Skip to main content
Top
Published in: Abdominal Radiology 2/2012

01-04-2012

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

Authors: Deepak Garg, Arpit Nagar, Shaile Philips, Naoki Takahashi, Srinivasa R. Prasad, Alampady K. Shanbhogue, Dushyant V. Sahani

Published in: Abdominal Radiology | Issue 2/2012

Login to get access

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.
Literature
1.
go back to reference Alpini G, Roberts S, Kuntz SM, et al. (1996) Morphological, molecular, and functional heterogeneity of cholangiocytes from normal rat liver. Gastroenterology 110:1636–1643PubMedCrossRef Alpini G, Roberts S, Kuntz SM, et al. (1996) Morphological, molecular, and functional heterogeneity of cholangiocytes from normal rat liver. Gastroenterology 110:1636–1643PubMedCrossRef
2.
go back to reference Hirschfield G, Heathcote J, Gershwin M (2010) Pathogenesis of cholestatic liver disease and therapeutic approaches. Gastroenterology 139:1481–1496PubMedCrossRef Hirschfield G, Heathcote J, Gershwin M (2010) Pathogenesis of cholestatic liver disease and therapeutic approaches. Gastroenterology 139:1481–1496PubMedCrossRef
3.
go back to reference Manns MP, Strassburg CP (2001) Autoimmune hepatitis: clinical challenges. Gastroenterology 120:1502–1517PubMedCrossRef Manns MP, Strassburg CP (2001) Autoimmune hepatitis: clinical challenges. Gastroenterology 120:1502–1517PubMedCrossRef
4.
go back to reference Belle SH, Beringer KC, Detre KM (1995) An update on liver transplantation in the United States: recipient characteristics and outcome. Clin Transpl 19–33 Belle SH, Beringer KC, Detre KM (1995) An update on liver transplantation in the United States: recipient characteristics and outcome. Clin Transpl 19–33
5.
go back to reference 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 33:99–103PubMedCrossRef 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 33:99–103PubMedCrossRef
6.
go back to reference Longhi MS, Ma Y, Mieli-Vergani G, Vergani D (2010) Aetiopathogenesis of autoimmune hepatitis. J Autoimmun 34:7–14PubMedCrossRef Longhi MS, Ma Y, Mieli-Vergani G, Vergani D (2010) Aetiopathogenesis of autoimmune hepatitis. J Autoimmun 34:7–14PubMedCrossRef
7.
go back to reference Manns MP, Czaja AJ, et al. (2010) Diagnosis and management of autoimmune hepatitis. Hepatology 51(6):2193–2213PubMedCrossRef Manns MP, Czaja AJ, et al. (2010) Diagnosis and management of autoimmune hepatitis. Hepatology 51(6):2193–2213PubMedCrossRef
8.
go back to reference Czaja AJ (2005) Current concepts in autoimmune hepatitis. Ann Hepatol 4:6–24PubMed Czaja AJ (2005) Current concepts in autoimmune hepatitis. Ann Hepatol 4:6–24PubMed
9.
go back to reference Czaja AJ, Manns MP (2010) Advances in the diagnosis, pathogenesis, and management of autoimmune hepatitis. Gastroenterology 139(1):58–72PubMedCrossRef Czaja AJ, Manns MP (2010) Advances in the diagnosis, pathogenesis, and management of autoimmune hepatitis. Gastroenterology 139(1):58–72PubMedCrossRef
10.
go back to reference Sahni VA, Raghunathan G, Mearadji B, et al. (2010) Autoimmune hepatitis: CT and MR imaging features with histopathological correlation. Abdom Imaging 35:75–84PubMedCrossRef Sahni VA, Raghunathan G, Mearadji B, et al. (2010) Autoimmune hepatitis: CT and MR imaging features with histopathological correlation. Abdom Imaging 35:75–84PubMedCrossRef
11.
go back to reference Bilaj F, Hyslop WB, Rivero H, et al. (2005) MR imaging findings in autoimmune hepatitis: correlation with clinical staging. Radiology 236:896–902PubMedCrossRef Bilaj F, Hyslop WB, Rivero H, et al. (2005) MR imaging findings in autoimmune hepatitis: correlation with clinical staging. Radiology 236:896–902PubMedCrossRef
12.
go back to reference Park SZ, Nagorney DM, Czaja AJ (2000) Hepatocellular carcinoma in autoimmune hepatitis. Dig Dis Sci 45:1944–1948PubMedCrossRef Park SZ, Nagorney DM, Czaja AJ (2000) Hepatocellular carcinoma in autoimmune hepatitis. Dig Dis Sci 45:1944–1948PubMedCrossRef
