2013, Number 1
<< Back Next >>
Ann Hepatol 2013; 12 (1)
Autoimmune hepatitis type-2 and Epstein-Barr virus infection in a toddler: art of facts or an artifact?
Zellos A, Spoulou V, Roma-Giannikou E, Karentzou O, Dalekos GN, Theodoridou M
Language: English
References: 27
Page: 147-151
PDF size: 124.69 Kb.
ABSTRACT
Epstein-Barr virus (EBV) can cause frequently asymptomatic (or anicteric) and self-limited hepatitis, while
occasionally may result in considerable cholestatic hepatitis. Herein, we describe the case of a previously
healthy toddler (26 month old girl) with prolonged cholestasis, elevated serum transaminases, EBV serology
compatible with recent EBV infection and positive anti liver kidney microsomal antibody type 1 which is
characteristic of new-onset autoimmune hepatitis type 2. Liver biopsy was also typical of autoimmune hepatitis
as attested by the presence of portal inflammation with predominant T-lymphocytes and plasma cells
and interface hepatitis. Persistent EBV-related hepatitis was excluded by the absence of viral inclusions and
steatosis on liver specimens and negative liver EBV-PCR. In conclusion, our case strongly suggests that in
children with prolonged cholestatic hepatitis, positive EBV serology cannot exclude the presence of other
causes of liver disease. In this context, autoimmune hepatitis should be considered as an alternate diagnosis,
particularly when there is specific liver-related autoantibody detection. In such conditions, liver biopsy
seems mandatory in an attempt to achieve a correct and timely diagnosis of a potentially catastrophic
disease as autoimmune hepatitis. Although some cases of autoimmune hepatitis type 1 following EBV infection
have been reported in adults, to the best of our knowledge, the present case of autoimmune hepatitis
type 2 after EBV infection represents the first case in children ever reported in the English literature.
REFERENCES
Shaukat A, Tsai HT, Rutherford R, Anania FA. Epstein-Barr virus induced hepatitis: An important cause of cholestasis. Hepatol Res 2005; 33: 24-6.
Niller HH, Wolf H, Minarovits J. Regulation and dysregulation of Epstein-Barr virus latency: implications for the development of autoimmune diseases. Autoimmunity 2008; 41: 298-328.
Vento S, Cainelli F. Is there a role for viruses in triggering autoimmune hepatitis? Autoimmunity Rev 2004; 3: 61-9.
Dalekos GN, Zachou K, Liaskos C, Gatselis N. Autoantibodies and defined target autoantigens in autoimmune hepatitis: an overview. Eur J Intern Med 2002; 13: 293-303.
Hennes EM, Zeniya M, Czaja AJ, Pares A, Dalekos GN, Krawitt EL, Bittencourt PL, et al. International Autoimmune Hepatitis Group. Simplified criteria for the diagnosis of autoimmune hepatitis. Hepatology 2008; 48: 169-76.
Zachou K, Gatselis N, Papadamou G, Rigopoulou EI, Dalekos GN. Mycophenolate for the treatment of autoimmune hepatitis: prospective assessment of its efficacy and safety for induction and maintenance of remission in a large cohort of treatment-naïve patients. J Hepatol 2011; 55: 636-46.
McClain MT, Poole BD, Bruner BF, Kaufman KM, Harley JB, James JA. An altered immune response to Epstein-Barr nuclear antigen 1 in pediatric systemic lupus erythematosus. Arthritis Rheum 2006; 54: 360-8.
Sevilla J, del Carmen Escudero M, Jiménez R, González-Vicent M, Manzanares J, García-Novo D, Madero L. Severe systemic autoimmune disease associated with Epstein- Barr virus infection. J Pediatr Hematol Oncol 2004; 26: 831-3.
Mieli-Vergani G, Heller S, Jara P, Vergani D, Chang MH, Fujisawa T, González-Peralta RP, et al. Autoimmune hepatitis. J Pediatr Gastroenterol Nutr 2009; 49: 158-64.
Gatselis NK, Georgiadou SP, Tassopoulos N, Zachou K, Liaskos C, Hatzakis A, Dalekos GN. Impact of parietal cell autoantibodies and non-organ-specific autoantibodies on the treatment outcome of patients with hepatitis C virus infection: a pilot study. World J Gastroenterol 2005; 11: 482-7.
