Abdominal Imaging

, Volume 37, Issue 2, pp 261–274

Immunological diseases of the pancreatico-hepatobiliary system: update on etiopathogenesis and cross-sectional imaging findings

  • Deepak Garg
  • Arpit Nagar
  • Shaile Philips
  • Naoki Takahashi
  • Srinivasa R. Prasad
  • Alampady K. Shanbhogue
  • Dushyant V. Sahani
Article

Abstract

Immunological diseases of the hepatobiliary system and the pancreas include a broad spectrum of disorders that manifest characteristic histopathology/serology and variable clinical features and imaging findings. Recent studies have thrown fresh light on the complex role of genetics and autoimmunity in the pathogenesis and natural history of these diverse disorders that include autoimmune hepatitis, primary biliary cirrhosis, primary sclerosing cholangitis, IgG4-related cholangitis, overlap/outlier syndromes, and autoimmune pancreatitis.

Key words

Autoimmune hepatitis Primary biliary cirrhosis Primary sclerosing cholangitis IgG-4-related disease Computed tomography Magnetic resonance imaging 

References

  1. 1.
    Alpini G, Roberts S, Kuntz SM, et al. (1996) Morphological, molecular, and functional heterogeneity of cholangiocytes from normal rat liver. Gastroenterology 110:1636–1643PubMedCrossRefGoogle Scholar
  2. 2.
    Hirschfield G, Heathcote J, Gershwin M (2010) Pathogenesis of cholestatic liver disease and therapeutic approaches. Gastroenterology 139:1481–1496PubMedCrossRefGoogle Scholar
  3. 3.
    Manns MP, Strassburg CP (2001) Autoimmune hepatitis: clinical challenges. Gastroenterology 120:1502–1517PubMedCrossRefGoogle Scholar
  4. 4.
    Belle SH, Beringer KC, Detre KM (1995) An update on liver transplantation in the United States: recipient characteristics and outcome. Clin Transpl 19–33Google Scholar
  5. 5.
    Boberg KM, Aadland E, Jahnsen J, et al. (1998) Incidence and prevalence of primary biliary cirrhosis, primary sclerosing cholangitis, and autoimmune hepatitis in a Norwegian population. Scand J Gastroenterol 33:99–103PubMedCrossRefGoogle Scholar
  6. 6.
    Longhi MS, Ma Y, Mieli-Vergani G, Vergani D (2010) Aetiopathogenesis of autoimmune hepatitis. J Autoimmun 34:7–14PubMedCrossRefGoogle Scholar
  7. 7.
    Manns MP, Czaja AJ, et al. (2010) Diagnosis and management of autoimmune hepatitis. Hepatology 51(6):2193–2213PubMedCrossRefGoogle Scholar
  8. 8.
    Czaja AJ (2005) Current concepts in autoimmune hepatitis. Ann Hepatol 4:6–24PubMedGoogle Scholar
  9. 9.
    Czaja AJ, Manns MP (2010) Advances in the diagnosis, pathogenesis, and management of autoimmune hepatitis. Gastroenterology 139(1):58–72PubMedCrossRefGoogle Scholar
  10. 10.
    Sahni VA, Raghunathan G, Mearadji B, et al. (2010) Autoimmune hepatitis: CT and MR imaging features with histopathological correlation. Abdom Imaging 35:75–84PubMedCrossRefGoogle Scholar
  11. 11.
    Bilaj F, Hyslop WB, Rivero H, et al. (2005) MR imaging findings in autoimmune hepatitis: correlation with clinical staging. Radiology 236:896–902PubMedCrossRefGoogle Scholar
  12. 12.
    Park SZ, Nagorney DM, Czaja AJ (2000) Hepatocellular carcinoma in autoimmune hepatitis. Dig Dis Sci 45:1944–1948PubMedCrossRefGoogle Scholar
  13. 13.
    Wong RJ, Gish R, Frederick T, Bzowej N, Frenette C (2011) The development of hepatocellular carcinoma in autoimmune hepatitis patients. A case series. Dig Dis Sci 56(2):276–278CrossRefGoogle Scholar
  14. 14.
    Sanchez-Urdazpal L, Czaja AJ, van Hoek B, Krom RA, Wiesner RH (1992) Prognostic features and role of liver transplantation in severe corticosteroid-treated autoimmune chronic active hepatitis. Hepatology 15:215–221PubMedCrossRefGoogle Scholar
  15. 15.
    Crosignani A, Battezzati PM, Invernizzi P, et al. (2008) Clinical features and management of primary biliary cirrhosis. World J Gastroenterol 14:3313–3327PubMedCrossRefGoogle Scholar