13.
go back to reference 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):276–278CrossRef 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):276–278CrossRef
14.
go back to reference 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 15:215–221PubMedCrossRef 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 15:215–221PubMedCrossRef
15.
go back to reference Crosignani A, Battezzati PM, Invernizzi P, et al. (2008) Clinical features and management of primary biliary cirrhosis. World J Gastroenterol 14:3313–3327PubMedCrossRef Crosignani A, Battezzati PM, Invernizzi P, et al. (2008) Clinical features and management of primary biliary cirrhosis. World J Gastroenterol 14:3313–3327PubMedCrossRef
16.
go back to reference Lleo A, Shimoda S, Ishibashi H, Gershwin ME (2011) Primary biliary cirrhosis and autoimmune hepatitis: apotopes and epitopes. J Gastroenterol 46(1):29–38PubMedCrossRef Lleo A, Shimoda S, Ishibashi H, Gershwin ME (2011) Primary biliary cirrhosis and autoimmune hepatitis: apotopes and epitopes. J Gastroenterol 46(1):29–38PubMedCrossRef
17.
go back to reference Bogdanos DP, Mieli-Vergani G, Vergani D (2009) Autoantibodies and their antigens in autoimmune hepatitis. Semin Liver Dis 29:241–253PubMedCrossRef Bogdanos DP, Mieli-Vergani G, Vergani D (2009) Autoantibodies and their antigens in autoimmune hepatitis. Semin Liver Dis 29:241–253PubMedCrossRef
18.
19.
go back to reference Hirschfield GM, Liu X, Xu C, et al. (2009) Primary biliary cirrhosis associated with HLA, IL12A, and IL12RB2 variants. N Engl J Med 360:2544–2555PubMedCrossRef Hirschfield GM, Liu X, Xu C, et al. (2009) Primary biliary cirrhosis associated with HLA, IL12A, and IL12RB2 variants. N Engl J Med 360:2544–2555PubMedCrossRef
20.
go back to reference Jones DE (2007) Pathogenesis of primary biliary cirrhosis. Gut 56:1615–1624PubMed Jones DE (2007) Pathogenesis of primary biliary cirrhosis. Gut 56:1615–1624PubMed
22.
go back to reference Kobayashi S, Matsui O, Gabata T, et al. (2005) MRI findings of primary biliary cirrhosis: correlation with Scheuer histologic staging. Abdom Imaging 30:71–76PubMedCrossRef Kobayashi S, Matsui O, Gabata T, et al. (2005) MRI findings of primary biliary cirrhosis: correlation with Scheuer histologic staging. Abdom Imaging 30:71–76PubMedCrossRef
23.
go back to reference 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 176:885–889PubMed 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 176:885–889PubMed
24.
go back to reference Blachar A, Federle MP, Brancatelli G (2001) Primary biliary cirrhosis: clinical, pathologic, and helical CT findings in 53 patients. Radiology 220:329–336PubMed Blachar A, Federle MP, Brancatelli G (2001) Primary biliary cirrhosis: clinical, pathologic, and helical CT findings in 53 patients. Radiology 220:329–336PubMed
25.
go back to reference Fattovich G, Stroffolini T, Zagni I, Donato F (2004) Hepatocellular carcinoma in cirrhosis: incidence and risk factors. Gastroenterology 127:S35–S50PubMedCrossRef Fattovich G, Stroffolini T, Zagni I, Donato F (2004) Hepatocellular carcinoma in cirrhosis: incidence and risk factors. Gastroenterology 127:S35–S50PubMedCrossRef
26.
go back to reference Washington MK (2007) Autoimmune liver disease: overlap and outliers. Mod Pathol 20(Suppl 1):S15–S30PubMedCrossRef Washington MK (2007) Autoimmune liver disease: overlap and outliers. Mod Pathol 20(Suppl 1):S15–S30PubMedCrossRef
27.
go back to reference Chapman R, Fevery J, Kalloo A, et al. (2010) Diagnosis and management of primary sclerosing cholangitis. Hepatology 51:660–678PubMedCrossRef Chapman R, Fevery J, Kalloo A, et al. (2010) Diagnosis and management of primary sclerosing cholangitis. Hepatology 51:660–678PubMedCrossRef
28.
go back to reference Mendes F, Lindor KD (2010) Primary sclerosing cholangitis: overview and update. Nat Rev Gastroenterol Hepatol 7(11):611–619PubMed Mendes F, Lindor KD (2010) Primary sclerosing cholangitis: overview and update. Nat Rev Gastroenterol Hepatol 7(11):611–619PubMed