Renaudineau Y, Dalekos GN, Gueguen P, Zachou K, Youinou P. Anti-α-actinin antibodies cross-react with anti-ss- DNA antibodies in active autoimmune hepatitis. Clin Rev Allergy Immunol 2008; 34: 321-5.
Knodell RG, Ishak KG, Black WC, Chen TS, Craig R, Kaplowitz N, Kiernan TW, et al. Formulation and application of a numerical scoring system for assessing histological activity in asymptomatic chronic active hepatitis. Hepatology 1981; 1: 431-5.
Alvarez F, Berg PA, Bianchi FB, Bianchi L, Burroughs AK, Cancado EL, Chapman RW, et al. International Autoimmune Hepatitis Group Report: review criteria for diagnosis of autoimmune hepatitis. J Hepatol 1999; 31: 929-38.
Ma Y, Bogdanos DP, Hussain MJ, Underhill J, Bansal S, Longhi MS, Cheeseman P, et al. Polyclonal T-cell responses to cytochrome P450IID6 are associated with disease activity in autoimmune hepatitis type 2. Gastroenterology 2006; 130: 868-82.
Pando M, Larriba J, Fernandez GC, Fainboim H, Ciocca M, Ramonet M, Badia I, et al. Pediatric and adult forms of type I autoimmune hepatitis in Argentina: evidence for differential genetic predisposition. Hepatology 1999; 30: 1374-80.
Czaja AJ, Souto EO, Bittencourt PL, Cancado EL, Porta G, Goldberg AC, Donaldson PT. Clinical distinctions and pathogenic implications of type 1 autoimmune hepatitis in Brazil and the United States.
Papamichalis PA, Zachou K, Koukoulis GK, Veloni A, Karacosta EG, Kypri L, Mamaloudis I, et al. The revised international autoimmune hepatitis score in chronic liver diseases including autoimmune hepatitis/overlap syndromes and autoimmune hepatitis with concurrent other liver disorders. J Autoimmune Dis 2007; 4: 3.
Gatselis NK, Zachou K, Papamichalis P, Koukoulis GK, Gabeta S, Dalekos GN, Rigopoulou EI. Comparison of simplified score with the revised original score for the diagnosis of autoimmune hepatitis: a new or a complementary diagnostic score? Dig Liver Dis 2010; 42: 807-12.
Gregorio GV, Portmann B, Reid F, Donaldson PT, Doherty DG, McCartney M, Mowat AP, et al. Autoimmune hepatitis in childhood: a 20 year experience. Hepatology 1997; 25: 541-7.
Bogdanos DP, Dalekos GN. Enzymes as target antigens of liver-specific autoimmunity: the case of cytochromes P450s. Curr Med Chem 2008; 15: 2285-92.
Vento S, Guella L, Mirandola F, Cainelli, Di Perri G, Solbiati M, Ferraro T, et al. Epstein-Barr virus as a trigger for autoimmune hepatitis in susceptible individuals. Lancet 1995; 346: 608-9.
Cabibi D. Autoimmune hepatitis following Epstein-Barr virus infection. BMJ Case Rep 2008; 2008: bcr0620080071.
Dalekos GN, Wedemeyer H, Obermayer-Straub P, Kayser A, Barut A, Frank H, Manns MP. Epitope mapping of cytochrome P4502D6 autoantigen in patients with chronic hepatitis C during alpha-interferon treatment. J Hepatol 1999; 30: 366-75.
Vento S, Cainelli F, Renzini C, Concia E. Autoimmune hepatitis type 2 induced by HCV and persisting after viral clearance. Lancet 1997; 350: 12.
Nakajima S, Umebayashi H, Kurosawa R, Imagawa T, Katakura S, Mori M, Aihara Y, et al. A case of autoimmune hepatitis needed to be differentiated from EBV hepatitis, in that the histology of liver biopsy specimen was useful for diagnosis. Nihon Rinsho Meneki Gakkai Kaishi 2005; 28: 154-8.
Rigopoulou EI, Dalekos GN. Autoimmune hepatitis: of host and pathogen. Hepatology 2008; 47: 2147-8.
Pender MP. Infection of autoreactive B lymphocytes with EBV, causing chronic autoimmune diseases. Trends Immunol 2003; 24: 584-8.