal of gastroenterology 41 (7):626-631. https://doi.org/10.1007/s00535-006-1868-0

    Article  PubMed  PubMed Central  Google Scholar 

  19. Choi EK, Kim MH, Lee TY, Kwon S, Oh HC, Hwang CY, Seo DW, Lee SS, Lee SK (2007) The sensitivity and specificity of serum immunoglobulin G and immunoglobulin G4 levels in the diagnosis of autoimmune chronic pancreatitis: Korean experience. Pancreas 35 (2):156-161. https://doi.org/10.1097/MPA.0b013e318053eacc

    Article  PubMed  Google Scholar 

  20. 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 (2007) Value of serum IgG4 in the diagnosis of autoimmune pancreatitis and in distinguishing it from pancreatic cancer. The American journal of gastroenterology 102 (8):1646-1653. https://doi.org/10.1111/j.1572-0241.2007.01264.x

    Article  CAS  PubMed  Google Scholar 

  21. Shimosegawa T, Chari ST, Frulloni L, Kamisawa T, Kawa S, Mino-Kenudson M, Kim MH, Kloppel G, Lerch MM, Lohr M, Notohara K, Okazaki K, Schneider A, Zhang L (2011) International consensus diagnostic criteria for autoimmune pancreatitis: guidelines of the International Association of Pancreatology. Pancreas 40 (3):352-358. https://doi.org/10.1097/MPA.0b013e3182142fd2

    Article  PubMed  Google Scholar 

  22. Majumder S, Takahashi N, Chari ST (2017) Autoimmune Pancreatitis. Digestive diseases and sciences 62 (7):1762-1769. https://doi.org/10.1007/s10620-017-4541-y

    Article  CAS  PubMed  Google Scholar 

  23. CCrosara S, D'Onofrio M, De Robertis R, Demozzi E, Canestrini S, Zamboni G, Pozzi Mucelli R (2014) Autoimmune pancreatitis: Multimodality non-invasive imaging diagnosis. World journal of gastroenterology 20 (45):16881-16890. https://doi.org/10.3748/wjg.v20.i45.16881

    Article  PubMed  PubMed Central  Google Scholar 

  24. Morse B, Centeno B, Vignesh S (2014) Autoimmune pancreatitis: updated concepts of a challenging diagnosis. The American journal of medicine 127 (10):1010.e1011-1019. https://doi.org/10.1016/j.amjmed.2014.04.033

    Article  Google Scholar 

  25. RRehnitz C, Klauss M, Singer R, Ehehalt R, Werner J, Buchler MW, Kauczor HU, Grenacher L (2011) Morphologic patterns of autoimmune pancreatitis in CT and MRI. Pancreatology : official journal of the International Association of Pancreatology (IAP) [et al] 11 (2):240-251. https://doi.org/10.1159/000327708

  26. Manfredi R, Frulloni L, Mantovani W, Bonatti M, Graziani R, Pozzi Mucelli R (2011) Autoimmune pancreatitis: pancreatic and extrapancreatic MR imaging-MR cholangiopancreatography findings at diagnosis, after steroid therapy, and at recurrence. Radiology 260 (2):428-436. https://doi.org/10.1148/radiol.11101729

    Article  PubMed  Google Scholar 

  27. Takahashi N, Fletcher JG, Hough DM, Fidler JL, Kawashima A, Mandrekar JN, Chari ST (2009) Autoimmune pancreatitis: differentiation from pancreatic carcinoma and normal pancreas on the basis of enhancement characteristics at dual-phase CT. AJR American journal of roentgenology 193 (2):479-484. https://doi.org/10.2214/ajr.08.1883

    Article  PubMed  Google Scholar 

  28. Kwon JH, Kim JH, Kim SY, Byun JH, Kim HJ, Lee MG, Lee SS (2019) Differentiating focal autoimmune pancreatitis and pancreatic ductal adenocarcinoma: contrast-enhanced MRI with special emphasis on the arterial phase. European radiology. https://doi.org/10.1007/s00330-019-06200-0

    Article  PubMed  Google Scholar 

  29. Choi SY, Kim SH, Kang TW, Song KD, Park HJ, Choi YH (2016) Differentiating Mass-Forming Autoimmune Pancreatitis From Pancreatic Ductal Adenocarcinoma on the Basis of Contrast-Enhanced MRI and DWI Findings. AJR American journal of roentgenology 206 (2):291-300. https://doi.org/10.2214/ajr.15.14974

    Article  PubMed  Google Scholar 

  30. Park SH, Kim MH, Kim SY, Kim HJ, Moon SH, Lee SS, Byun JH, Lee SK, Seo DW, Lee MG (2010) Magnetic resonance cholangiopancreatography for the diagnostic evaluation of autoimmune pancreatitis. Pancreas 39 (8):1191-1198. https://doi.org/10.1097/MPA.0b013e3181dbf469

    Article  PubMed  Google Scholar 

  31. Kamisawa T, Tu Y, Egawa N, Tsuruta K, Okamoto A, Kodama M, Kamata N (2009) Can MRCP replace ERCP for the diagnosis of autoimmune pancreatitis? Abdominal imaging 34 (3):381-384. https://doi.org/10.1007/s00261-008-9401-y

