eMedicine Specialties > Pediatrics: General Medicine > Gastroenterology

Autoimmune Chronic Active Hepatitis: Follow-up

Author: Husam H Sukerek, MD, Consulting Staff, Department of Gastroenterology, Sabine Medical Center
Coauthor(s): Mohammad F El-Baba, MD, Assistant Professor of Pediatrics, Division of Pediatric Gastroenterology, Wayne State University School of Medicine; Divison Chief of Pediatric Gastroenterology, Children's Hospital of Michigan
Contributor Information and Disclosures

Updated: Dec 1, 2008

Follow-up

Further Outpatient Care

  • Perform liver function tests in patients with autoimmune hepatitis (AIH) weekly during the first 6-8 weeks of treatment and then every 2-3 months, based on results.
  • Schedule regular follow-up visits to assess disease activity and to search for signs and symptoms of chronic liver disease.

Complications

  • Cirrhosis and complications of cirrhosis (eg, ascites, coagulopathy, hepatic coma)
  • Portal hypertension
  • Esophageal varices
  • Poor growth and malnutrition

Prognosis

  • Despite an apparent initial response to immunosuppressive therapy, histologic progress may be gradual and require several years.
  • Ferreira et al concluded that immunosuppressive treatment improved the fibrosis scores, with an arrest in progression and no development into cirrhosis.8
  • In a series reported by Gregorio et al in 1997, 70% of children with autoimmune hepatitis type 1 (AIH-1) and 40% of children with autoimmune hepatitis type 2 (AIH-2) developed cirrhosis.3 Of the 52 children, 17% had multiacinar or panacinar collapse with acute liver failure. The patients with the worst prognosis in this study, resulting either in death or liver transplantation, were the children who were young at presentation and who had AIH-2, coagulopathy, high bilirubin counts, and severe initial histologic activity.
  • In general, the following factors are associated with a worse prognosis:
    • Young age at presentation
    • Diagnosis of AIH-2
    • Coagulopathy
    • Severe initial histologic activity

Patient Education

Miscellaneous

Medicolegal Pitfalls

  • Failure to explain to the family the seriousness of autoimmune hepatitis (AIH) and the importance of follow-up visits
  • Failure to diagnose the disease in its early stage, especially when evaluating a patient with jaundice and negative results to hepatitis screening tests
 


More on Autoimmune Chronic Active Hepatitis

Overview: Autoimmune Chronic Active Hepatitis
Differential Diagnoses & Workup: Autoimmune Chronic Active Hepatitis
Treatment & Medication: Autoimmune Chronic Active Hepatitis
Follow-up: Autoimmune Chronic Active Hepatitis
References
[ CLOSE WINDOW ]

References

  1. Czaja AJ, Cassani F, Cataleta M, et al. Frequency and significance of antibodies to actin in type 1 autoimmune hepatitis. Hepatology. Nov 1996;24(5):1068-73. [Medline].

  2. Wen L, Peakman M, Lobo-Yeo A, et al. T-cell-directed hepatocyte damage in autoimmune chronic active hepatitis. Lancet. Dec 22-29 1990;336(8730):1527-30. [Medline].

  3. Gregorio GV, Portmann B, Reid F, et al. Autoimmune hepatitis in childhood: a 20-year experience. Hepatology. Mar 1997;25(3):541-7. [Medline].

  4. Mieli-Vergani G, Vergani D. Autoimmune paediatric liver disease. World J Gastroenterol. Jun 7 2008;14(21):3360-7. [Medline].

  5. Oettinger R, Brunnberg A, Gerner P, et al. Clinical features and biochemical data of Caucasian children at diagnosis ofautoimmune hepatitis. J Autoimmun. Feb 2005;24(1):79-84. [Medline].

  6. Alvarez F, Ciocca M, Canero-Velasco C, et al. Short-term cyclosporine induces a remission of autoimmune hepatitis in children. J Hepatol. Feb 1999;30(2):222-7. [Medline].

  7. Sciveres M, Caprai S, Palla G, et al. Effectiveness and safety of ciclosporin as therapy for autoimmune diseasesof the liver in children and adolescents. Aliment Pharmacol Ther. Jan 15 2004;19(2):209-17. [Medline].

  8. Ferreira AR, Roquete ML, Toppa NH, de Castro LP, Fagundes ED, Penna FJ. Effect of treatment of hepatic histopathology in children and adolescents with autoimmune hepatitis. J Pediatr Gastroenterol Nutr. Jan 2008;46(1):65-70. [Medline].

  9. Bellary S, Schiano T, Hartman G, Black M. Chronic hepatitis with combined features of autoimmune chronic hepatitis and chronic hepatitis C: favorable response to prednisone and azathioprine. Ann Intern Med. Jul 1 1995;123(1):32-4. [Medline].

