Abstract
Primary biliary cirrhosis (PBC) is characterized by features consistent with autoimmune aetiological mechanisms. However, the initiating events in autoimmunity, by which tolerance to self-antigens is broken, are still poorly understood. Indeed, as will be considered, characteristic features of infection and autoimmunity are not mutually exclusive. Recent reports increasingly lend support to earlier speculation and hypotheses that proposed involvement of infectious agents in aetiological mechanisms in autoimmunity. Conversely, autoimmune phenomena are associated with certain infections. It has long been suggested that some features of PBC may have more in common with a response to infection than an autoimmune reaction. Evidence for involvement of an infectious agent in the aetiology of PBC will be considered in this chapter.
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References
James OFW. Definition and epidemiology of primary biliary cirrhosis. In: Neuberger JM, editor. Primary Biliary Cirrhosis. Eastbourne, UK: West End Studios; 1999:53–59.
Anand AC, Elias E, Neuberger JM. End-stage primary biliary cirrhosis in a first generation migrant South Asian population. Eur J Gastroenterol Hepatol. 1996;7:663–666.
Triger DR. Primary biliary cirrhosis: an epidemiological study. Br Med J. 1984;281:772–775.
Agarwal K, Jones DEJ, Bassendine MF. Genetic susceptibility to primary biliary cirrhosis. Eur J Gastroenterol Hepatol. 1999;11:603–606.
Fussey SPM, Guest JR, James OFW et al. Identification and analysis of the major M2 autoantigens in PBC. Proc Natl Acad Sci USA. 1988;85:8654–8658.
Coppell RL, McNeilage J, Surh CD et al. Primary structure of the human M2 mitochondrial autoantigen of PBC: dihydrolipoamide acteyltransferase. Proc Natl Acad Sci USA. 1988;85:7317–7321.
Leung PSC, Van de Water J, Coppel RL et al. Molecular characterisation of the mitochondrial autoantigens in primary biliary cirrhosis. Immunol Res 1991;10:518–543.
Joplin R, Johnson GD, Matthews JB et al. Distribution of pyruvate dehydrogenase dihydrolipoamide acetyltransferase (PDC-E2) and another mitochondrial marker in salivary gland and biliary epithelium from patients with primary biliary cirrhosis. Hepatol. 1994;19:1375–1380.
Joplin R, Wallace LL, Johnson GD et al. Subcellular localisation of pyruvate dheydrogenase dihydrolipoamide acetyltransferase in human intrahepatic biliary epithelial cells. J Pathol. 1995;176:381–390.
Van de Water J, Ansari AA, Surh CD et al. Evidence for the targeting by 2-oxo-dehydrogenase enzymes in the T-cell response of primary biliary cirrhosis. J Immunol. 1991;146:89–94.
Joplin R, Wallace LL, Lindsay JG et al. The human biliary epithelial cell plasma membrane antigen in primary biliary cirrhosis: pyruvate dehydrogenase X? Gastroenterology. 1997;113:1727–1733.
Ayres RCS, Neuberger JM, Shaw J et al. Intercellular-adhesion molecule and MHC antigens on human intrahepatic bile-duct cells-effect of pro-inflammatory cytokines. Gut. 1993;9:1245–1249.
Saidman S, Duquesnoy RJ, Zeevi A et al. Recognition of major histocompatibility complex antigens on cultured human biliary epithelial cells by alloreactive lymphocytes. Hepatology. 1991;13:239–246.
Grotzinger T, Sternsdorf T, Jensen K, Will H. Interferon-modulated expression of genes encoding the nuclear-dot-associated proteins Sp100 and promyelocytic leukemia protein (PML). Eur J Biochem. 1996;238:554–560.
Harada K, Van de Water J, Leung PSC et al. In situnucleic acid hybridization of cytokines in primary biliary cirrhosis: predominance of the Th1 subset. Hepatology. 1997;25:791–796.
