Skip to main content
Top
Published in: Abdominal Radiology 1/2017

01-01-2017 | Invited Article

Imaging of autoimmune hepatitis and overlap syndromes

Authors: Neera Malik, Sudhakar K. Venkatesh

Published in: Abdominal Radiology | Issue 1/2017

Login to get access

Abstract

Autoimmune hepatitis (AIH) is an uncommon, chronic inflammatory, and relapsing liver disease of unknown origin that may lead to liver cirrhosis, hepatocellular carcinoma, liver transplantation, or death. AIH occurs in all age groups and races but can frequently manifest as acute fulminant hepatitis. Clinical presentation of AIH can have features similar to primary sclerosing cholangitis (PSC) and primary biliary cirrhosis (PBC), and these diseases may coexist leading to overlap syndromes. Although histological diagnosis is necessary, imaging features often can demonstrate characteristics that may be helpful to distinguish these diseases. Imaging features of AIH are those of chronic liver disease, and imaging plays important role in detection of complications and ruling out other possible causes of chronic liver disease. Emerging techniques such as elastography provide non-invasive options for diagnosis of significant fibrosis and cirrhosis during clinical follow-up as well as assessment of response to treatment. In this study, we will describe imaging findings in AIH and overlap syndromes.
Literature
1.
go back to reference Ilyas JA, O’Mahony CA, Vierling JM (2011) Liver transplantation in autoimmune liver diseases. Best Pract Res Clin Gastroenterol 25:765–782CrossRefPubMed Ilyas JA, O’Mahony CA, Vierling JM (2011) Liver transplantation in autoimmune liver diseases. Best Pract Res Clin Gastroenterol 25:765–782CrossRefPubMed
2.
go back to reference Bader TR, Beavers KL, Semelka RC (2003) MR imaging features of primary sclerosing cholangitis: patterns of cirrhosis in relationship to clinical severity of disease. Radiology 226:675–685CrossRefPubMed Bader TR, Beavers KL, Semelka RC (2003) MR imaging features of primary sclerosing cholangitis: patterns of cirrhosis in relationship to clinical severity of disease. Radiology 226:675–685CrossRefPubMed
3.
go back to reference Bilaj F, Hyslop WB, Rivero H, et al. (2005) MR imaging findings in autoimmune hepatitis: correlation with clinical staging. Radiology 236:896–902CrossRefPubMed Bilaj F, Hyslop WB, Rivero H, et al. (2005) MR imaging findings in autoimmune hepatitis: correlation with clinical staging. Radiology 236:896–902CrossRefPubMed
4.
go back to reference Kobayashi S, Matsui O, Gabata T, et al. (2004) MRI findings of primary biliary cirrhosis: correlation with Scheuer histologic staging. Abdom Imaging 30:71–76CrossRef Kobayashi S, Matsui O, Gabata T, et al. (2004) MRI findings of primary biliary cirrhosis: correlation with Scheuer histologic staging. Abdom Imaging 30:71–76CrossRef
5.
go back to reference Wenzel JS, Donohoe A, Ford KL 3rd, et al. (2001) Primary biliary cirrhosis: MR imaging findings and description of MR imaging periportal halo sign. Am J Roentgenol 176:885–889CrossRef Wenzel JS, Donohoe A, Ford KL 3rd, et al. (2001) Primary biliary cirrhosis: MR imaging findings and description of MR imaging periportal halo sign. Am J Roentgenol 176:885–889CrossRef
6.
go back to reference Grunwald D, Kothari D, Malik R (2014) Noninvasive markers in the assessment and management of autoimmune liver diseases. Eur J Gastroenterol Hepatol 26:1065–1072CrossRefPubMed Grunwald D, Kothari D, Malik R (2014) Noninvasive markers in the assessment and management of autoimmune liver diseases. Eur J Gastroenterol Hepatol 26:1065–1072CrossRefPubMed
8.