  10. Czaja AJ. Drug therapy in the management of type 1 autoimmune hepatitis. Drugs. Jan 1999;57(1):49-68. [Medline].

  11. Debray D, Maggiore G, Girardet JP, et al. Efficacy of cyclosporin A in children with type 2 autoimmune hepatitis. J Pediatr. Jul 1999;135(1):111-4. [Medline].

  12. Desmorat H, Combis JM, Pradat P. [Assessment of quality of life in chronic hepatitis C: effect of treatment]. Gastroenterol Clin Biol. Dec 2003;27(12):1084-90. [Medline].

  13. Djilali-Saiah I, Renous R, Caillat-Zucman S, et al. Linkage disequilibrium between HLA class II region and autoimmune hepatitisin pediatric patients. J Hepatol. Jun 2004;40(6):904-9. [Medline].

  14. Evans JS. Acute and chronic hepatitis. In: Wyllie R, Hyams JS, Fletcher J, eds. Pediatric Gastrointestinal Disease: Pathophysiology, Diagnosis, Management. 2nd ed. WB Saunders Co; 1999:600-23.

  15. Fernandes NF, Redeker AG, Vierling JM, et al. Cyclosporine therapy in patients with steroid resistant autoimmune hepatitis. Am J Gastroenterol. Jan 1999;94(1):241-8. [Medline].

  16. Garcia-Buey L, Garcia-Monzon C, Rodriguez S, et al. Latent autoimmune hepatitis triggered during interferon therapy in patients with chronic hepatitis C. Gastroenterology. Jun 1995;108(6):1770-7. [Medline].

  17. Jackson LD, Song E. Cyclosporin in the treatment of corticosteroid resistant autoimmune chronic active hepatitis. Gut. Mar 1995;36(3):459-61. [Medline].

  18. Klein C, Philipp T, Greiner P, et al. Asymptomatic autoimmune hepatitis associated with anti-LC-1 autoantibodies. J Pediatr Gastroenterol Nutr. Nov 1996;23(4):461-5. [Medline].

  19. Krawitt EL. Autoimmune hepatitis. N Engl J Med. Apr 4 1996;334(14):897-903. [Medline].

  20. Krawitt EL. Autoimmune hepatitis. N Engl J Med. Jan 5 2006;354(1):54-66. [Medline].

  21. Manns M, Gerken G, Kyriatsoulis A, et al. Characterisation of a new subgroup of autoimmune chronic active hepatitis by autoantibodies against a soluble liver antigen. Lancet. Feb 7 1987;1(8528):292-4. [Medline].

  22. Manns MP. Autoimmune hepatitis. In: Schiff ER, Sorrell MF, Maddrey WC, eds. Schiff's Diseases of the Liver. 8th ed. Philadelphia, PA: Lippincott-Raven; 1999:919-35.

  23. Manns MP, Kruger M. Immunogenetics of chronic liver diseases. Gastroenterology. Jun 1994;106(6):1676-97. [Medline].

  24. Manns MP, Obermayer-Straub P. Cytochromes P450 and uridine triphosphate-glucuronosyltransferases: model autoantigens to study drug-induced, virus-induced, and autoimmune liver disease. Hepatology. Oct 1997;26(4):1054-66. [Medline].

  25. McCaughan GW, George J. Fibrosis progression in chronic hepatitis C virus infection. Gut. Mar 2004;53(3):318-21. [Medline]. [Full Text].

  26. McFarlane IG. Pathogenesis of autoimmune hepatitis. Biomed Pharmacother. Jun 1999;53(5-6):255-63. [Medline].

  27. Mieli-Vergani G, Vergani D. Immunological liver diseases in children. Semin Liver Dis. 1998;18(3):271-9. [Medline].

  28. Obermayer-Straub P, Strassburg CP, Manns MP. Autoimmune hepatitis. J Hepatol. 2000;32(1 Suppl):181-97. [Medline].

  29. Paradis K, Alvarez F, Seidman E, et al. Pitfalls in the diagnosis of autoimmune hepatitis associated with liver and kidney microsomal proteins. J Pediatr Gastroenterol Nutr. Nov 1994;19(4):453-9. [Medline].

  30. Roberts SK, Therneau TM, Czaja AJ. Prognosis of histological cirrhosis in type 1 autoimmune hepatitis. Gastroenterology. Mar 1996;110(3):848-57. [Medline].

  31. Robertson DA, Zhang SL, Guy EC, Wright R. Persistent measles virus genome in autoimmune chronic active hepatitis. Lancet. Jul 4 1987;2(8549):9-11. [Medline].

  32. Treichel U, Poralla T, Hess G, et al. Autoantibodies to human asialoglycoprotein receptor in autoimmune-type chronic hepatitis. Hepatology. Apr 1990;11(4):606-12. [Medline].