Shindo M, Mullin G, Braun-Elwert L et al. Cytokine mRNA expression in the liver of patients with primary biliary cirrhosis (PBC) and chronic hepatitis B (CHB). Clin Exp Immunol. 1996;105:254–259.
Ascher NL. The role of the host immune state in recurrent hepatitis C. Liver Transplant Surg. 1997;3:179–180.
Sadamoto T, Neuberger JM, Joplin R. Effect of pro-inflammatory cytokines on human biliary epithelial cells (BEC): interleukin-1 up-regulates pyruvate dehydrogenase E2/X (PDC-E2/X). Hepatology. 1997;26:1264 (abstract).
Leuschner U. Medical treatment of vanishing bile duct syndrome. In: Alvaro D, Benedetti A, Strazzabosco M. editors. Vanishing Bile Duct Syndrome. Pathophysiology and Treatment. Lancaster: Kluwer; 1997;213–223.
Neuberger JM. Primary biliary cirrhosis transplantation and recurrent disease. In: Lindor KD, Heathcote JE, Poupon R, editors. Primary Biliary Cirrhosis: from Pathogenesis to Clinical Treatment. Lancaster: Kluwer; 1998:155–161.
Dmetrewski J, Mayer AD, Hubscher SG et al. Histological recurrence of primary biliary cirrhosis after liver transplantation: effect of immunosuppresssion. J Hepatol. 1996;24:253–257.
Van de Water J, Gerson LB, Ferrell LD et al. Immunohistochemical evidence of disease recurrence after liver transplantation for primary biliary cirrhosis. Hepatology. 1996;24:1079–1084.
Jones DEJ, Watt FE, Metcalf JV et al. Familial primary biliary cirrhosis reassessed: a geographically-based population study. J Hepatol. 1999;30:402–407.
Delpre G, Niv Y. Primary biliary cirrhosis occurring in a married couple. J Hepatol. 1998;28:232 (abstract).
Sadomoto T, Joplin R, Keogh A et al. Expression of pyruvate dehydrogenase complex E2 on normal biliary epithelial cells induced by lymph nodes from primary biliary cirrhosis. Lancet. 1998;352:1595–1596.
Klein R, Wiebel M, Engelhart S et al. Sera from patients with tuberculosis recognise the M2a-epitope (E2-subunit of pyruvate dehydrogenase) specific for primary biliary cirrhosis. Clin Exp Immunol. 1993;92:308–316.
Vilagut L, Vila J, Pares A et al. Cross-reactivity of anti-mycobacterium gordonae antibodies with the major mitochondrial autoantigens in primary biliary cirrhosis. J Hepatol. 1994;21:673–677.
O’Donohue J, Fiddler H, Garcia-Barcelo M et al. Mycobacterial DNA not detected in liver sections from patients with primary biliary cirrhosis. J Hepatol. 1998;28:433–438.
Hopf U, Stemerowicz R, Rodloff A et al. Relation between Escherichia coliR (rough)-forms in gut, lipid A and primary biliary cirrhosis. Lancet. 1989;2:1421–1422.
Butler P, Valle F, Hamilton-Miller J et al. M2 mitochondrial antibodies and urinary rough mutant bacteria in patients with primary biliary cirrhosis and recurrent bacteriuria. J Hepatol. 1993;17:408–414.
O’Donohue J, Workman MR, Rolando N et al. Urinary tract infections in primary biliary cirrhosis and other chronic liver diseases. Eur J Clin Microbiol Infect Dis. 1997;16:743–746.
Nickowitz RE, Worman HJ. Autoantibodies from patients with primary biliary cirrhosis recognise a restricted region within the cytoplasmic tail of nuclear-pore membrane glycoprotein gp210. J Exp Med. 1993;6:2237–2242.
Fussey SPM, Lindsay JG, Fuller C et al. Autoantibodies in primary biliary cirrhosis-analysis of reactivity against eukaryotic and prokaryotic 2-oxo acid dehydrogenase complexes. Hepatology. 1991;13:467–474.
