Abstract
Primary biliary cirrhosis (PBC) and autoimmune hepatitis (AIH) differ in clinical, laboratory, and histological features, as well as response to therapy. Some patients with PBC present with characteristic features of AIH; however, there is no formal designation for conditions in which features of PBC and AIH overlap (PBC-AIH overlap syndrome). The pathogenesis of PBC-AIH overlap syndrome is not well understood and its diagnosis is challenging. The International Autoimmune Hepatitis Group (IAIHG) scoring system for the diagnosis of AIH has been widely used to describe PBC-AIH overlap syndrome, but has not been proven to be appropriate for this purpose. Due to the low prevalence of this disorder, recommendations on therapy focus on treatments available for each individual disorder and on findings from retrospective, non-randomized studies. The aim of this report is to provide a current overview of the clinical and histological features, diagnosis, treatment, and prognosis of PBC-AIH overlap syndrome.
Access this chapter
Tax calculation will be finalised at checkout
Purchases are for personal use only
References
Chazouilleres O, Wendum D, Serfaty L et al (1998) Primary biliary cirrhosis- autoimmune hepatitis overlap syndrome; clinical features and response to therapy. Hepatology 28:296–301
Lohse AW, Zum Büschenfelde KH, Franz B et al (1999) Characterization of the overlap syndrome of primary biliary cirrhosis (PBC) and autoimmune hepatitis: evidence for it being a hepatitic form of PBC in genetically susceptible individuals. Hepatology 29:1078–1084
Popper H, Schaffner F (1970) Nonsuppurative destructive chronic cholangitis and chronic hepatitis. Prog Liver Dis 3:336–354
Geubel AP, Baggenstoss AH, Summerskill WH (1976) Responses to treatment can differentiate chronic active liver disease with cholangitic features from the primary biliary cirrhosis syndrome. Gastroenterology 71:444–449
Lindgren S, Glaumann H, Almer S et al (2009) Transitions between variant forms of primary biliary cirrhosis during long-term follow-up. Eur J Intern Med 20:398–402
Poupon R, Chazouilleres O, Corpechot C et al (2006) Development of autoimmune hepatitis in patients with typical primary biliary cirrhosis. Hepatology 44:85–90
Colombato LA, Alvarez F, Côté J et al (1994) Autoimmune cholangiopathy: the result of consecutive primary biliary cirrhosis and autoimmune hepatitis? Gastroenterology 107:1839–1843
Gossard AA, Lindor KD (2007) Development of autoimmune hepatitis in primary biliary cirrhosis. Liver Int 27:1086–1090
Horsmans Y, Piret A, Brenard R et al (1994) Autoimmune chronic active hepatitis responsive to immunosuppressive therapy evolving into a typical primary biliary cirrhosis syndrome: a case report. J Hepatol 21:194–198
Tanaka A, Harada K, Ebinuma H et al (2011) Primary biliary cirrhosis – autoimmune hepatitis overlap syndrome: a rationale for corticosteroids use based on a nation-wide retrospective study in Japan. Hepatol Res 41:877–886
Silveira MG, Talwalkar JA, Angulo P et al (2007) Overlap of autoimmune hepatitis and primary biliary cirrhosis: long-term outcomes. Am J Gastroenterol 102:1244–1250
Neuhauser M, Bjornsson E, Treeprasertsuk S et al (2010) Autoimmune hepatitis-PBC overlap syndrome: a simplified scoring system may assist in the diagnosis. Am J Gastroenterol 105:345–353
Yokokawa J, Saito H, Kanno Y et al (2010) Overlap of primary biliary cirrhosis and autoimmune hepatitis: characteristics, therapy, and long term outcomes. J Gastroenterol Hepatol 25:376–382
Muratori P, Granito A, Pappas G et al (2009) The serological profile of the autoimmune hepatitis/primary biliary cirrhosis overlap syndrome. Am J Gastroenterol 104:1420–1425
Kuiper EM, Zondervan PE, van Buuren HR (2010) Paris criteria are effective in diagnosis of primary biliary cirrhosis and autoimmune hepatitis overlap syndrome. Clin Gastroenterol Hepatol 8:530–534
Yoshioka Y, Taniai M, Hashimoto E, et al (2013) Clinical profile of primary biliary cirrhosis with features of autoimmune hepatitis: Importance of corticosteroid therapy. Hepatol Res. doi:10.1111/hepr.12210
European Association for the Study of the Liver (2009) EASL Clinical Practice Guidelines: management of cholestatic liver diseases. J Hepatol 51:237–267
Lindor KD, Gershwin ME, Poupon R, American Association for Study of Liver Diseases (2009) Primary biliary cirrhosis. Hepatology 50:291–308
Johnson PJ, McFarlane IG (1993) Meeting report: international autoimmune hepatitis group. Hepatology 18:998–1005
