Other autoantibodies may be evident. Atypical perinuclear antineutrophil cytoplasmic antibodies (pANCA) are frequently present. Czaja et al have shown that patients with autoimmune hepatitis who have positive test results for actin antibody are younger, more commonly test positive for human leukocyte antigen (HLA)–DR3, and required transplantation more frequently than patients with ANAs who test negative for actin antibody. [42]
Related Questions:
Oettinger R, Brunnberg A, Gerner P, Wintermeyer P, Jenke A, Wirth S. Clinical features and biochemical data of Caucasian children at diagnosis of autoimmune hepatitis. J Autoimmun. 2005 Feb. 24(1):79-84. [Medline].
Strassburg CP, Manns MP. Treatment of autoimmune hepatitis. Semin Liver Dis. 2009 Aug. 29(3):273-85. [Medline].
Centers for Disease Control and Prevention. Recommendations for the identification of chronic hepatitis C virus infection among persons born during 1945-1965. MMWR Recomm Rep. 2012 Aug 17. 61:1-32. [Medline]. [Full Text].
Waldenstrom J. The diagnostic importance of ACTH. Acta Endocrinol (Copenh). 1950. 5(3):235-42. [Medline].
Kunkel HG, Ahrens EH, Eisenmenger WJ. Extreme hypergammaglobulinemia in young women with liver disease of unknown etiology. J Clin Invest. 1950. 30:654.
Bearn AG, Kunkel HG, Slater RJ. The problems of chronic liver disease in young women. Am J Med. 1956. 21:3-15.
Joske RA, King WE. The L.E.-cell phenomenon in active chronic viral hepatitis. Lancet. 1955 Sep 3. 269(6888):477-80. [Medline].
Cowling DC, Mackay IR, Taft LI. Lupoid hepatitis. Lancet. 1956 Dec 29. 271(6957):1323-6. [Medline].
Johnson PJ, McFarlane IG. Meeting report: International Autoimmune Hepatitis Group. Hepatology. 1993 Oct. 18(4):998-1005. [Medline].
Scully LJ, Toze C, Sengar DP, et al. Early-onset autoimmune hepatitis is associated with a C4A gene deletion. Gastroenterology. 1993 May. 104(5):1478-84. [Medline].
Czaja AJ, Carpenter HA, Santrach PJ, et al. Genetic predispositions for the immunological features of chronic active hepatitis. Hepatology. 1993 Oct. 18(4):816-22. [Medline].
Longhi MS, Ma Y, Mieli-Vergani G, Vergani D. Aetiopathogenesis of autoimmune hepatitis. J Autoimmun. 2010 Feb. 34(1):7-14. [Medline].
Vento S, Cainelli F. Is there a role for viruses in triggering autoimmune hepatitis?. Autoimmun Rev. 2004 Jan. 3(1):61-9. [Medline].
Wen L, Peakman M, Lobo-Yeo A, et al. T-cell-directed hepatocyte damage in autoimmune chronic active hepatitis. Lancet. 1990 Dec 22-29. 336(8730):1527-30. [Medline].
Umemura T, Ota M. Genetic factors affect the etiology, clinical characteristics and outcome of autoimmune hepatitis. Clin J Gastroenterol. 2015 Dec. 8(6):360-6. [Medline].
Wang Q, Yang F, Miao Q, Krawitt EL, Gershwin ME, Ma X. The clinical phenotypes of autoimmune hepatitis: A comprehensive review. J Autoimmun. 2016 Jan. 66:98-107. [Medline].
Czaja AJ. Diagnosis and management of autoimmune hepatitis. Clin Liver Dis. 2015 Feb. 19(1):57-79. [Medline].
Pathmakanthan S, Kay EW, Murray FE. Autoimmune chronic active hepatitis associated with the presence of antiphospholipid antibodies. Eur J Gastroenterol Hepatol. 1998 Feb. 10(2):155-7. [Medline].
Czaja AJ. Autoimmune hepatitis. Feldman M, Scharschmidt BF, Sleisenger MH, eds. Sleisenger and Fordtran's Gastrointestinal and Liver Disease. 6th ed. Philadelphia, Pa: WB Saunders Company; 1998. 1265-74.