  33. Vento S, Guella L, Mirandola F, et al. Epstein-Barr virus as a trigger for autoimmune hepatitis in susceptible individuals. Lancet. Sep 2 1995;346(8975):608-9. [Medline].

  34. Vergani D, Mieli-Vergani G. Autoimmune liver disease. In: Walker WA, Durie PR, et al, eds. Pediatric Gastrointestinal Disease. 3rd ed. Ontario, Canada: BC Decker Inc; 2000:1007-14.

  35. Villalta D, Girolami D, Bidoli E, et al. High prevalence of celiac disease in autoimmune hepatitis detected by anti-tissue tranglutaminase autoantibodies. J Clin Lab Anal. 2005;19(1):6-10. [Medline].

[ CLOSE WINDOW ]

Further Reading

[ CLOSE WINDOW ]

Keywords

autoimmune chronic active hepatitis, lupoid hepatitis, plasma cell hepatitis, autoimmune hepatitis, pediatric hepatitis, liver disease in children, AIH, AIH-1, AIH-2, necroinflammatory hepatitis, autoimmune disease, juvenile cirrhosis, acute hepatitis, rubella, Epstein-Barr, hepatitis A, hepatitis B, hepatitis C, hepatitis C virus, HCV, fulminant hepatic failure, autoimmune thyroiditis, celiac disease, inflammatory bowel disease, diabetes mellitus, autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy, APECED,  hepatomegaly, splenomegaly, ascites, ulcerative colitis, sclerosing cholangitis, arthritis, vasculitis, glomerulonephritis

[ CLOSE WINDOW ]

Contributor Information and Disclosures

Author

Husam H Sukerek, MD, Consulting Staff, Department of Gastroenterology, Sabine Medical Center
Husam H Sukerek, MD is a member of the following medical societies: American Academy of Pediatrics and American Medical Association
Disclosure: Nothing to disclose.

Coauthor(s)

Mohammad F El-Baba, MD, Assistant Professor of Pediatrics, Division of Pediatric Gastroenterology, Wayne State University School of Medicine; Divison Chief of Pediatric Gastroenterology, Children's Hospital of Michigan
Mohammad F El-Baba, MD is a member of the following medical societies: American Gastroenterological Association and North American Society for Pediatric Gastroenterology and Nutrition
Disclosure: Nothing to disclose.

Medical Editor

Robert Baldassano, MD, Director, Center for Pediatric Inflammatory Bowel Disease, Division of Gastroenterology and Nutrition, Associate Professor, Department of Pediatrics, The Children's Hospital of Philadelphia, University of Pennsylvania
Robert Baldassano, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Pediatrics, American Gastroenterological Association, and North American Society for Pediatric Gastroenterology and Nutrition
Disclosure: Nothing to disclose.

Pharmacy Editor

Mary L Windle, PharmD, Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy, Pharmacy Editor, eMedicine
Disclosure: Pfizer Inc Stock Investment from broker recommendation; Avanir Pharma Stock Investment from broker recommendation

CME Editor

Steven M Schwarz, MD, FAAP, FACN, AGAF, Professor of Pediatrics, State University of New York, Downstate Medical Center College of Medicine; Distinguished Lecturer, New York Medical College, School of Public Health
Steven M Schwarz, MD, FAAP, FACN, AGAF is a member of the following medical societies: American Academy of Pediatrics, American College of Nutrition, American College of Physician Executives, American Gastroenterological Association, American Pediatric Society, Gastroenterology Research Group, New York Academy of Medicine, North American Society for Pediatric Gastroenterology and Nutrition, and Society for Pediatric Research
Disclosure: TAP Pharmaceuticals Honoraria Speaking and teaching; Curemark, LLC Consulting fee Board membership

Chief Editor

Carmen Cuffari, MD, Associate Professor, Department of Pediatrics, Division of Gastroenterology/Nutrition, Johns Hopkins University School of Medicine
Carmen Cuffari, MD is a member of the following medical societies: American College of Gastroenterology, American Gastroenterological Association, North American Society for Pediatric Gastroenterology, Hepatology and Nutrition, and Royal College of Physicians and Surgeons of Canada
Disclosure: Nothing to disclose.

 
 
HONcode

We subscribe to the
HONcode principles of the
Health On the Net Foundation

All material on this website is protected by copyright, Copyright© 1994- by Medscape.
This website also contains material copyrighted by 3rd parties.

DISCLAIMER: The content of this Website is not influenced by sponsors. The site is designed primarily for use by qualified physicians and other medical professionals. The information contained herein should NOT be used as a substitute for the advice of an appropriately qualified and licensed physician or other health care provider. The information provided here is for educational and informational purposes only. In no way should it be considered as offering medical advice. Please check with a physician if you suspect you are ill.