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Systemic extrapancreatic lesions associated with autoimmune pancreatitis

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Abstract

Autoimmune pancreatitis (AIP) is frequently associated with sclerosing cholangitis (SC). SC with AIP has a cholangiographic appearance that is often confused with primary sclerosing cholangitis (PSC) but only the former responds well to corticosteroid therapy. Detailed study of cholangiographic findings allows discrimination of SC with AIP from PSC. Band-like strictures, a beaded or pruned-tree appearance, and diverticulum-like outpouching were significantly more frequently observed in cases of PSC. In contrast, segmental strictures, dilation after confluent stricture, and strictures of the lower common bile duct were significantly more common in SC with AIP. The other systemic extrapancreatic lesions associated with AIP found in the literature were Sjögren's syndrome, ulcerative colitis, retroperitoneal fibrosis, sialadenitis, thyroiditis, and idiopathic thrombocytopenic purpura. In a comparison of the clinical course and laboratory data of our cases, gamma-globulin, IgG, and IgG4 levels were significantly higher in patients with AIP with systemic extrapancreatic lesions than those without them. In our immunohistochemical study, marked infiltration of IgG4+ plasma cells was frequently observed in the pancreas, liver, bile duct, and salivary glands of the AIP patients examined. In contrast, the degree of infiltration of IgG4+ plasma cells around the bile duct in the portal areas and the extrahepatic bile duct with PSC was significantly lower than with AIP. These results also suggest that AIP is a disease state clearly different from PSC. In addition, the normal epithelia of the pancreatic ducts, bile ducts, gallbladder, and salivary gland ducts reacting with the patients' sera was detectable by the anti-IgG4 antibody. Therefore, AIP may also affect extrapancreatic organs, and the sera of AIP patients may contain an IgG4 autoantibody to various organs.

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References

  1. IG Renner GC Ponto WT Savage Suffix3rd WD Boswell (1980) ArticleTitleIdiopathic retroperitoneal fibrosis producing common bile duct and pancreatic duct obstruction Gastroenterology 79 348–51 Occurrence Handle7399241 Occurrence Handle1:STN:280:DyaL3c3kslelsA%3D%3D

    PubMed  CAS  Google Scholar 

  2. A Chutaputti MI Burrell JL Boyer (1995) ArticleTitlePseudotumor of the pancreas associated with retroperitoneal fibrosis: a dramatic response to corticosteroid therapy Am J Gastroenterol 90 1155–8 Occurrence Handle7611217 Occurrence Handle1:STN:280:DyaK2MzjsFGquw%3D%3D

    PubMed  CAS  Google Scholar 

  3. T Nakazawa H Ohara T Yamada H Ando H Sano S Kajino et al. (2001) ArticleTitleAtypical primary sclerosing cholangitis cases associated with unusual pancreatitis Hepatogastroenterology 48 625–30 Occurrence Handle11462890 Occurrence Handle1:STN:280:DC%2BD38%2FitFOntA%3D%3D

    PubMed  CAS  Google Scholar 

  4. H Hamano S Kawa Y Ochi H Unnno N Shiba M Wajiki et al. (2002) ArticleTitleHydronephrosis associated with retroperitoneal fibrosis and sclerosing pancreatitis Lancet 359 1403–4 Occurrence Handle11978339 Occurrence Handle10.1016/S0140-6736(02)08359-9

    Article  PubMed  Google Scholar 

  5. T Kamisawa N Funata Y Hayashi K Tsuruta A Okamoto K Amemiya et al. (2003) ArticleTitleClose relationship between autoimmune pancreatitis and multifocal fibrosclerosis Gut 52 683–7 Occurrence Handle12692053 Occurrence Handle10.1136/gut.52.5.683 Occurrence Handle1:STN:280:DC%2BD3s7ntlGqug%3D%3D

    Article  PubMed  CAS  Google Scholar 

  6. A Clark RK Zeman PL Choyke EM White MI Burrell EG Grant et al. (1988) ArticleTitlePancreatic pseudotumors associated with idiopathic fibrosclerosis Gastrointest Radiol 13 30–2 Occurrence Handle3350266 Occurrence Handle10.1007/BF01889019 Occurrence Handle1:STN:280:DyaL1c7mslGnug%3D%3D

    Article  PubMed  CAS  Google Scholar 

  7. JM Levey J Mathai (1998) ArticleTitleDiffuse pancreatic fibrosis: an uncommon feature of multifocal idiopathic fibrosclerosis Am J Gastroenterol 93 640–2 Occurrence Handle9576463 Occurrence Handle10.1111/j.1572-0241.1998.181_b.x Occurrence Handle1:STN:280:DyaK1c3jsF2qug%3D%3D