  16. 16.
    Lleo A, Shimoda S, Ishibashi H, Gershwin ME (2011) Primary biliary cirrhosis and autoimmune hepatitis: apotopes and epitopes. J Gastroenterol 46(1):29–38PubMedCrossRefGoogle Scholar
  17. 17.
    Bogdanos DP, Mieli-Vergani G, Vergani D (2009) Autoantibodies and their antigens in autoimmune hepatitis. Semin Liver Dis 29:241–253PubMedCrossRefGoogle Scholar
  18. 18.
    Lindor KD, Gershwin ME, Poupon R, et al. (2009) Primary biliary cirrhosis. Hepatology 50:291–308PubMedCrossRefGoogle Scholar
  19. 19.
    Hirschfield GM, Liu X, Xu C, et al. (2009) Primary biliary cirrhosis associated with HLA, IL12A, and IL12RB2 variants. N Engl J Med 360:2544–2555PubMedCrossRefGoogle Scholar
  20. 20.
    Jones DE (2007) Pathogenesis of primary biliary cirrhosis. Gut 56:1615–1624PubMedGoogle Scholar
  21. 21.
    Kumagi T, Heathcote EJ (2008) Primary biliary cirrhosis. Orphanet J Rare Dis 3:1PubMedCrossRefGoogle Scholar
  22. 22.
    Kobayashi S, Matsui O, Gabata T, et al. (2005) MRI findings of primary biliary cirrhosis: correlation with Scheuer histologic staging. Abdom Imaging 30:71–76PubMedCrossRefGoogle Scholar
  23. 23.
    Wenzel JS, Donohoe A, Ford KL III, et al. (2001) Primary biliary cirrhosis: MR imaging findings and description of MR imaging periportal halo sign. AJR Am J Roentgenol 176:885–889PubMedGoogle Scholar
  24. 24.
    Blachar A, Federle MP, Brancatelli G (2001) Primary biliary cirrhosis: clinical, pathologic, and helical CT findings in 53 patients. Radiology 220:329–336PubMedGoogle Scholar
  25. 25.
    Fattovich G, Stroffolini T, Zagni I, Donato F (2004) Hepatocellular carcinoma in cirrhosis: incidence and risk factors. Gastroenterology 127:S35–S50PubMedCrossRefGoogle Scholar
  26. 26.
    Washington MK (2007) Autoimmune liver disease: overlap and outliers. Mod Pathol 20(Suppl 1):S15–S30PubMedCrossRefGoogle Scholar
  27. 27.
    Chapman R, Fevery J, Kalloo A, et al. (2010) Diagnosis and management of primary sclerosing cholangitis. Hepatology 51:660–678PubMedCrossRefGoogle Scholar
  28. 28.
    Mendes F, Lindor KD (2010) Primary sclerosing cholangitis: overview and update. Nat Rev Gastroenterol Hepatol 7(11):611–619PubMedGoogle Scholar
  29. 29.
    Aron JH, Bowlus CL (2009) The immunobiology of primary sclerosing cholangitis. Semin Immunopathol 31:383–397PubMedCrossRefGoogle Scholar
  30. 30.
    Juran BD, Lazaridis KN (2006) Genetics of hepatobiliary diseases. Clin Gastroenterol Hepatol 4:548–557PubMedCrossRefGoogle Scholar
  31. 31.
    Karlsen T, Schrumpf E, Boberg KM (2010) Primary sclerosing cholangitis. Best Pract Res Clin Gastroenterol 24(5):655–666PubMedCrossRefGoogle Scholar
  32. 32.
    Silveira MG, Lindor KD (2008) Primary sclerosing cholangitis. Can J Gastroenterol 22:689–698PubMedGoogle Scholar
  33. 33.
    Broome U, Glaumann H, Hellers G, et al. (1994) Liver disease in ulcerative colitis: an epidemiological and follow up study in the county of Stockholm. Gut 35:84–89PubMedCrossRefGoogle Scholar
  34. 34.
    Pokorny CS, McCaughan GW, Gallagher ND, Selby WS (1992) Sclerosing cholangitis and biliary tract calculi—primary or secondary? Gut 33:1376–1380PubMedCrossRefGoogle Scholar
  35. 35.
    LaRusso NF, Shneider BL, Black D, et al. (2006) Primary sclerosing cholangitis: summary of a workshop. Hepatology 44:746–764PubMedCrossRefGoogle Scholar
  36. 36.
    Beuers U, Rust C (2005) Overlap syndromes. Semin Liver Dis 25:311–320PubMedCrossRefGoogle Scholar
  37. 37.
    Ben-Ari Z, Czaja AJ (2001) Autoimmune hepatitis and its variant syndromes. Gut 49:589–594PubMedCrossRefGoogle Scholar
  38. 38.
    Hyslop WB, Kierans AS, Leonardou P, et al. (2010) Overlap syndrome of autoimmune chronic liver diseases: MRI findings. J Magn Reson Imaging 31:383–389PubMedCrossRefGoogle Scholar
  39. 39.