Ramakrishna J, Johnson AR, Banner BF. Long-term minocycline use for acne in healthy adolescents can cause severe autoimmune hepatitis. J Clin Gastroenterol. 2009 Sep. 43(8):787-90. [Medline].
Adar T, Mizrahi M, Pappo O, Scheiman-Elazary A, Shibolet O. Adalimumab-induced autoimmune hepatitis. J Clin Gastroenterol. 2010 Jan. 44(1):e20-2. [Medline].
Fairhurst DA, Sheehan-Dare R. Autoimmune hepatitis associated with infliximab in a patient with palmoplantar pustular psoriaisis. Clin Exp Dermatol. 2009 Apr. 34(3):421-2. [Medline].
Liu ZX, Kaplowitz N. Immune-mediated drug-induced liver disease. Clin Liver Dis. 2002 Aug. 6(3):755-74. [Medline].
Casswall TH, Nemeth A, Nilsson I, Wadstrom T, Nilsson HO. Helicobacter species DNA in liver and gastric tissues in children and adolescents with chronic liver disease. Scand J Gastroenterol. 2010. 45(2):160-7. [Medline].
Michitaka K, Nishiguchi S, Aoyagi Y, et al, for the Japan Etiology of Liver Cirrhosis Study Group. Etiology of liver cirrhosis in Japan: a nationwide survey. J Gastroenterol. 2010. 45(1):86-94. [Medline].
Seki T, Kiyosawa K, Inoko H, et al. Association of autoimmune hepatitis with HLA-Bw54 and DR4 in Japanese patients. Hepatology. 1990 Dec. 12(6):1300-4. [Medline].
Boberg KM. Prevalence and epidemiology of autoimmune hepatitis. Clin Liver Dis. 2002 Aug. 6(3):635-47. [Medline].
Czaja AJ. Special clinical challenges in autoimmune hepatitis: the elderly, males, pregnancy, mild disease, fulminant onset, and nonwhite patients. Semin Liver Dis. 2009 Aug. 29(3):315-30. [Medline].
McFarlane IG, Heneghan MA. Autoimmunity and the female liver. Hepatol Res. 2004 Apr. 28(4):171-6. [Medline].
Mieli-Vergani G, Vergani D. Autoimmune hepatitis in children: what is different from adult AIH?. Semin Liver Dis. 2009 Aug. 29(3):297-306. [Medline].
Haider AS, Kaye G, Thomson A. Autoimmune hepatitis in a demographically isolated area of Australia. Intern Med J. 2010 Apr. 40(4):281-5. [Medline].
Czaja AJ, Carpenter HA. Distinctive clinical phenotype and treatment outcome of type 1 autoimmune hepatitis in the elderly. Hepatology. 2006 Mar. 43(3):532-8. [Medline].
Kirk AP, Jain S, Pocock S, et al. Late results of the Royal Free Hospital prospective controlled trial of prednisolone therapy in hepatitis B surface antigen negative chronic active hepatitis. Gut. 1980 Jan. 21(1):78-83. [Medline].
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. 2008 Jan. 46(1):65-70. [Medline].
Greene MT, Whitington PF. Outcomes in pediatric autoimmune hepatitis. Curr Gastroenterol Rep. 2009 Jun. 11(3):248-51. [Medline].
[Guideline] Gleeson D, Heneghan MA, British Society of Gastroenterology. British Society of Gastroenterology (BSG) guidelines for management of autoimmune hepatitis. Gut. 2011 Dec. 60(12):1611-29. [Medline].
Gregorio GV, Portmann B, Reid F, et al. Autoimmune hepatitis in childhood: a 20-year experience. Hepatology. 1997 Mar. 25(3):541-7. [Medline].
Caprai S, Vajro P, Ventura A, et al. Autoimmune liver disease associated with celiac disease in childhood: a multicenter study. Clin Gastroenterol Hepatol. 2008 Jul. 6(7):803-6. [Medline].
Mieli-Vergani G, Vergani D. Autoimmune paediatric liver disease. World J Gastroenterol. 2008 Jun 7. 14(21):3360-7. [Medline]. [Full Text].