29.
go back to reference Aron JH, Bowlus CL (2009) The immunobiology of primary sclerosing cholangitis. Semin Immunopathol 31:383–397PubMedCrossRef Aron JH, Bowlus CL (2009) The immunobiology of primary sclerosing cholangitis. Semin Immunopathol 31:383–397PubMedCrossRef
30.
go back to reference Juran BD, Lazaridis KN (2006) Genetics of hepatobiliary diseases. Clin Gastroenterol Hepatol 4:548–557PubMedCrossRef Juran BD, Lazaridis KN (2006) Genetics of hepatobiliary diseases. Clin Gastroenterol Hepatol 4:548–557PubMedCrossRef
31.
go back to reference Karlsen T, Schrumpf E, Boberg KM (2010) Primary sclerosing cholangitis. Best Pract Res Clin Gastroenterol 24(5):655–666PubMedCrossRef Karlsen T, Schrumpf E, Boberg KM (2010) Primary sclerosing cholangitis. Best Pract Res Clin Gastroenterol 24(5):655–666PubMedCrossRef
32.
go back to reference Silveira MG, Lindor KD (2008) Primary sclerosing cholangitis. Can J Gastroenterol 22:689–698PubMed Silveira MG, Lindor KD (2008) Primary sclerosing cholangitis. Can J Gastroenterol 22:689–698PubMed
33.
go back to reference 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 35:84–89PubMedCrossRef 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 35:84–89PubMedCrossRef
34.
go back to reference Pokorny CS, McCaughan GW, Gallagher ND, Selby WS (1992) Sclerosing cholangitis and biliary tract calculi—primary or secondary? Gut 33:1376–1380PubMedCrossRef Pokorny CS, McCaughan GW, Gallagher ND, Selby WS (1992) Sclerosing cholangitis and biliary tract calculi—primary or secondary? Gut 33:1376–1380PubMedCrossRef
35.
go back to reference LaRusso NF, Shneider BL, Black D, et al. (2006) Primary sclerosing cholangitis: summary of a workshop. Hepatology 44:746–764PubMedCrossRef LaRusso NF, Shneider BL, Black D, et al. (2006) Primary sclerosing cholangitis: summary of a workshop. Hepatology 44:746–764PubMedCrossRef
37.
38.
go back to reference Hyslop WB, Kierans AS, Leonardou P, et al. (2010) Overlap syndrome of autoimmune chronic liver diseases: MRI findings. J Magn Reson Imaging 31:383–389PubMedCrossRef Hyslop WB, Kierans AS, Leonardou P, et al. (2010) Overlap syndrome of autoimmune chronic liver diseases: MRI findings. J Magn Reson Imaging 31:383–389PubMedCrossRef
39.
go back to reference 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 40:1561–1568PubMedCrossRef 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 40:1561–1568PubMedCrossRef
40.
go back to reference Takahashi N, Kawashima A, Fletcher JG, Chari ST (2007) Renal involvement in patients with autoimmune pancreatitis: CT and MR imaging findings. Radiology 242:791–801PubMedCrossRef Takahashi N, Kawashima A, Fletcher JG, Chari ST (2007) Renal involvement in patients with autoimmune pancreatitis: CT and MR imaging findings. Radiology 242:791–801PubMedCrossRef
41.
go back to reference Sato Y, Notohara K, Kojima M, et al. (2010) IgG4-related disease: historical overview and pathology of hematological disorders. Pathol Int 60:247–258PubMedCrossRef Sato Y, Notohara K, Kojima M, et al. (2010) IgG4-related disease: historical overview and pathology of hematological disorders. Pathol Int 60:247–258PubMedCrossRef
42.
43.
go back to reference Takuma K, Kamisawaa T, Igarashi Y (2011) Autoimmune pancreatitis and IgG4-related sclerosing cholangitis. Curr Opin Rheumatol 23:80–87PubMedCrossRef Takuma K, Kamisawaa T, Igarashi Y (2011) Autoimmune pancreatitis and IgG4-related sclerosing cholangitis. Curr Opin Rheumatol 23:80–87PubMedCrossRef
44.
go back to reference Pezzilli R, Labate A (2010) The concept of autoimmune pancreatitis and its immunological background. Expert Rev Clin Immunol 6(1):125–136PubMedCrossRef Pezzilli R, Labate A (2010) The concept of autoimmune pancreatitis and its immunological background. Expert Rev Clin Immunol 6(1):125–136PubMedCrossRef
45.