    Article  PubMed  CAS  Google Scholar 

  8. T Kamisawa N Funata Y Hayashi Y Eishi M Koike K Tsuruta et al. (2003) ArticleTitleA new clinicopathological entity of IgG4-related autoimmune disease J Gastroenterol 38 982–4 Occurrence Handle14614606 Occurrence Handle10.1007/s00535-003-1175-y Occurrence Handle1:CAS:528:DC%2BD3sXovVKnu7Y%3D

    Article  PubMed  CAS  Google Scholar 

  9. H Sarles JC Sarles R Muratore C Guien (1961) ArticleTitleChronic inflammatory sclerosis of the pancreas: an autonomous pancreatic disease? Am J Dig Dis 6 688–98 Occurrence Handle13746542 Occurrence Handle10.1007/BF02232341 Occurrence Handle1:STN:280:DyaF3c%2Fms1amsQ%3D%3D

    Article  PubMed  CAS  Google Scholar 

  10. T Nakazawa H Ohara H Sano T Ando S Aoki S Kobayashi et al. (2005) ArticleTitleClinical differences between primary sclerosing cholangitis and sclerosing cholangitis with autoimmune pancreatitis Pancreas 30 20–5 Occurrence Handle15632695

    PubMed  Google Scholar 

  11. H Takikawa T Manabe (1997) ArticleTitlePrimary sclerosing cholangitis in Japan: analysis of 192 cases J Gastroenterol 32 134–7 Occurrence Handle9058310 Occurrence Handle10.1007/BF01213311 Occurrence Handle1:STN:280:DyaK2s3htFeisA%3D%3D

    Article  PubMed  CAS  Google Scholar 

  12. H Ishimaru T Matsuda R Igarashi T Maeba M Tsuzura K Yamamura et al. (1984) ArticleTitlePrimary sclerosing cholangitis associated with submandibular adenitis, chronic pancreatitis and diabetes mellitus Jpn Arch Intern Med 31 25–34

    Google Scholar 

  13. K Kyokane T Ichihara M Horisawa N Suzuki S Ichihara S Suga et al. (1994) ArticleTitleSuccessful treatment of primary sclerosing cholangitis with cyclosporine and corticosteroid Hepatogastroenterology 41 449–52 Occurrence Handle7851854 Occurrence Handle1:STN:280:DyaK2M7lsVOrtQ%3D%3D

    PubMed  CAS  Google Scholar 

  14. T Nakazawa H Ohara H Sano S Aoki S Kobayashi T Okamoto et al. (2004) ArticleTitleCholangiography can discriminate sclerosing cholangitis with autoimmune pancreatitis from primary sclerosing cholantis Gastrointest Endosc 60 937–44 Occurrence Handle15605009 Occurrence Handle10.1016/S0016-5107(04)02229-1

    Article  PubMed  Google Scholar 

  15. P Angulo KP Batts RA Jorgensen NA LaRusso KD Lindor (2000) ArticleTitleOral budesonide in the treatment of primary sclerosing cholangitis Am J Gastroenterol 95 2333–7 Occurrence Handle11007238 Occurrence Handle10.1111/j.1572-0241.2000.02323.x Occurrence Handle1:CAS:528:DC%2BD3cXntFCmtrY%3D

    Article  PubMed  CAS  Google Scholar 

  16. HJF Hoogstraten FP Vleggaar GJ Boland W Steenbergen P Griffoen WCJ Hop et al. (2000) ArticleTitleBudesonide or prednisone in combination with ursodeoxycholic acid in primary sclerosing cholangitis: a randomized double-blind pilot study Am J Gastroenterol 95 2015–22 Occurrence Handle10950051 Occurrence Handle10.1111/j.1572-0241.2000.02267.x

    Article  PubMed  Google Scholar 

  17. Nakazawa T, Ohara H, Yamada T, Sano H, Ando H, Kajino S, et al. Atypical primary sclerosing cholangitis. Autoimmune liver disease: its recent advances. In: Nishioka M, Watanabe S, Akima K, editors. Proceedings of the International Symposium of Digestive Diseases Week; 1999 Oct 29–30; Hiroshima, Japan. International Congress Series 1213. Amsterdam: Elsevier Science, p. 215–23

  18. H Ohara T Nakazawa H Sano T Ando T Okamoto H Takada et al. (2005) ArticleTitleSystemic extrapancreatic lesions associated with autoimmune pancreatitis Pancreas 31 232–7 Occurrence Handle16163054 Occurrence Handle10.1097/01.mpa.0000175178.85786.1d

