Autoimmune Liver Diseases in Children. Part I
Authors:
P. Dědek
Authors place of work:
Dětská klinika Fakultní nemocnice, Hradec Králové, přednosta prof. MUDr. M. Bayer, CSc.
Published in the journal:
Čes-slov Pediat 2010; 65 (12): 694-705.
Category:
Review
Summary
Autoimmune liver diseases form a group of chronic inflammatory illnesses associated with serious prognosis. Autoimmune hepatitis, autoimmune sclerosing cholangitis, and primary sclerosing cholangitis are the most common entities seen during childhood. Clinical findings of liver disorder are not typical, even the disease course may be asymptomatic. We can frequently found other illnesses with autoimmune pathogenesis in patients or their first-degree relatives. Laboratory diagnostics is based on detection of different types of autoantibodies, hypergammaglobulinemia, and elevated IgG. The definitive diagnosis is provided by liver biopsy and imaging of biliary tree.
Early and proper diagnosis followed by prompt therapy affects the patient’s prognosis significantly. Corticosteroids alone or paired with azathioprine have proven efficacy, administration of ursodeoxycholic acid is still a matter of debate. Chronic liver failure requiring liver transplant occurs despite treatment in many patients, especially those with sclerosing cholangitis.
Key words:
autoimmune liver disease, autoimmune hepatitis, primary sclerosing cholangitis, autoimmune sclerosing cholangitis, primary biliary cirrhosis, childhood, overlap syndrome
Zdroje
1. Krawitt EL. Autoimmune hepatitis. N. Engl. J. Med. 1996; 11: 897–903.
2. Gregorio GV, Portmann B, Reid F, et al. Autoimmune hepatitis in childhood: a 20-year experience. Hepatology 1997; 25: 541–547.
3. Alvarez F. Autoimmune hepatitis and primary sclerosing cholangitis. Clin. Liver Dis. 2006; 10: 89–107.
4. Czaja AJ. Natural history, clinical features, and treatment of autoimmune hepatitis. Semin. Liver Dis. 1984; 4: 1–12.
5. Saadah OI, Smith AL, Hardikar W. Long-term outcome of autoimmune hepatitis in children. J. Gastroenterol. Hepatol. 2001; 16: 1297–302.
6. Maggiore G, Bernard O, Homberg JC, et al. Liver disease associated with anti-liver-kidney microsome antibody in children. J. Pediatr. 1986; 108: 399–404.
7. Maggiore G, Veber F, Bernard O, et al. Autoimmune hepatitis with anti-actin antibodies in children and adolescents. J. Pediatr. Gastroenterol. Nutr. 1993; 17: 376–381.
8. Vergani D, Alvarez F, Bianchi FB, et al. Liver autoimmune serology: a consensus statement from the committee for autoimmune serology of the International Autoimmune Hepatitis Group. J. Hepatol. 2004; 41: 677–683.
9. Kenny KP, Czaja AJ, Ludwig J, et al. Frequency and significance of antimitochodrial antibodies in severe chronic active hepatitis. Dig. Dis. Sci. 1986; 31: 705–711.
10. Czaja AJ, Carpenter HA. Autoimmune hepatitis with incidental histologic features of bile duct injury. Hepatology 2001; 34: 659–665.
11. Misdraji J, Thiim M, Graeme-Cook FM. Autoimmune hepatitis with centrilobular necrosis. Am. J. Surg. Pathol. 2004; 28: 471–478.
12. Czaja AJ, Carpenter HA. Decreased fibrosis during corticosteroid therapy of autoimmune hepatitis. J. Hepatol. 2004; 40: 646–652.
13. Dufour JF, DeLellis R, Kaplan MM. Reversibility of hepatic fibrosis in autoimmune hepatitis. Ann. Intern. Med. 1997; 127: 981–985.
14. Ferreira AR, Roquete ML, Toppa NH, et al. Effect of treatment of hepatic histopathology in children and adolescents with autoimmune hepatitis. J. Pediatr. Gastroenterol. Nutr. 2008; 46: 65–70.
15. Maddrey WC, Combes B. Therapeutic concepts for the management of idiopathic autoimmune chronic hepatitis. Semin. Liver Dis. 1991; 11: 248–255.
16. Johnson PJ, McFarlane IG. Meeting report: International Autoimmune Hepatitis Group Hepatology 1993; 18: 998–1005.
17. Alvarez F, Berg PA, Bianchi FB, et al. International Autoimmune Hepatitis Group Report: review of criteria for diagnosis of autoimmune hepatitis. J. Hepatol. 1999; 31: 929–938.
18. Ebbeson RL, Screiber RA. Diagnosing autoimmune hepatitis in children: is the International Autoimmune Hepatitis Group scoring systém useful? Clin. Gastroenterol. Hepatol. 2004; 2: 935–940.
