Journal of Gastroenterology

, Volume 42, Supplement 18, pp 39–41 | Cite as

Diagnosis of autoimmune pancreatitis using its five cardinal features: introducing the Mayo Clinic's HISORt criteria

  • Suresh T. Chari
Diagnostic Criteria


Autoimmune pancreatitis (AIP) is a recently defined form of chronic pancreatitis. While numerous case reports and small case series of AIP have been reported from Japan, there have been relatively few from the West. Based on a retrospective review of our experience with resected AIP and a review of the literature, we have identified five cardinal features of AIP in histology, imaging, serology, other organ involvement, and response to steroid therapy, which are summarized in the mnemonic HISORt. A combination of the HISORt criteria can be used to definitively diagnose a wide spectrum of manifestations of AIP.

Key words

autoimmune diseases pancreatitis steroids 


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  1. 1.
    Chari, ST, Smyrk, TC, Levy, MJ, Topazian, MD, Takahashi, N, Zhang, L,  et al. 2006Diagnosis of autoimmune pancreatitis: the Mayo Clinic experienceClin Gastroenterol Hepatol410106PubMedCrossRefGoogle Scholar
  2. 2.
    Kloppel, G, Luttges, J, Lohr, M, Zamboni, G, Longnecker, D 2003Autoimmune pancreatitis: pathological, clinical, and immunological featuresPancreas27149PubMedCrossRefGoogle Scholar
  3. 3.
    Notohara, K, Burgart, LJ, Yadav, D, Chari, S, Smyrk, TC 2003Idiopathic chronic pancreatitis with periductal lymphoplasmacytic infiltration: clinicopathologic features of 35 casesAm J Surg Pathol27111927PubMedCrossRefGoogle Scholar
  4. 4.
    Ectors, N, Maillet, B, Aerts, R, Geboes, K, Donner, A, Borchard, F,  et al. 1997Non-alcoholic duct-destructive chronic pancreatitisGut412638PubMedCrossRefGoogle Scholar
  5. 5.
    Abraham, SC, Wilentz, RE, Yeo, CJ, Sohn, TA, Cameron, JL, Boitnott, JK,  et al. 2003Pancreaticoduodenectomy (Whipple resections) in patients without malignancy: are they all “chronic pancreatitisAm J Surg Pathol2711020PubMedCrossRefGoogle Scholar
  6. 6.
    Weber, SM, Cubukcu-Dimopulo, O, Palesty, JA, Suriawinata, A, Klimstra, D, Brennan, MF,  et al. 2003Lymphoplasmacytic sclerosing pancreatitis: inflammatory mimic of pancreatic carcinomaJ Gastrointest Surg712937PubMedCrossRefGoogle Scholar
  7. 7.
    Yadav, D, Notahara, K, Smyrk, TC, Clain, JE, Pearson, RK, Farnell, MB,  et al. 2003Idiopathic tumefactive chronic pancreatitis: clinical profile, histology, and natural history after resectionClin Gastroenterol Hepatol112935PubMedCrossRefGoogle Scholar
  8. 8.
    Kamisawa, T, Funata, N, Hayashi, Y, Eishi, Y, Koike, M, Tsuruta, K,  et al. 2003A new clinicopathological entity of IgG4-related autoimmune diseaseJ Gastroenterol389824PubMedCrossRefGoogle Scholar
  9. 9.
    Kamisawa, T, Funata, N, Hayashi, Y, Tsuruta, K, Okamoto, A, Amemiya, K,  et al. 2003Close relationship between autoimmune pancreatitis and multifocal fibrosclerosisGut526837PubMedCrossRefGoogle Scholar
  10. 10.
    Kamisawa, T, Funata, N, Hayashi, Y 2004Lymphoplasmacytic sclerosing pancreatitis is a pancreatic lesion of IgG4-related systemic diseaseAm J Surg Pathol281114PubMedGoogle Scholar
  11. 11.
    Zhang, L, Notohara, K, Levy, MJ, Chari, ST, Smyrk, TC 2007IgG4-positive plasma cell infiltration in the diagnosis of autoimmune pancreatitisModern Pathology20238PubMedCrossRefGoogle Scholar
  12. 12.
    Irie, H, Honda, H, Baba, S, Kuroiwa, T, Yoshimitsu, K, Tajima, T,  et al. 1998Autoimmune pancreatitis: CT and MR characteristicsAm J Roentgenol17013237Google Scholar
  13. 13.
    Yoshida, K, Toki, F, Takeuchi, T, Watanabe, S, Shiratori, K, Hayashi, N 1995Chronic pancreatitis caused by an autoimmune abnormality. Proposal of the concept of autoimmune pancreatitisDig Dis Sci4015618PubMedCrossRefGoogle Scholar
  14. 14.
    Horiuchi, A, Kawa, S, Hamano, H, Hayama, M, Ota, H, Kiyosawa, K 2002ERCP features in 27 patients with autoimmune pancreatitisGastrointest Endosc554949PubMedGoogle Scholar