Czaja AJ. Autoimmune hepatitis and viral infection. Gastroenterol Clin North Am. 1994 Sep. 23(3):547-66. [Medline].
Floreani A, Rizzotto ER, Ferrara F, et al. Clinical course and outcome of autoimmune hepatitis/primary sclerosing cholangitis overlap syndrome. Am J Gastroenterol. 2005 Jul. 100(7):1516-22. [Medline].
Czaja AJ, Cassani F, Cataleta M, Valentini P, Bianchi FB. Frequency and significance of antibodies to actin in type 1 autoimmune hepatitis. Hepatology. 1996 Nov. 24(5):1068-73. [Medline].
Czaja AJ, Carpenter HA. Sensitivity, specificity, and predictability of biopsy interpretations in chronic hepatitis. Gastroenterology. 1993 Dec. 105(6):1824-32. [Medline].
Alvarez F, Berg PA, Bianchi FB, et al. International Autoimmune Hepatitis Group Report: review of criteria for diagnosis of autoimmune hepatitis. J Hepatol. 1999 Nov. 31(5):929-38. [Medline].
Wiegard C, Schramm C, Lohse AW. Scoring systems for the diagnosis of autoimmune hepatitis: past, present, and future. Semin Liver Dis. 2009 Aug. 29(3):254-61. [Medline].
Tucker SM, Jonas MM, Perez-Atayde AR. Hyaline droplets in Kupffer cells: a novel diagnostic clue for autoimmune hepatitis. Am J Surg Pathol. 2015 Jun. 39(6):772-8. [Medline].
Lichtenstein GR. Use of laboratory testing to guide 6-mercaptopurine/azathioprine therapy. Gastroenterology. 2004 Nov. 127(5):1558-64. [Medline].
Muratori P, Lalanne C, Barbato E, et al. Features and progression of asymptomatic autoimmune hepatitis in Italy. Clin Gastroenterol Hepatol. 2016 Jan. 14(1):139-46. [Medline].
[Guideline] Manns MP, Czaja AJ, Gorham JD, et al. Diagnosis and management of autoimmune hepatitis. Hepatology. 2010 Jun. 51(6):2193-213. [Medline].
Manns MP, Woynarowski M, Kreisel W, et al. Budesonide induces remission more effectively than prednisone in a controlled trial of patients with autoimmune hepatitis. Gastroenterology. 2010 Oct. 139(4):1198-206. [Medline].
Alvarez F, Ciocca M, Canero-Velasco C, et al. Short-term cyclosporine induces a remission of autoimmune hepatitis in children. J Hepatol. 1999 Feb. 30(2):222-7. [Medline].
Sciveres M, Caprai S, Palla G, Ughi C, Maggiore G. Effectiveness and safety of ciclosporin as therapy for autoimmune diseases of the liver in children and adolescents. Aliment Pharmacol Ther. 2004 Jan 15. 19(2):209-17. [Medline].
Fernandes NF, Redeker AG, Vierling JM, et al. Cyclosporine therapy in patients with steroid resistant autoimmune hepatitis. Am J Gastroenterol. 1999 Jan. 94(1):241-8. [Medline].
Aqel BA, Machicao V, Rosser B, et al. Efficacy of tacrolimus in the treatment of steroid refractory autoimmune hepatitis. J Clin Gastroenterol. 2004 Oct. 38(9):805-9. [Medline].
Van Thiel DH, Wright H, Carroll P, et al. Tacrolimus: a potential new treatment for autoimmune chronic active hepatitis: results of an open-label preliminary trial. Am J Gastroenterol. 1995 May. 90(5):771-6. [Medline].
Than NN, Wiegard C, Weiler-Normann C, et al. Long-term follow-up of patients with difficult to treat type 1 autoimmune hepatitis on tacrolimus therapy. Scand J Gastroenterol. 2016 Mar. 51(3):329-36. [Medline].
Devlin SM, Swain MG, Urbanski SJ, et al. Mycophenolate mofetil for the treatment of autoimmune hepatitis in patients refractory to standard therapy. Can J Gastroenterol. 2004 May. 18(5):321-6. [Medline].