    Article  PubMed  Google Scholar 

  19. H Hamano S Kawa A Horiuchi H Unno N Furuya T Akamatsu et al. (2001) ArticleTitleHigh serum IgG4 concentrations in patients with sclerosing pancreatitis N Engl J Med 344 732–8 Occurrence Handle11236777 Occurrence Handle10.1056/NEJM200103083441005 Occurrence Handle1:CAS:528:DC%2BD3MXitFamt7k%3D

    Article  PubMed  CAS  Google Scholar 

  20. M Yamamoto S Harada M Ohara C Suzuki Y Naishiro H Yamamoto et al. (2005) ArticleTitleClinical and pathological differences between Mikulicz's disease and Sjogren's syndrome Rheumatology 44 227–34 Occurrence Handle15509627 Occurrence Handle10.1093/rheumatology/keh447 Occurrence Handle1:STN:280:DC%2BD2M%2FlsVSlsw%3D%3D

    Article  PubMed  CAS  Google Scholar 

  21. S Kitagawa Y Zen K Harada M Sasaki Y Sato H Minato et al. (2005) ArticleTitleAbundant IgG4-positive plasma cell infiltration characterizes chronic sclerosing sialadenitis (Kuttner's tumor) Am J Surg Pathol 29 783–91 Occurrence Handle15897744 Occurrence Handle10.1097/01.pas.0000164031.59940.fc

    Article  PubMed  Google Scholar 

  22. K Notohara LJ Burgart D Yadav S Chari TC Smyrk (2003) ArticleTitleIdiopathic chronic pancreatitis with periductal lymphoplasmacytic infiltration Am J Surg Pathol 27 1119–27 Occurrence Handle12883244 Occurrence Handle10.1097/00000478-200308000-00009

    Article  PubMed  Google Scholar 

  23. G Zamboni J Luttges P Capelli L Frulloni G Cavallini P Pederzoli et al. (2004) ArticleTitleHistopathological features of diagnostic and clinical relevance in autoimmune pancreatitis: a study on 53 resection specimens and 9 biopsy specimens Virchows Arch 445 552–63 Occurrence Handle15517359 Occurrence Handle10.1007/s00428-004-1140-z

    Article  PubMed  Google Scholar 

  24. S Aoki T Nakazawa H Ohara H Sano H Nakao T Joh et al. (2005) ArticleTitleImmunohistochemical study of autoimmune pancreatitis using anti-IgG4 antibody and patient's sera Histopathology 47 147–58 Occurrence Handle16045775 Occurrence Handle10.1111/j.1365-2559.2005.02204.x Occurrence Handle1:STN:280:DC%2BD2Mzoslyguw%3D%3D

    Article  PubMed  CAS  Google Scholar 

  25. Y Yoshimura S Takeda Y Ieki E Takazakura H Koizumi K Takagawa (2006) ArticleTitleIgG4-associated prostatitis complicating autoimmune pancreatitis Intern Med 45 IssueID15 897–901 Occurrence Handle16946571 Occurrence Handle10.2169/internalmedicine.45.1752

    Article  PubMed  Google Scholar 

  26. T Taniguchi A Hamasaki M Okamoto (2006) ArticleTitleA case of suspected lymphocytic hypophysitis and organizing pneumonia during maintenance therapy for autoimmune pancreatitis associated with autoimmune thrombocytopenia Endocr J 53 563–6 Occurrence Handle16849834 Occurrence Handle10.1507/endocrj.K05-179

    Article  PubMed  Google Scholar 

  27. T Nakazawa H Ohara H Sano T Andoh H Imai H Takada et al. (2007) ArticleTitleDifficulty in diagnosing autoimmune pancreatitis by imaging findings Gastrointest Endosc 65 99–108 Occurrence Handle17185087 Occurrence Handle10.1016/j.gie.2006.03.929

    Article  PubMed  Google Scholar 

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Authors and Affiliations

  1. Department of Internal Medicine and Bioregulation, Nagoya City University Graduate School of Medical Sciences, 1 Kawasumi, Mizuho-cho, Mizuho-ku, Nagoya, Aichi, 467-8601, Japan

    Hirotaka Ohara, Takahiro Nakazawa, Tomoaki Ando & Takashi Joh

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  1. Hirotaka Ohara
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  2. Takahiro Nakazawa
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  3. Tomoaki Ando
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  4. Takashi Joh
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Ohara, H., Nakazawa, T., Ando, T. et al. Systemic extrapancreatic lesions associated with autoimmune pancreatitis. J Gastroenterol 42 (Suppl 18), 15–21 (2007). https://doi.org/10.1007/s00535-007-2045-9

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