  15. 15.
    Pearson, RK, Longnecker, DS, Chari, ST, Smyrk, TC, Okazaki, K, Frulloni, L,  et al. 2003Controversies in clinical pancreatology. Autoimmune pancreatitis: does it exist?Pancreas27113PubMedCrossRefGoogle Scholar
  16. 16.
    Wakabayashi, T, Kawaura, Y, Satomura, Y, Fujii, T, Motoo, Y, Okai, T,  et al. 2002Clinical study of chronic pancreatitis with focal irregular narrowing of the main pancreatic duct and mass formation: comparison with chronic pancreatitis showing diffuse irregular narrowing of the main pancreatic ductPancreas252839PubMedCrossRefGoogle Scholar
  17. 17.
    Wakabayashi, T, Kawaura, Y, Satomura, Y, Watanabe, H, Motoo, Y, Okai, T,  et al. 2003Clinical and imaging features of autoimmune pancreatitis with focal pancreatic swelling or mass formation: comparison with so-called tumor-forming pancreatitis and pancreatic carcinomaAm J Gastroenterol98267987PubMedCrossRefGoogle Scholar
  18. 18.
    Takayama, M, Hamano, H, Ochi, Y, Saegusa, H, Komatsu, K, Muraki, T,  et al. 2004Recurrent attacks of autoimmune pancreatitis result in pancreatic stone formationAm J Gastroenterol999327PubMedCrossRefGoogle Scholar
  19. 19.
    Nishimura, T, Masaoka, T, Suzuki, H, Aiura, K, Nagata, H, Ishii, H 2004Autoimmune pancreatitis with pseudocystsJ Gastroenterol39100510PubMedCrossRefGoogle Scholar
  20. 20.
    Ichikawa, T, Nakao, K, Hamasaki, K, Ohkubo, K, Toriyama, K, Eguchi, K 2005An autopsy case of acute pancreatitis with a high serum IgG4 complicated by amyloidosis and rheumatoid arthritisWorld J Gastroenterol1120324PubMedGoogle Scholar
  21. 21.
    Okazaki, K, Chiba, T 2002Autoimmune-related pancreatitisGut5114PubMedCrossRefGoogle Scholar
  22. 22.
    Kino-Ohsaki, J, Nishimori, I, Morita, M, Okazaki, K, Yamamoto, Y, Onishi, S,  et al. 1996Serum antibodies to carbonic anhydrase I and II in patients with idiopathic chronic pancreatitis and Sjogren's syndromeGastroenterology110157986PubMedCrossRefGoogle Scholar
  23. 23.
    Hamano, H, Kawa, S, Horiuchi, A, Unno, H, Furuya, N, Akamatsu, T,  et al. 2001High serum IgG4 concentrations in patients with sclerosing pancreatitisN Engl J Med3447328PubMedCrossRefGoogle Scholar
  24. 24.
    Kamisawa, T, Egawa, N, Nakajima, H 2003Autoimmune pancreatitis is a systemic autoimmune diseaseAm J Gastroenterol9828112PubMedCrossRefGoogle Scholar
  25. 25.
    Kamisawa, T 2004IgG4-positive plasma cells specifically infiltrate various organs in autoimmune pancreatitisPancreas291678PubMedCrossRefGoogle Scholar
  26. 26.
    Zen, Y, Harada, K, Sasaki, M, Sato, Y, Tsuneyama, K, Haratake, J,  et al. 2004IgG4-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 Pathol281193203PubMedCrossRefGoogle Scholar
  27. 27.
    Nishino, T, Toki, F, Oyama, H, Oi, I, Kobayashi, M, Takasaki, K,  et al. 2005Biliary tract involvement in autoimmune pancreatitisPancreas307682PubMedGoogle Scholar
  28. 28.
    Kamisawa, T, Egawa, N, Inokuma, S, Tsuruta, K, Okamoto, A, Kamata, N,  et al. 2003Pancreatic endocrine and exocrine function and salivary gland function in autoimmune pancreatitis before and after steroid therapyPancreas272358PubMedCrossRefGoogle Scholar
  29. 29.
    Kamisawa, T, Yoshiike, M, Egawa, N, Nakajima, H, Tsuruta, K, Okamoto, A 2005Treating patients with autoimmune pancreatitis: results from a long-term follow-up studyPancreatology52348discussion 238–40PubMedCrossRefGoogle Scholar
  30. 30.
    Yamamoto, M, Takahashi, H, Sugai, S, Imai, K 2005Clinical and pathological characteristics of Mikulicz's disease (IgG4-related plasmacytic exocrinopathy)Autoimmun Rev4195200PubMedCrossRefGoogle Scholar

Copyright information

© Springer-Verlag Tokyo 2007

Authors and Affiliations

  • Suresh T. Chari
    • 1
  1. 1.Division of Gastroenterology and HepatologyMayo Clinic College of MedicineRochesterUSA

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