    Yoshida K, Toki F, Takeuchi T, et al. (1995) Chronic pancreatitis caused by an autoimmune abnormality. Proposal of the concept of autoimmune pancreatitis. Dig Dis Sci 40:1561–1568PubMedCrossRefGoogle Scholar
  40. 40.
    Takahashi N, Kawashima A, Fletcher JG, Chari ST (2007) Renal involvement in patients with autoimmune pancreatitis: CT and MR imaging findings. Radiology 242:791–801PubMedCrossRefGoogle Scholar
  41. 41.
    Sato Y, Notohara K, Kojima M, et al. (2010) IgG4-related disease: historical overview and pathology of hematological disorders. Pathol Int 60:247–258PubMedCrossRefGoogle Scholar
  42. 42.
    Okazaki K, Uchida K, Miyoshi H, et al. (2010) Recent concepts of autoimmune pancreatitis and IgG4-related disease. Clin Rev Allergy Immunol. doi:10.1007/S12016-010-8214-2
  43. 43.
    Takuma K, Kamisawaa T, Igarashi Y (2011) Autoimmune pancreatitis and IgG4-related sclerosing cholangitis. Curr Opin Rheumatol 23:80–87PubMedCrossRefGoogle Scholar
  44. 44.
    Pezzilli R, Labate A (2010) The concept of autoimmune pancreatitis and its immunological background. Expert Rev Clin Immunol 6(1):125–136PubMedCrossRefGoogle Scholar
  45. 45.
    Manfredi R, Graziani R, Cicero C, et al. (2008) Autoimmune pancreatitis: CT patterns and their changes after steroid treatment. Radiology 247:435–443PubMedCrossRefGoogle Scholar
  46. 46.
    Sah RP, Chari ST, Pannala R, et al. (2010) Differences in clinical profile and relapse rate of type 1 versus type 2 autoimmune pancreatitis. Gastroenterology 139(1):140–148 (quiz e112–e143)PubMedCrossRefGoogle Scholar
  47. 47.
    Bodily K, Takahashi N, Fletcher J, et al. (2009) Autoimmune pancreatitis: pancreatic and extrapancreatic imaging findings. AJR Am J Roentgenol 192:431–437PubMedCrossRefGoogle Scholar
  48. 48.
    Gerstenmaier JF, Malone DE (2010) Mass lesions in chronic pancreatitis: benign or malignant? An “evidence-based practice approach”. Abdom Imaging. doi:10.1007/s00261-010-9658-9
  49. 49.
    Yang DH, Kim KW, Kim TK, et al. (2006) Autoimmune pancreatitis: radiologic findings in 20 patients. Abdom Imaging 31:94–102PubMedCrossRefGoogle Scholar
  50. 50.
    Sahani DV, Sainani NI, Deshpande V, et al. (2009) Autoimmune pancreatitis: disease evolution, staging, response assessment, and CT features that predict response to corticosteroid therapy. Radiology 250:118–129PubMedCrossRefGoogle Scholar
  51. 51.
    Law R, Bronner M, Vogt D, Stevens T (2009) Autoimmune pancreatitis: a mimic of pancreatic cancer. Clevel Clin J Med 76:607–615CrossRefGoogle Scholar
  52. 52.
    Kim H, Chung M, Chung J (2010) Remission and relapse of autoimmune pancreatitis: focusing on corticosteroid treatment. Pancreas 39(5):555–560PubMedCrossRefGoogle Scholar
  53. 53.
    Kamisawa T, Tsuruta K, Okamoto A, et al. (2009) Frequent and significant K-ras mutation in the pancreas, the bile duct, and the gallbladder in autoimmune pancreatitis. Pancreas 38:890–895PubMedCrossRefGoogle Scholar
  54. 54.
    Forcione D, Brugge W (2010) New kid on the block? Autoimmune pancreatitis. Best Pract Res Clin Gastroenterol 24:361–378PubMedCrossRefGoogle Scholar

Copyright information

© Springer Science+Business Media, LLC 2011

Authors and Affiliations

  • Deepak Garg
    • 1
  • Arpit Nagar
    • 2
  • Shaile Philips
    • 1
  • Naoki Takahashi
    • 3
  • Srinivasa R. Prasad
    • 4
  • Alampady K. Shanbhogue
    • 1
  • Dushyant V. Sahani
    • 5
  1. 1.Department of RadiologyUniversity of Texas Health Science CenterSan AntonioUSA
  2. 2.Department of RadiologyOhio State University Medical CenterColumbusUSA
  3. 3.Department of RadiologyMayo ClinicRochesterUSA
  4. 4.Department of RadiologyUniversity of Texas MD Anderson Cancer CenterHoustonUSA
  5. 5.Department of RadiologyMassachusetts General HospitalBostonUSA

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