    Article  CAS  PubMed  Google Scholar 

  32. Sahani DV, Kalva SP, Farrell J, Maher MM, Saini S, Mueller PR, Lauwers GY, Fernandez CD, Warshaw AL, Simeone JF (2004) Autoimmune pancreatitis: imaging features. Radiology 233 (2):345-352. https://doi.org/10.1148/radiol.2332031436

    Article  PubMed  Google Scholar 

  33. Kim HJ, Kim YK, Jeong WK, Lee WJ, Choi D (2015) Pancreatic duct "Icicle sign" on MRI for distinguishing autoimmune pancreatitis from pancreatic ductal adenocarcinoma in the proximal pancreas. European radiology 25 (6):1551-1560 https://doi.org/10.1007/s00330-014-3548-4

    Article  PubMed  Google Scholar 

  34. Kamisawa T, Takuma K, Anjiki H, Egawa N, Hata T, Kurata M, Honda G, Tsuruta K, Suzuki M, Kamata N, Sasaki T (2010) Differentiation of autoimmune pancreatitis from pancreatic cancer by diffusion-weighted MRI. The American journal of gastroenterology 105 (8):1870-1875. https://doi.org/10.1038/ajg.2010.87

    Article  PubMed  Google Scholar 

  35. Klauss M, Maier-Hein K, Tjaden C, Hackert T, Grenacher L, Stieltjes B (2016) IVIM DW-MRI of autoimmune pancreatitis: therapy monitoring and differentiation from pancreatic cancer. European radiology 26 (7):2099-2106. https://doi.org/10.1007/s00330-015-4041-4

    Article  PubMed  Google Scholar 

  36. Raina A, Yadav D, Krasinskas AM, McGrath KM, Khalid A, Sanders M, Whitcomb DC, Slivka A (2009) Evaluation and management of autoimmune pancreatitis: experience at a large US center. The American journal of gastroenterology 104 (9):2295-2306. https://doi.org/10.1038/ajg.2009.325

    Article  PubMed  PubMed Central  Google Scholar 

  37. Kawamoto S, Siegelman SS, Hruban RH, Fishman EK (2008) Lymphoplasmacytic sclerosing pancreatitis (autoimmune pancreatitis): evaluation with multidetector CT. Radiographics : a review publication of the Radiological Society of North America, Inc 28 (1):157-170. https://doi.org/10.1148/rg.281065188

  38. Ikeura T, Miyoshi H, Shimatani M, Uchida K, Takaoka M, Okazaki K (2016) Long-term outcomes of autoimmune pancreatitis. World journal of gastroenterology 22 (34):7760-7766. https://doi.org/10.3748/wjg.v22.i34.7760

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  39. Negrelli R, Boninsegna E, Avesani G, Zamboni GA, Brozzi L, Frulloni L, Manfredi R, Pozzi Mucelli R (2018) Type 1 and Type 2 Autoimmune Pancreatitis: Distinctive Clinical and Pathological Features, But Are There Any Differences at Magnetic Resonance? Experience From a Referral Center. Pancreas 47 (9):1115-1122. https://doi.org/10.1097/mpa.0000000000001142

    Article  PubMed  Google Scholar 

  40. Maruyama M, Arakura N, Ozaki Y, Watanabe T, Ito T, Yoneda S, Maruyama M, Muraki T, Hamano H, Matsumoto A, Kawa S (2012) Risk factors for pancreatic stone formation in autoimmune pancreatitis over a long-term course. Journal of gastroenterology 47 (5):553-560. https://doi.org/10.1007/s00535-011-0510-y

    Article  CAS  PubMed  Google Scholar 

  41. Ballehaninna UK, Chamberlain RS (2012) The clinical utility of serum CA 19-9 in the diagnosis, prognosis and management of pancreatic adenocarcinoma: An evidence based appraisal. Journal of gastrointestinal oncology 3 (2):105-119. https://doi.org/10.3978/j.issn.2078-6891.2011.021

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  42. Choi YJ, Byun JH, Kim JY, Kim MH, Jang SJ, Ha HK, Lee MG (2008) Diffuse pancreatic ductal adenocarcinoma: characteristic imaging features. European journal of radiology 67 (2):321-328. https://doi.org/10.1016/j.ejrad.2007.07.010

    Article  PubMed  Google Scholar 

  43. Levy MJ, Reddy RP, Wiersema MJ, Smyrk TC, Clain JE, Harewood GC, Pearson RK, Rajan E, Topazian MD, Yusuf TE, Chari ST, Petersen BT (2005) EUS-guided trucut biopsy in establishing autoimmune pancreatitis as the cause of obstructive jaundice. Gastrointestinal endoscopy 61 (3):467-472

    Article  Google Scholar 

  44. Nishino T, Oyama H, Toki F, Shiratori K (2010) Differentiation between autoimmune pancreatitis and pancreatic carcinoma based on endoscopic retrograde cholangiopancreatography findings. Journal of gastroenterology 45 (9):988-996. https://doi.org/10.1007/s00535-010-0250-4