Teoh KL, Mackay IR, Rowley MJ et al. Enzyme inhibitory autoantibodies to pyruvate dehydrogenase complex in primary biliary cirrhosis differ for mammalian, yeast and bacterial enzymes: implications for molecular mimicry. Hepatology. 1994;19:1029–1033.
Howell CD, Li J, Chen W. Animal models of primary biliary cirrhosis. In: Lindor KD, Heathcote JE, Poupon R, editors. Primary Biliary Cirrhosis: from Pathogenesis to Clinical Treatment. Lancaster: Kluwer; 1998;53–63.
Hyoty H, Hilttunen M, Lonnrot M. Enterovirus infections and insulin dependent diabetes mellitus-evidence for causality. Clin Diagnostic Virol. 1998;9:77–84.
Andreoletti L, Hober D, Heber-Vandenberghe C et al. Coxsackie B virus infection and beta cell autoantibodies in newly diagnosed IDDM adult patients. Clin Diagnostic Virol. 1998; 9:125–133.
Sekigawa I, Kaneko H, Hishikawa T et al. HIV infection and SLE: their pathogenic relationship. Clin Exp Rheumatol. 1998;6:175–180.
Mason AL, Xu LZ, Guo LS et al. Detection of retroviral antibodies in primary biliary cirrhosis and other idiopathic biliary disorders. Lancet. 1998;351:1620–1624.
Xu L, Guo L, Joplin R et al. A viral etiology of primary biliary cirrhosis. Hepatology. 1997;26:1250 (abstract).
Joplin R, Neuberger JM. Immunopathology of primary biliary cirrhosis. Eur J Gastroenterol Hepatol. 1999;11:587–593.
Picker LJ, Treer JR, Ferguson-Darnell JR et al. Control of lymphocyte recirculation in man. Differential regulation of peripheral lymph node homing receptor L-selectin on T-cells during virgin to memory transition. J Immunol. 1993; 150:1105–1121.
Leon MP, Spickett G, Jones DEJ et al. CD4+ T-cell subsets defined by isoforms of CD45 in primary biliary cirrhosis. Clin Exp Immunol. 1995;99:233–239.
Joplin R, Lindsay JG, Hubscher SG et al. Distribution of dihydrolipoamide acetyltransferase (E2) in the liver and portal lymph nodes of patients with primary biliary cirrhosis: an immunohistochemical study. Hepatology. 1991;14:442–447.
Leon MP, Bassendine MF, Wilson JL et al. Immunogenicity of biliary epithelium: investigation of antigen presentation to CD4+ T-cells. Hepatology. 1996;24:561–567.
Jones DEJ, Palmer JM, Yeaman SJ et al. T-cell responses to native human proteins in primary biliary cirrhosis. Clin Exp Immunol. 1997;107:562–568.
Van de Water J, Ansari A, Prindiville T et al. Heterogeneity of autoreactive T-cell clones specific for the E2 component of the pyruvate dehydrogenase complex in primary biliary cirrhosis. J Exp Med. 1995;181:723–733.
Shimoda S, Nakamura M, Ishibishi H et al. HLA DRB4 0101-restricted immunodominant T-cell autoepitope of pyruvate dehydrogenase complex in primary biliary cirrhosis: evidence of molecular mimicry in human autoimmune disease. J Exp Med. 1995;181:1835–1845.
Anderson KL, Anderson G, Michell RH et al. Intracellular signalling pathways involved in the induction of apoptosis in immature thymic T-lymphocytes. J Immunol. 1996; 11:4083–4091.
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Joplin, R. (2000). Thoughts on the infectious aetiology of primary biliary cirrhosis. In: Manns, M.P., Paumgartner, G., Leuschner, U. (eds) Immunology and Liver. Falk Symposium, vol 114. Springer, Dordrecht. https://doi.org/10.1007/978-94-011-4000-3_23
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DOI: https://doi.org/10.1007/978-94-011-4000-3_23
Publisher Name: Springer, Dordrecht
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