go back to reference Corpechot C, Gaouar F, El Naggar A, et al. (2014) Baseline values and changes in liver stiffness measured by transient elastography are associated with severity of fibrosis and outcomes of patients with primary sclerosing cholangitis. Gastroenterology 146:970–979 (quiz e15-6) Corpechot C, Gaouar F, El Naggar A, et al. (2014) Baseline values and changes in liver stiffness measured by transient elastography are associated with severity of fibrosis and outcomes of patients with primary sclerosing cholangitis. Gastroenterology 146:970–979 (quiz e15-6)
9.
go back to reference Corpechot C, Carrat F, Poujol-Robert A, et al. (2012) Noninvasive elastography-based assessment of liver fibrosis progression and prognosis in primary biliary cirrhosis. Hepatology 56:198–208CrossRefPubMed Corpechot C, Carrat F, Poujol-Robert A, et al. (2012) Noninvasive elastography-based assessment of liver fibrosis progression and prognosis in primary biliary cirrhosis. Hepatology 56:198–208CrossRefPubMed
10.
go back to reference Poupon R, Corpechot C (2011) Elastography-based assessment of primary biliary cirrhosis staging. Dig Liver Dis 43:839–840CrossRefPubMed Poupon R, Corpechot C (2011) Elastography-based assessment of primary biliary cirrhosis staging. Dig Liver Dis 43:839–840CrossRefPubMed
11.
go back to reference Lewin M, Vilgrain V, Ozenne V, et al. (2009) Prevalence of sclerosing cholangitis in adults with autoimmune hepatitis: a prospective magnetic resonance imaging and histological study. Hepatology 50:528–537CrossRefPubMed Lewin M, Vilgrain V, Ozenne V, et al. (2009) Prevalence of sclerosing cholangitis in adults with autoimmune hepatitis: a prospective magnetic resonance imaging and histological study. Hepatology 50:528–537CrossRefPubMed
12.
go back to reference Eaton JE, Dzyubak B, Venkatesh SK, et al. (2016) Performance of magnetic resonance elastography in primary sclerosing cholangitis. J Gastroenterol Hepatol 31:1184–1190CrossRefPubMedPubMedCentral Eaton JE, Dzyubak B, Venkatesh SK, et al. (2016) Performance of magnetic resonance elastography in primary sclerosing cholangitis. J Gastroenterol Hepatol 31:1184–1190CrossRefPubMedPubMedCentral
13.
14.
go back to reference Czaja AJ, Freese DK (2002) Diagnosis and treatment of autoimmune hepatitis. Hepatology 36:479–497CrossRefPubMed Czaja AJ, Freese DK (2002) Diagnosis and treatment of autoimmune hepatitis. Hepatology 36:479–497CrossRefPubMed
15.
go back to reference Montano Loza AJ, Czaja AJ (2007) Current therapy for autoimmune hepatitis. Nat Clin Pract Gastroenterol Hepatol 4:202–214CrossRefPubMed Montano Loza AJ, Czaja AJ (2007) Current therapy for autoimmune hepatitis. Nat Clin Pract Gastroenterol Hepatol 4:202–214CrossRefPubMed
16.
go back to reference Wang Q, Yang F, Miao Q, et al. (2016) The clinical phenotypes of autoimmune hepatitis: a comprehensive review. J Autoimmun 66:98–107CrossRefPubMed Wang Q, Yang F, Miao Q, et al. (2016) The clinical phenotypes of autoimmune hepatitis: a comprehensive review. J Autoimmun 66:98–107CrossRefPubMed
17.
go back to reference Muratori P, Czaja AJ, Muratori L, et al. (2005) Genetic distinctions between autoimmune hepatitis in Italy and North America. World J Gastroenterol 11:1862–1866CrossRefPubMedPubMedCentral Muratori P, Czaja AJ, Muratori L, et al. (2005) Genetic distinctions between autoimmune hepatitis in Italy and North America. World J Gastroenterol 11:1862–1866CrossRefPubMedPubMedCentral
18.
go back to reference Bjornsson E, Talwalkar J, Treeprasertsuk S, et al. (2010) Drug-induced autoimmune hepatitis: clinical characteristics and prognosis. Hepatology 51:2040–2048CrossRefPubMed Bjornsson E, Talwalkar J, Treeprasertsuk S, et al. (2010) Drug-induced autoimmune hepatitis: clinical characteristics and prognosis. Hepatology 51:2040–2048CrossRefPubMed
19.