19. Smolka V. Autoimunitní hepatitida – použití diagnostického skórovacího systému. Čes.-slov. Pediat. 1996; 51: 200–202.
20. Hennes EM, Zeniva M, Czaja AJ, et al. Simplified criteria for the diagnosis of autoimmune hepatitis. Hepatology 2008; 48: 169–176.
21. Roberts SK, Therneau TM, Czaja AJ. Prognosis of histological cirrhosis in type 1 autoimmune hepatitis. Gastroenterology 1996; 110: 848–857.
22. Czaja AJ, Bayraktar Y. Non-classifical phenotypes of autoimmune hepatitis and advances in diagnosis and treatment. World J. Gastroenterol. 2009; 15: 2314–2328.
23. Ishibashi H, Komori A, Shimoda S, et al. Guidelines for therapy of autoimmune liver disease. Semin. Liver Dis. 2007; 27: 214–226.
24. Czaja AJ, Freese DK. Diagnosis and treatment of autoimmune hepatitis. Hepatology 2002; 36: 479–497.
25. Mieli-Vergani G, Vergani D. Autoimmune paediatric liver disease. World J. Gastroenterol. 2008; 14: 3360–3367.
26. Kerkar N, Annunziato RA, Foley L, et al. Prospective analysis of nonadherence in autoimmune hepatitis: a common proflem. J. Pediatr. Gastroenterol. Nutr. 2006; 43: 629–634.
27. Muratori L, Muratori P, Granito A, et al. Current topics in autoimmune hepatitis. Dig. Liver Dis. 2010; Jul 6 [Epub ahead of print].
28. Manns MP. Recent developments in autoimmune liver diseases. J. Gastroenterol. Hepatol. 1997; 12: S256–S271.
29. Mieli-Vergani G, Heller S, Jara P, et al. Autoimmune hepatitis. J. Pediatr. Gastroenterol. Nutr. 2009; 4: 158–164.
30. Banerjee S, Rahhal R, Bishop WP. Azathioprine monotherapy for maintenance of remission in pediatric patients with autoimmune hepatitis. J. Pediatr. Gastroenterol. Nutr. 2006; 43: 353–356.
31. Alvarez F, Ciocca M, Cañero-Velasco C, et al. Short-term cyclosporine induces a remission of autoimmune hepatitis in children. J. Hepatol. 1999; 30: 222–227.
32. Johnson PJ, McFarlane IG, Williams R. Azathioprine for long-term maintenance of remission in autoimmune hepatitis. N. Engl. J. Med. 1995; 333: 958–963.
33. Debray D, Maggiore G, Girardet JP, et al. Efficacy of cyclosporin A in children with type 2 autoimmune hepatitis. J. Pediatr. 1999; 135: 111–114.
34. Aw MM, Dhawan A, Samyn M, et al. Mycophenolate mofetil as rescue treatment for autoimmune liver disease in children: a 5-year follow-up. J. Hepatol. 2009; 51: 156–160.
35. Devlin SM, Swain MG, Urbanski SJ, et al. Mycophenolate mofetil for the treatment of autoimmune hepatitis in patients refracory to standard therapy. Can. J. Gastroenterol. 2004; 18: 321–326.
36. Strassburg CP, Manns MP. Treatment of autoimmune hepatitis. Semin. Liver Dis. 2009; 29: 273–285.
37. Teufel A, Galle PR, Kanzler S. Update on autoimmune hepatitis. World J. Gastroenterol. 2009; 15: 1035–1041.
38. Manns MP, Vogel A. Autoimmune hepatitis, from mechanisms to therapy. Hepatology 2006; 43: S132–S144.
39. Montano-Loza AJ, Carpenter HA, Czaja AJ. Improving the end point of corticosteroid therapy in type 1 autoimmune hepatitis to reduce the frequency of relapse. J. Gastroenterol. 2007; 102: 1005–1012.
40. Ahmed M, Mutimer D, Hathaway M, et al. Liver transplantation for autoimmune hepatitis: a 12-year experience. Transplant. Proc. 1997; 29: 496.
41. Chai PF, Lee WS, Brown RM, et al. Childhood autoimmune liver disease: indications and outcome of liver transplantation. J. Pediatr. Gastroenterol. Nutr. 2010; 50: 295–302.
42. Ratziu V, Samuel D, Sebagh M, et al. Long term follow-up after liver transplantation for autoimmune hepatitis: evidence of recurrence of primary disease. J. Hepatol. 1999; 30: 131–41.
43. Birnbaum AH, Benkow KJ, Pittman NS, et al. Recurrence of autoimmune hepatitis in children after liver transplantation. J. Pediatr. Gastroenterol. Nutr. 1997; 25: 20–25.
Štítky
Neonatology Paediatrics Pneumology and ftiseology General practitioner for children and adolescents General practitioner for adultsČlánok vyšiel v časopise
Czech-Slovak Pediatrics
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