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Clinicopathological differentiation between sclerosing cholangitis with autoimmune pancreatitis and primary sclerosing cholangitis

  • Liver, Pancreas, and Biliary Tract
  • Published:
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Abstract

Background

The present study was undertaken to identify the clinicopathological differences between sclerosing cholangitis with autoimmune pancreatitis (SC-AIP) and primary sclerosing cholangitis (PSC).

Methods

We retrospectively compared the clinical, cholangiographic, and liver biopsy findings between 24 cases of PSC and 24 cases of SC-AIP.

Results

Patient age at the time of diagnosis was significantly lower in the PSC group than in the SC-AIP group. The peripheral blood eosinophil count was significantly higher in the PSC group than in the SC-AIP group, but the serum IgG4 level was significantly higher in the SC-AIP group. Cholangiography revealed band-like strictures, beaded appearance, and pruned-tree appearance significantly more frequently in PSC, whereas segmental strictures and strictures of the distal third of the common bile duct were significantly more common in SC-AIP. Liver biopsy revealed fibrous obliterative cholangitis only in the PSC specimens. No advanced fibrous change corresponding to Ludwig's stages 3 and 4 was observed in any of the SC-AIP specimens. IgG4-positive plasma cell infiltration of the liver was significantly more severe in SC-AIP than in PSC. Subsequent cholangiography showed no improvement in any of the PSC cases, but all SC-AIP patients responded to steroid therapy, and improvement in the strictures was observed cholangiographically.

Conclusions

Based on the differences between the patients' ages and blood chemistry, cholangiographic, and liver biopsy findings, SC-AIP should be differentiated from PSC.

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References

  1. K Yoshida F Toki T Takeuchi S Watanabe K Shiratori N Hayashi (1995) ArticleTitleChronic pancreatitis caused by an autoimmune abnormality Dig Dis Sci 40 1561–8 Occurrence Handle7628283 Occurrence Handle10.1007/BF02285209 Occurrence Handle1:STN:280:DyaK2MzlsVCqtg%3D%3D

    Article  PubMed  CAS  Google Scholar 

  2. K Okazaki K Uchida M Ohana H Nakase S Uose M Inai et al. (2000) ArticleTitleAutoimmune-related pancreatitis is associated with autoantibodies and a Th1/Th2-type cellular immune response Gastroenterology 118 573–81 Occurrence Handle10702209 Occurrence Handle10.1016/S0016-5085(00)70264-2 Occurrence Handle1:CAS:528:DC%2BD3cXit1Smsb0%3D

    Article  PubMed  CAS  Google Scholar 

  3. 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 

  4. K Kawaguchi M Koike K Tsuruta A Okamoto I Tabata N Fujita (1991) ArticleTitleLymphoplasmacytic sclerosing pancreatitis with cholangitis: a variant of primary sclerosing cholangitis extensively involving pancreas Hum Pathol 22 387–95 Occurrence Handle2050373 Occurrence Handle10.1016/0046-8177(91)90087-6 Occurrence Handle1:STN:280:DyaK3M3mslGgsg%3D%3D

    Article  PubMed  CAS  Google Scholar 

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

    PubMed  CAS  Google Scholar 

  7. T Kuroiwa T Suda T Takahashi H Hirano M Natsui H Motoyama et al. (2002) ArticleTitleBile duct involvement in a case of autoimmune pancreatitis successfully treated with an oral steroid Dig Dis Sci 47 1810–6 Occurrence Handle12184534 Occurrence Handle10.1023/A:1016452813815

    Article  PubMed  Google Scholar 

  8. K Hirano Y Shiratori Y Komatsu N Yamamoto N Sasahira N Toda et al. (2003) ArticleTitleInvolvement of the biliary system in autoimmune pancreatitis: a follow-up study Clin Gastroenterol Hepatol 1 453–64 Occurrence Handle15017645 Occurrence Handle10.1016/S1542-3565(03)00221-0

    Article  PubMed  Google Scholar 

  9. T Nishino F Toki H Oyama I Oi M Kobayashi K Takasaki et al. (2005) ArticleTitleBiliary tract involvement in autoimmune pancreatitis Pancreas 30 76–82 Occurrence Handle15632703

    PubMed  Google Scholar 

  10. 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 cholangitis Gastrointest Endosc 60 937–44 Occurrence Handle15605009 Occurrence Handle10.1016/S0016-5107(04)02229-1

    Article  PubMed  Google Scholar 

  11. T Nakazawa H Ohara H Sano S Aoki S Kobayashi T Okamoto et al. (2005) ArticleTitleClinical differences between primary sclerosing cholangitis and sclerosing cholangitis with autoimmune pancreatitis Pancreas 30 20–4 Occurrence Handle15632695

    PubMed  Google Scholar 

  12. SC Abraham M Cruz-Corea O Argani EE Furth RH Hurban JK Boitnott (2003) ArticleTitleLymphoplasmic chronic cholecystitis and biliary tract disease in a patient with lymphoplasmacytic sclerosing pancreatitis Am J Surg Pathol 27 441–51 Occurrence Handle12657928 Occurrence Handle10.1097/00000478-200304000-00003

    Article  PubMed  Google Scholar 

  13. Y Zen K Harada M Saeki Y Sato K Tsuneyama J Haratake et al. (2004) ArticleTitleIgG4-related sclerosing cholangitis with and without hepatic inflammatory pseudotumor, and sclerosing pancreatitis-associated sclerosing cholangitis Am J Surg Pathol 28 1193–202 Occurrence Handle15316319 Occurrence Handle10.1097/01.pas.0000136449.37936.6c