go back to reference Liberal R, Krawitt EL, Vierling JM, et al. (2016) Cutting edge issues in autoimmune hepatitis. J Autoimmun 75:6–19CrossRefPubMed Liberal R, Krawitt EL, Vierling JM, et al. (2016) Cutting edge issues in autoimmune hepatitis. J Autoimmun 75:6–19CrossRefPubMed
21.
go back to reference Liberal R, Grant CR, Longhi MS, et al. (2014) Diagnostic criteria of autoimmune hepatitis. Autoimmun Rev 13:435–440CrossRefPubMed Liberal R, Grant CR, Longhi MS, et al. (2014) Diagnostic criteria of autoimmune hepatitis. Autoimmun Rev 13:435–440CrossRefPubMed
22.
go back to reference Kogan J, Safadi R, Ashur Y, et al. (2002) Prognosis of symptomatic versus asymptomatic autoimmune hepatitis: a study of 68 patients. J Clin Gastroenterol 35:75–81CrossRefPubMed Kogan J, Safadi R, Ashur Y, et al. (2002) Prognosis of symptomatic versus asymptomatic autoimmune hepatitis: a study of 68 patients. J Clin Gastroenterol 35:75–81CrossRefPubMed
23.
go back to reference Manns MP, Czaja AJ, Gorham JD, et al. (2010) Diagnosis and management of autoimmune hepatitis. Hepatology 51:2193–2213CrossRefPubMed Manns MP, Czaja AJ, Gorham JD, et al. (2010) Diagnosis and management of autoimmune hepatitis. Hepatology 51:2193–2213CrossRefPubMed
24.
go back to reference Hoeroldt B, McFarlane E, Dube A, et al. (2011) Long-term outcomes of patients with autoimmune hepatitis managed at a nontransplant center. Gastroenterology 140:1980–1989CrossRefPubMed Hoeroldt B, McFarlane E, Dube A, et al. (2011) Long-term outcomes of patients with autoimmune hepatitis managed at a nontransplant center. Gastroenterology 140:1980–1989CrossRefPubMed
25.
go back to reference Gatselis NK, Zachou K, Koukoulis GK, et al. (2015) Autoimmune hepatitis, one disease with many faces: etiopathogenetic, clinico-laboratory and histological characteristics. World J Gastroenterol 21:60–83CrossRefPubMedPubMedCentral Gatselis NK, Zachou K, Koukoulis GK, et al. (2015) Autoimmune hepatitis, one disease with many faces: etiopathogenetic, clinico-laboratory and histological characteristics. World J Gastroenterol 21:60–83CrossRefPubMedPubMedCentral
26.
go back to reference Gregorio GV, Portmann B, Reid F, et al. (1997) Autoimmune hepatitis in childhood: a 20-year experience. Hepatology 25:541–547CrossRefPubMed Gregorio GV, Portmann B, Reid F, et al. (1997) Autoimmune hepatitis in childhood: a 20-year experience. Hepatology 25:541–547CrossRefPubMed
27.
go back to reference Teufel A, Weinmann A, Kahaly GJ, et al. (2010) Concurrent autoimmune diseases in patients with autoimmune hepatitis. J Clin Gastroenterol 44:208–213CrossRefPubMed Teufel A, Weinmann A, Kahaly GJ, et al. (2010) Concurrent autoimmune diseases in patients with autoimmune hepatitis. J Clin Gastroenterol 44:208–213CrossRefPubMed
28.
go back to reference Czaja AJ (2013) Review article: the management of autoimmune hepatitis beyond consensus guidelines. Aliment Pharmacol Ther 38:343–364CrossRefPubMed Czaja AJ (2013) Review article: the management of autoimmune hepatitis beyond consensus guidelines. Aliment Pharmacol Ther 38:343–364CrossRefPubMed
29.