Alvarez F, Berg PA, Bianchi FB et al (1999) International Autoimmune Hepatitis Group Report: review of criteria for diagnosis of autoimmune hepatitis. J Hepatol 31:929–938
Talwalkar JA, Keach JC, Angulo P et al (2002) Overlap of autoimmune hepatitis and primary biliary cirrhosis: an evaluation of a modified scoring system. Am J Gastroenterol 97:1191–1197
Lohse AW (2011) Recognizing autoimmune hepatitis: scores help, but no more. J Hepatol 54:193–194
Hennes EM, Zeniya M, Czaja AJ, International Autoimmune Hepatitis Group et al (2008) Simplified diagnostic criteria for autoimmune hepatitis. Hepatology 48:169–176
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 35:409–413
Chazouilleres O, Wendum D, Serfaty L et al (2006) Long term outcome and response to therapy of primary biliary cirrhosis-autoimmune hepatitis overlap syndrome. J Hepatol 44:400–406
Ozaslan E, Ozaslan E, Efe C et al (2010) Therapy response and outcome of overlap syndromes: autoimmune hepatitis and primary biliary cirrhosis compared to autoimmune hepatitis and autoimmune cholangitis. Hepatogastroenterology 57:441–446
Muratori L, Cassani F, Pappas G et al (2002) The hepatitic/cholestatic “overlap” syndrome: an Italian experience. Autoimmunity 35:565–568
Boberg KM, Chapman RW, Hirschfield GM et al (2011) International Autoimmune Hepatitis Group. Overlap syndromes: the International Autoimmune Hepatitis Group (IAIHG) position statement on a controversial issue. J Hepatol 54:374–385
Suzuki Y, Arase Y, Ikeda K, et al (2004) Clinical and pathological characteristics of the autoimmune hepatitis and primary biliary cirrhosis overlap syndrome. J Gastroenterol Hepatol 19:699–706
Scheuer PJ (1967) Primary biliary cirrhosis. Proc R Soc Med 60:1257–1260
Rubin E, Schaffner F, Popper H (1965) Primary biliary cirrhosis. Am J Pathol 46:387–407
Ludwig J, Dickson ER, McDonald GS (1978) Staging of chronic nonsuppurative destructive cholangitis (syndrome of primary biliary cirrhosis). Virchows Arch A 379:103–112
Wiesner RH, LaRusso NF, Ludwig J et al (1985) Comparison of the clinicopathologic features of primary sclerosing cholangitis and primary biliary cirrhosis. Gastroenterology 88:108–114
Nakanuma Y, Zen Y, Harada K et al (2010) Application of a new histological staging and grading system for primary biliary cirrhosis to liver biopsy specimens: interobserver agreement. Pathol Int 60:167–174
Poupon R (2010) Primary biliary cirrhosis: a 2010 update. J Hepatol 52:745–758
Nakanuma Y, Harada K (2011) The role of the pathologist in diagnosing and grading biliary diseases. Clin Res Hepatol Gastroenterol 35:347–352
Poupon R (2003) Autoimmune overlapping syndromes. Clin Liver Dis 7:865–878
Degott C, Zafrani ES, Callard P et al (1999) Histopathological study of primary biliary cirrhosis and the effect of ursodeoxycholic acid treatment on histology progression. Hepatology 29:1007–1012
Poupon RE, Lindor KD, Cauch-Dudek K et al (1997) Combined analysis of randomized controlled trials of ursodeoxycholic acid in primary biliary cirrhosis. Gastroenterology 113:884–890
Cook GC, Mulligan R, Sherlock S (1971) Controlled prospective trial of corticosteroid therapy in active chronic hepatitis. Q J Med 40:159–185
Czaja AJ (1998) Frequency and nature of the variant syndromes of autoimmune liver disease. Hepatology 28:360–365
Corpechot C, Carrat F, Poupon R et al (2002) Primary biliary cirrhosis: incidence and predictive factors of cirrhosis development in ursodiol-treated patients. Gastroenterology 122:652–658
Huet PM, Vincent C, Deslaurier J et al (2008) Portal hypertension and primary biliary cirrhosis: effect of long-term ursodeoxycholic acid treatment. Gastroenterology 135:1552–1560
Mitchison HC, Bassendine MF, Malcolm AJ et al (1989) A pilot, double-blind, controlled 1-year trial of prednisolone treatment in primary biliary cirrhosis: hepatic improvement but greater bone loss. Hepatology 10:420–429
Author information
Authors and Affiliations
Corresponding author
Editor information
Editors and Affiliations
Rights and permissions
Copyright information
© 2014 Springer Japan
About this chapter
Cite this chapter
Abe, K., Takahashi, A., Ohira, H. (2014). Management of the Patients with Feature of Autoimmune Hepatitis. In: Ohira, H. (eds) Autoimmune Liver Diseases. Springer, Tokyo. https://doi.org/10.1007/978-4-431-54789-1_20
Download citation
DOI: https://doi.org/10.1007/978-4-431-54789-1_20
Published:
Publisher Name: Springer, Tokyo
Print ISBN: 978-4-431-54788-4
Online ISBN: 978-4-431-54789-1
eBook Packages: MedicineMedicine (R0)