    Article  PubMed  Google Scholar 

  45. Nakajo M, Jinnouchi S, Fukukura Y, Tanabe H, Tateno R, Nakajo M (2007) The efficacy of whole-body FDG-PET or PET/CT for autoimmune pancreatitis and associated extrapancreatic autoimmune lesions. European journal of nuclear medicine and molecular imaging 34 (12):2088-2095. https://doi.org/10.1007/s00259-007-0562-7

    Article  PubMed  Google Scholar 

  46. Lee TY, Kim MH, Park DH, Seo DW, Lee SK, Kim JS, Lee KT (2009) Utility of 18F-FDG PET/CT for differentiation of autoimmune pancreatitis with atypical pancreatic imaging findings from pancreatic cancer. AJR American journal of roentgenology 193 (2):343-348. https://doi.org/10.2214/ajr.08.2297

    Article  PubMed  Google Scholar 

  47. Kamisawa T, Takum K, Anjiki H, Egawa N, Kurata M, Honda G, Tsuruta K (2010) FDG-PET/CT findings of autoimmune pancreatitis. Hepato-gastroenterology 57 (99-100):447-450

    PubMed  Google Scholar 

  48. Takahashi N, Kawashima A, Fletcher JG, Chari ST (2007) Renal involvement in patients with autoimmune pancreatitis: CT and MR imaging findings. Radiology 242 (3):791-801. https://doi.org/10.1148/radiol.2423060003

    Article  PubMed  Google Scholar 

  49. Inoue D, Zen Y, Abo H, Gabata T, Demachi H, Yoshikawa J, Miyayama S, Nakanuma Y, Matsui O (2011) Immunoglobulin G4-related periaortitis and periarteritis: CT findings in 17 patients. Radiology 261 (2):625-633. https://doi.org/10.1148/radiol.11102250

    Article  PubMed  Google Scholar 

  50. Sakamoto M, Moriyama M, Shimizu M, Chinju A, Mochizuki K, Munemura R, Ohyama K, Maehara T, Ogata K, Ohta M, Yamauchi M, Ishiguro N, Matsumura M, Ohyama Y, Kiyoshima T, Nakamura S (2019) The diagnostic utility of submandibular gland sonography and labial salivary gland biopsy in IgG4-related dacryoadenitis and sialadenitis: Its potential application to the diagnostic criteria. Modern rheumatology:1-6. https://doi.org/10.1080/14397595.2019.1576271

  51. Inoue D, Zen Y, Sato Y, Abo H, Demachi H, Uchiyama A, Gabata T, Matsui O (2012) IgG4-Related Perineural Disease. International journal of rheumatology 2012:401890. https://doi.org/10.1155/2012/401890

    Article  PubMed  PubMed Central  Google Scholar 

  52. Inoue D, Zen Y, Abo H, Gabata T, Demachi H, Kobayashi T, Yoshikawa J, Miyayama S, Yasui M, Nakanuma Y, Matsui O (2009) Immunoglobulin G4-related lung disease: CT findings with pathologic correlations. Radiology 251 (1):260-270. https://doi.org/10.1148/radiol.2511080965

    Article  PubMed  Google Scholar 

  53. IInoue D, Zen Y, Komori T, Yoshida K, Yoneda N, Kitao A, Kozaka K, Izumozaki A, Matsumoto J, Toshima F, Kobayashi S, Gabata T (2019) CT Findings of Thoracic Paravertebral Lesions in IgG4-Related Disease. AJR American journal of roentgenology:1-6. https://doi.org/10.2214/ajr.18.20834

  54. Ghazale A, Chari ST, Zhang L, Smyrk TC, Takahashi N, Levy MJ, Topazian MD, Clain JE, Pearson RK, Petersen BT, Vege SS, Lindor K, Farnell MB (2008) Immunoglobulin G4-associated cholangitis: clinical profile and response to therapy. Gastroenterology 134 (3):706-715. https://doi.org/10.1053/j.gastro.2007.12.009

    Article  PubMed  Google Scholar 

  55. HHart PA, Kamisawa T, Brugge WR, Chung JB, Culver EL, Czako L, Frulloni L, Go VL, Gress TM, Kim MH, Kawa S, Lee KT, Lerch MM, Liao WC, Lohr M, Okazaki K, Ryu JK, Schleinitz N, Shimizu K, Shimosegawa T, Soetikno R, Webster G, Yadav D, Zen Y, Chari ST (2013) Long-term outcomes of autoimmune pancreatitis: a multicentre, international analysis. Gut 62 (12):1771-1776. https://doi.org/10.1136/gutjnl-2012-303617

    Article  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to Naoki Takahashi.

Additional information

Publisher's Note

Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Khandelwal, A., Inoue, D. & Takahashi, N. Autoimmune pancreatitis: an update. Abdom Radiol 45, 1359–1370 (2020). https://doi.org/10.1007/s00261-019-02275-x

Download citation

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00261-019-02275-x

Keywords

Navigation