go back to reference Czaja AJ (2009) Rapidity of treatment response and outcome in type 1 autoimmune hepatitis. J Hepatol 51:161–167CrossRefPubMed Czaja AJ (2009) Rapidity of treatment response and outcome in type 1 autoimmune hepatitis. J Hepatol 51:161–167CrossRefPubMed
30.
go back to reference Montano-Loza AJ, Carpenter HA, Czaja AJ (2007) Features associated with treatment failure in type 1 autoimmune hepatitis and predictive value of the model of end-stage liver disease. Hepatology 46:1138–1145CrossRefPubMed Montano-Loza AJ, Carpenter HA, Czaja AJ (2007) Features associated with treatment failure in type 1 autoimmune hepatitis and predictive value of the model of end-stage liver disease. Hepatology 46:1138–1145CrossRefPubMed
31.
go back to reference Tripathi D, Neuberger J (2009) Autoimmune hepatitis and liver transplantation: indications, results, and management of recurrent disease. Semin Liver Dis 2009:286–296CrossRef Tripathi D, Neuberger J (2009) Autoimmune hepatitis and liver transplantation: indications, results, and management of recurrent disease. Semin Liver Dis 2009:286–296CrossRef
33.
go back to reference Vukotic R, Vitale G, D’Errico-Grigioni A, et al. (2016) De novo autoimmune hepatitis in liver transplant: state-of-the-art review. World J Gastroenterol 22:2906–2914CrossRefPubMedPubMedCentral Vukotic R, Vitale G, D’Errico-Grigioni A, et al. (2016) De novo autoimmune hepatitis in liver transplant: state-of-the-art review. World J Gastroenterol 22:2906–2914CrossRefPubMedPubMedCentral
34.
go back to reference Czaja AJ (2013) Hepatocellular carcinoma and other malignancies in autoimmune hepatitis. Dig Dis Sci 58:1459–1476CrossRefPubMed Czaja AJ (2013) Hepatocellular carcinoma and other malignancies in autoimmune hepatitis. Dig Dis Sci 58:1459–1476CrossRefPubMed
35.
go back to reference Wang QX, Shen L, Qiu DK, et al. (2011) Validation of transient elastography (Fibroscan) in assessment of hepatic fibrosis in autoimmune hepatitis. Zhonghua Gan Zang Bing Za Zhi 19:782–784PubMed Wang QX, Shen L, Qiu DK, et al. (2011) Validation of transient elastography (Fibroscan) in assessment of hepatic fibrosis in autoimmune hepatitis. Zhonghua Gan Zang Bing Za Zhi 19:782–784PubMed
36.
go back to reference Obara N, Ueno Y, Fukushima K, et al. (2008) Transient elastography for measurement of liver stiffness measurement can detect early significant hepatic fibrosis in Japanese patients with viral and nonviral liver diseases. J Gastroenterol 43:720–728CrossRefPubMed Obara N, Ueno Y, Fukushima K, et al. (2008) Transient elastography for measurement of liver stiffness measurement can detect early significant hepatic fibrosis in Japanese patients with viral and nonviral liver diseases. J Gastroenterol 43:720–728CrossRefPubMed
37.
go back to reference Andersson KL, Salomon JA, Goldie SJ, et al. (2008) Cost effectiveness of alternative surveillance strategies for hepatocellular carcinoma in patients with cirrhosis. Clin Gastroenterol Hepatol 6:1418–1424CrossRefPubMedPubMedCentral Andersson KL, Salomon JA, Goldie SJ, et al. (2008) Cost effectiveness of alternative surveillance strategies for hepatocellular carcinoma in patients with cirrhosis. Clin Gastroenterol Hepatol 6:1418–1424CrossRefPubMedPubMedCentral
39.