go back to reference Manfredi R, Graziani R, Cicero C, et al. (2008) Autoimmune pancreatitis: CT patterns and their changes after steroid treatment. Radiology 247:435–443PubMedCrossRef Manfredi R, Graziani R, Cicero C, et al. (2008) Autoimmune pancreatitis: CT patterns and their changes after steroid treatment. Radiology 247:435–443PubMedCrossRef
46.
go back to reference 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)PubMedCrossRef 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)PubMedCrossRef
47.
go back to reference Bodily K, Takahashi N, Fletcher J, et al. (2009) Autoimmune pancreatitis: pancreatic and extrapancreatic imaging findings. AJR Am J Roentgenol 192:431–437PubMedCrossRef Bodily K, Takahashi N, Fletcher J, et al. (2009) Autoimmune pancreatitis: pancreatic and extrapancreatic imaging findings. AJR Am J Roentgenol 192:431–437PubMedCrossRef
48.
go back to reference 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 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.
go back to reference Yang DH, Kim KW, Kim TK, et al. (2006) Autoimmune pancreatitis: radiologic findings in 20 patients. Abdom Imaging 31:94–102PubMedCrossRef Yang DH, Kim KW, Kim TK, et al. (2006) Autoimmune pancreatitis: radiologic findings in 20 patients. Abdom Imaging 31:94–102PubMedCrossRef
50.
go back to reference 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 250:118–129PubMedCrossRef 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 250:118–129PubMedCrossRef
51.
go back to reference Law R, Bronner M, Vogt D, Stevens T (2009) Autoimmune pancreatitis: a mimic of pancreatic cancer. Clevel Clin J Med 76:607–615CrossRef Law R, Bronner M, Vogt D, Stevens T (2009) Autoimmune pancreatitis: a mimic of pancreatic cancer. Clevel Clin J Med 76:607–615CrossRef
52.
go back to reference Kim H, Chung M, Chung J (2010) Remission and relapse of autoimmune pancreatitis: focusing on corticosteroid treatment. Pancreas 39(5):555–560PubMedCrossRef Kim H, Chung M, Chung J (2010) Remission and relapse of autoimmune pancreatitis: focusing on corticosteroid treatment. Pancreas 39(5):555–560PubMedCrossRef
53.
go back to reference 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 38:890–895PubMedCrossRef 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 38:890–895PubMedCrossRef
54.
go back to reference Forcione D, Brugge W (2010) New kid on the block? Autoimmune pancreatitis. Best Pract Res Clin Gastroenterol 24:361–378PubMedCrossRef Forcione D, Brugge W (2010) New kid on the block? Autoimmune pancreatitis. Best Pract Res Clin Gastroenterol 24:361–378PubMedCrossRef
Metadata
Title
Immunological diseases of the pancreatico-hepatobiliary system: update on etiopathogenesis and cross-sectional imaging findings
Authors
Deepak Garg
Arpit Nagar
Shaile Philips
Naoki Takahashi
Srinivasa R. Prasad
Alampady K. Shanbhogue
Dushyant V. Sahani
Publication date
01-04-2012
Publisher
Springer-Verlag
Published in
Abdominal Radiology / Issue 2/2012
Print ISSN: 2366-004X
Electronic ISSN: 2366-0058
DOI
https://doi.org/10.1007/s00261-011-9759-0

Other articles of this Issue 2/2012

Abdominal Radiology 2/2012 Go to the issue
Live Webinar | 27-06-2024 | 18:00 (CEST)

Keynote webinar | Spotlight on medication adherence

Live: Thursday 27th June 2024, 18:00-19:30 (CEST)

WHO estimates that half of all patients worldwide are non-adherent to their prescribed medication. The consequences of poor adherence can be catastrophic, on both the individual and population level.

Join our expert panel to discover why you need to understand the drivers of non-adherence in your patients, and how you can optimize medication adherence in your clinics to drastically improve patient outcomes.

Prof. Kevin Dolgin
Prof. Florian Limbourg
Prof. Anoop Chauhan
Developed by: Springer Medicine
Obesity Clinical Trial Summary

At a glance: The STEP trials

A round-up of the STEP phase 3 clinical trials evaluating semaglutide for weight loss in people with overweight or obesity.

Developed by: Springer Medicine