    Article  PubMed  Google Scholar 

  14. A Horiuchi S Kawa H Hamano M Hayama H Ota K Kiyosawa (2002) ArticleTitleERCP features in 27 patients with autoimmune pancreatitis Gastrointest Endosc 55 494–9 Occurrence Handle11923760

    PubMed  Google Scholar 

  15. P Angulo KP Batts RA Jorgensen NA LaRusso KD Linder (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 Griffioen 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. K Linder NF LaRusso (2003) Primary sclerosing cholangitis ER Schiff MF Sorrell WC Maddrey (Eds) Disease of the liver EditionNumber9th ed JB Lippincott Philadelphia 673–84

    Google Scholar 

  18. K Okazaki S Kawa T Kamisawa S Naruse S Tanaka I Nishimori et al. (2006) ArticleTitleClinical diagnostic criteria of autoimmune pancreatitis: revised proposal J Gastroenterol 41 626–31 Occurrence Handle16932998 Occurrence Handle10.1007/s00535-006-1868-0

    Article  PubMed  Google Scholar 

  19. DA Craig RL MacCarty RH Wiesner PM Gramsch NF LaRusso (1991) ArticleTitlePrimary sclerosing cholangitis: value of cholangiography in determining the prognosis AJR Am J Roentgenol 157 959–64 Occurrence Handle1927817 Occurrence Handle1:STN:280:DyaK38%2FisFOlug%3D%3D

    PubMed  CAS  Google Scholar 

  20. J Ludwig (1989) ArticleTitleSurgical pathology of the syndrome of primary sclerosing cholangitis Am J Surg Pathol 13 43–9 Occurrence Handle2699167

    PubMed  Google Scholar 

  21. H Takikawa Y Takamori A Tanaka H Kurihara Y Nakamura (2004) ArticleTitleAnalysis of 388 cases of primary sclerosing cholangitis in Japan. Presence of a subgroup without pancreatic involvement in older patients Hepatol Res 29 153–9 Occurrence Handle15203079 Occurrence Handle10.1016/j.hepres.2004.03.006

    Article  PubMed  Google Scholar 

  22. U Broome RR Lofberg B Veress LS Eriksson (1995) ArticleTitlePrimary sclerosing cholangitis and ulcerative colitis: evidence for increased neoplastic potential Hepatology 22 1404–8 Occurrence Handle7590655 Occurrence Handle1:STN:280:DyaK28%2FlsV2mtQ%3D%3D

    PubMed  CAS  Google Scholar 

  23. D Kornfeld A Ekbom T Ihre (1997) ArticleTitleIs there an excess risk for colorectal cancer in patients with ulcerative colitis and concomitant primary sclerosing cholangitis? A population-based study Gut 41 522–5 Occurrence Handle9391253 Occurrence Handle1:STN:280:DyaK1c%2Flt1KnsA%3D%3D Occurrence Handle10.1136/gut.41.4.522

    Article  PubMed  CAS  Google Scholar 

  24. N Uchida T Ezaki H Fukuma K Tsustui H Kobara M Matsuoka et al. (2003) ArticleTitleConcomitant colitis associated with primary sclerosing cholangitis J Gastroenterol 38 482–7 Occurrence Handle12768392 Occurrence Handle10.1007/s00535-002-1085-4

    Article  PubMed  Google Scholar 

  25. N Oshitani Y Jinno Y Sawa S Nakamura T Matsumoto S Nishiguchi et al. (2003) ArticleTitleDoes colitis associated with primary sclerosing cholangitis represent an actual subsequela of ulcerative colitis? Hepatogastroenterology 50 1830–5 Occurrence Handle14696415

    PubMed  Google Scholar 

  26. T Nishino F Toki H Oyama K Shimizu K Shiratori (2006) ArticleTitleLong-term outcome of autoimmune pancreatitis after oral prednisolone therapy Intern Med 45 497–501 Occurrence Handle16702740 Occurrence Handle10.2169/internalmedicine.45.1565

    Article  PubMed  Google Scholar 

  27. J Ludwig A Czaja ER Dickson NF LaRusso RH Wiesner (1984) ArticleTitleManifestations of nonsuppurative cholangitis in chronic hepatobiliary diseases: morphologic spectrum, clinical correlations and terminology Liver 4 105–16 Occurrence Handle6727581 Occurrence Handle1:STN:280:DyaL2c3hvVGksw%3D%3D

    PubMed  CAS  Google Scholar 

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Authors and Affiliations

  1. Institute of Gastroenterology, Department of Medicine, Tokyo Women's Medical University, School of Medicine, 8-1 Kawada-cho, Shinjuku-ku, Tokyo, 162-8666, Japan

    Takayoshi Nishino, Etsuko Hashimoto & Keiko Shiratori

  2. Department of Clinical Laboratory, Tokyo Women's Medical University, School of Medicine, Tokyo, Japan

    Hiroyasu Oyama

  3. Toki Clinic, Gunma, Japan

    Fumitake Toki

  4. Institute of Gastroenterology, Department of Endoscopy, Tokyo Women's Medical University, Tokyo, Japan

    Itaru Oi

  5. Department of Pathology, Tokyo Women's Medical University, School of Medicine, Tokyo, Japan

    Makio Kobayashi

Authors
  1. Takayoshi Nishino
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  2. Hiroyasu Oyama
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  3. Etsuko Hashimoto
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  4. Fumitake Toki
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  5. Itaru Oi
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  6. Makio Kobayashi
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  7. Keiko Shiratori
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Nishino, T., Oyama, H., Hashimoto, E. et al. Clinicopathological differentiation between sclerosing cholangitis with autoimmune pancreatitis and primary sclerosing cholangitis. J Gastroenterol 42, 550–559 (2007). https://doi.org/10.1007/s00535-007-2038-8

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  • DOI: https://doi.org/10.1007/s00535-007-2038-8

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