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 35:75–84CrossRefPubMed Sahni VA, Raghunathan G, Mearadji B, et al. (2010) Autoimmune hepatitis: CT and MR imaging features with histopathological correlation. Abdom Imaging 35:75–84CrossRefPubMed
40.
go back to reference Qayyum A, Graser A, Westphalen A, et al. (2004) CT of benign hypervascular liver nodules in autoimmune hepatitis. Am J Roentgenol 183:1573–1576CrossRef Qayyum A, Graser A, Westphalen A, et al. (2004) CT of benign hypervascular liver nodules in autoimmune hepatitis. Am J Roentgenol 183:1573–1576CrossRef
41.
go back to reference Cassani F, Valentini P, Cataleta M, et al. (1997) Ultrasound-detected abdominal lymphadenopathy in chronic hepatitis C: high frequency and relationship with viremia. J Hepatol 26:479–483CrossRefPubMed Cassani F, Valentini P, Cataleta M, et al. (1997) Ultrasound-detected abdominal lymphadenopathy in chronic hepatitis C: high frequency and relationship with viremia. J Hepatol 26:479–483CrossRefPubMed
42.
go back to reference Gutkowski K, Hartleb M, Kacperek-Hartleb T, et al. (2013) Laboratory-based scoring system for prediction of hepatic inflammatory activity in patients with autoimmune hepatitis. Liver Int 33:1370–1377CrossRefPubMed Gutkowski K, Hartleb M, Kacperek-Hartleb T, et al. (2013) Laboratory-based scoring system for prediction of hepatic inflammatory activity in patients with autoimmune hepatitis. Liver Int 33:1370–1377CrossRefPubMed
43.
go back to reference Singh S, Venkatesh SK, Wang Z, et al. (2015) Diagnostic performance of magnetic resonance elastography in staging liver fibrosis: a systematic review and meta-analysis of individual participant data. Clin Gastroenterol Hepatol 13(440–451):e6 Singh S, Venkatesh SK, Wang Z, et al. (2015) Diagnostic performance of magnetic resonance elastography in staging liver fibrosis: a systematic review and meta-analysis of individual participant data. Clin Gastroenterol Hepatol 13(440–451):e6
44.
go back to reference Wang J, Malik N, Yin M, et al. (2016) Magnetic resonance elastography in autoimmune hepatitis. World J Gastroenterol Wang J, Malik N, Yin M, et al. (2016) Magnetic resonance elastography in autoimmune hepatitis. World J Gastroenterol
45.
go back to reference Czaja AJ (2013) The overlap syndromes of autoimmune hepatitis. Digest Dis Sci 58:326–343PubMed Czaja AJ (2013) The overlap syndromes of autoimmune hepatitis. Digest Dis Sci 58:326–343PubMed
46.
go back to reference Abdalian R, Dhar P, Jhaveri K, et al. (2008) Prevalence of sclerosing cholangitis in adults with autoimmune hepatitis: evaluating the role of routine magnetic resonance imaging. Hepatology 47:949–957CrossRefPubMed Abdalian R, Dhar P, Jhaveri K, et al. (2008) Prevalence of sclerosing cholangitis in adults with autoimmune hepatitis: evaluating the role of routine magnetic resonance imaging. Hepatology 47:949–957CrossRefPubMed
47.
go back to reference Hunter M, Loughrey MB, Gray M, et al. (2011) Evaluating distinctive features for early diagnosis of primary sclerosing cholangitis overlap syndrome in adults with autoimmune hepatitis. Ulster Med J 80:15–18PubMedPubMedCentral Hunter M, Loughrey MB, Gray M, et al. (2011) Evaluating distinctive features for early diagnosis of primary sclerosing cholangitis overlap syndrome in adults with autoimmune hepatitis. Ulster Med J 80:15–18PubMedPubMedCentral
48.
go back to reference Gheorghe L, Iacob S, Gheorghe C, et al. (2004) Frequency and predictive factors for overlap syndrome between autoimmune hepatitis and primary cholestatic liver disease. Eur J Gastroenterol Hepatol 16:585–592CrossRefPubMed Gheorghe L, Iacob S, Gheorghe C, et al. (2004) Frequency and predictive factors for overlap syndrome between autoimmune hepatitis and primary cholestatic liver disease. Eur J Gastroenterol Hepatol 16:585–592CrossRefPubMed
49.
go back to reference Chazouilleres O, Wendum D, Serfaty L, et al. (1998) Primary biliary cirrhosis-autoimmune hepatitis overlap syndrome: clinical features and response to therapy. Hepatology 28:296–301CrossRefPubMed Chazouilleres O, Wendum D, Serfaty L, et al. (1998) Primary biliary cirrhosis-autoimmune hepatitis overlap syndrome: clinical features and response to therapy. Hepatology 28:296–301CrossRefPubMed
50.
go back to reference Joshi S, Cauch-Dudek K, Wanless IR, et al. (2002) Primary biliary cirrhosis with additional features of autoimmune hepatitis: response to therapy with ursodeoxycholic acid. Hepatology 35:409–413CrossRefPubMed Joshi S, Cauch-Dudek K, Wanless IR, et al. (2002) Primary biliary cirrhosis with additional features of autoimmune hepatitis: response to therapy with ursodeoxycholic acid. Hepatology 35:409–413CrossRefPubMed
51.
go back to reference Czaja AJ (1998) Frequency and nature of the variant syndromes of autoimmune liver disease. Hepatology 28:360–365CrossRefPubMed Czaja AJ (1998) Frequency and nature of the variant syndromes of autoimmune liver disease. Hepatology 28:360–365CrossRefPubMed
52.
go back to reference Boberg KM, Chapman RW, Hirschfield GM, et al. (2011) Overlap syndromes: the International Autoimmune Hepatitis Group (IAIHG) position statement on a controversial issue. J Hepatol 54:374–385CrossRefPubMed Boberg KM, Chapman RW, Hirschfield GM, et al. (2011) Overlap syndromes: the International Autoimmune Hepatitis Group (IAIHG) position statement on a controversial issue. J Hepatol 54:374–385CrossRefPubMed
54.
go back to reference Perdigoto R, Carpenter HA, Czaja AJ (1992) Frequency and significance of chronic ulcerative colitis in severe corticosteroid-treated autoimmune hepatitis. J Hepatol 14:325–331CrossRefPubMed Perdigoto R, Carpenter HA, Czaja AJ (1992) Frequency and significance of chronic ulcerative colitis in severe corticosteroid-treated autoimmune hepatitis. J Hepatol 14:325–331CrossRefPubMed
55.
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 31:383–389CrossRefPubMed Hyslop WB, Kierans AS, Leonardou P, et al. (2010) Overlap syndrome of autoimmune chronic liver diseases: MRI findings. J Magn Reson Imaging 31:383–389CrossRefPubMed
56.
go back to reference Vierling JM (2015) Autoimmune hepatitis and overlap syndromes: diagnosis and management. Clin Gastroenterol Hepatol 13:2088–2108CrossRefPubMed Vierling JM (2015) Autoimmune hepatitis and overlap syndromes: diagnosis and management. Clin Gastroenterol Hepatol 13:2088–2108CrossRefPubMed
57.
go back to reference Liberal R, Grant CR, Mieli-Vergani G, et al. (2013) Autoimmune hepatitis: a comprehensive review. J Autoimmun 41:126–139CrossRefPubMed Liberal R, Grant CR, Mieli-Vergani G, et al. (2013) Autoimmune hepatitis: a comprehensive review. J Autoimmun 41:126–139CrossRefPubMed
58.
go back to reference Zachou K, Muratori P, Koukoulis GK, et al. (2013) Review article: autoimmune hepatitis—current management and challenges. Aliment Pharmacol Ther 38:887–913CrossRefPubMed Zachou K, Muratori P, Koukoulis GK, et al. (2013) Review article: autoimmune hepatitis—current management and challenges. Aliment Pharmacol Ther 38:887–913CrossRefPubMed
Metadata
Title
Imaging of autoimmune hepatitis and overlap syndromes
Authors
Neera Malik
Sudhakar K. Venkatesh
Publication date
01-01-2017
Publisher
Springer US
Published in
Abdominal Radiology / Issue 1/2017
Print ISSN: 2366-004X
Electronic ISSN: 2366-0058
DOI
https://doi.org/10.1007/s00261-016-1019-x

Other articles of this Issue 1/2017

Abdominal Radiology 1/2017 Go to the issue