Inductivo-Yu I, Adams A, Gish RG, et al. Mycophenolate mofetil in autoimmune hepatitis patients not responsive or intolerant to standard immunosuppressive therapy. Clin Gastroenterol Hepatol. 2007 Jul. 5(7):799-802. [Medline].
Richardson PD, James PD, Ryder SD. Mycophenolate mofetil for maintenance of remission in autoimmune hepatitis in patients resistant to or intolerant of azathioprine. J Hepatol. 2000 Sep. 33(3):371-5. [Medline].
Wolf DC, Bojito L, Facciuto M, Lebovics E. Mycophenolate mofetil for autoimmune hepatitis: a single practice experience. Dig Dis Sci. 2009 Nov. 54(11):2519-22. [Medline].
Aw MM, Dhawan A, Samyn M, Bargiota A, Mieli-Vergani G. Mycophenolate mofetil as rescue treatment for autoimmune liver disease in children: a 5-year follow-up. J Hepatol. 2009 Jul. 51(1):156-60. [Medline].
Czaja AJ, Lindor KD. Failure of budesonide in a pilot study of treatment-dependent autoimmune hepatitis. Gastroenterology. 2000 Nov. 119(5):1312-6. [Medline].
Zandieh I, Krygier D, Wong V, et al. The use of budesonide in the treatment of autoimmune hepatitis in Canada. Can J Gastroenterol. 2008 Apr. 22(4):388-92. [Medline]. [Full Text].
Osterman MT, Kundu R, Lichtenstein GR, et al. Association of 6-thioguanine nucleotide levels and inflammatory bowel disease activity: a meta-analysis. Gastroenterology. 2006 Apr. 130(4):1047-53. [Medline].
Johnson PJ, McFarlane IG, Williams R. Azathioprine for long-term maintenance of remission in autoimmune hepatitis. N Engl J Med. 1995 Oct 12. 333(15):958-63. [Medline].
Faisal N, Renner EL. Recurrence of autoimmune liver diseases after liver transplantation. World J Hepatol. 2015 Dec 18. 7(29):2896-905. [Medline].
Tripathi D, Neuberger J. Autoimmune hepatitis and liver transplantation: indications, results, and management of recurrent disease. Semin Liver Dis. 2009 Aug. 29(3):286-96. [Medline].
Reich DJ, Fiel I, Guarrera JV, et al. Liver transplantation for autoimmune hepatitis. Hepatology. 2000 Oct. 32(4 Pt 1):693-700. [Medline].
Duclos-Vallee JC, Sebagh M, Rifai K, et al. A 10 year follow up study of patients transplanted for autoimmune hepatitis: histological recurrence precedes clinical and biochemical recurrence. Gut. 2003 Jun. 52(6):893-7. [Medline].
Montano-Loza AJ, Mason AL, Ma M, Bastiampillai RJ, Bain VG, Tandon P. Risk factors for recurrence of autoimmune hepatitis after liver transplantation. Liver Transpl. 2009 Sep 29. 15(10):1254-1261. [Medline].
Chazouilleres O, Wendum D, Serfaty L, et al. Long term outcome and response to therapy of primary biliary cirrhosis-autoimmune hepatitis overlap syndrome. J Hepatol. 2006 Feb. 44(2):400-6. [Medline].
Douglas D. Budesonide helpful in pediatric autoimmune hepatitis. July 10, 2013. Medscape from WebMD. Available at https://www.medscape.com/viewarticle/807623. Accessed: July 22, 2013.
Woynarowski M, Nemeth A, Baruch Y, et al, for the European Autoimmune Hepatitis-Budesonide Study Group. Budesonide versus prednisone with azathioprine for the treatment of autoimmune hepatitis in children and adolescents. J Pediatr. 2013 Nov. 163(5):1347-53.e1. [Medline].
[Guideline] Dalekos GN, Koskinas J, Papatheodoridis GV. Hellenic Association for the Study of the Liver Clinical Practice Guidelines: Autoimmune hepatitis. Ann Gastroenterol. 2019 Jan-Feb. 32 (1):1-23. [Medline]. [Full Text].
Sebode M, Hartl J, Vergani D, Lohse AW, for the International Autoimmune Hepatitis Group (IAIHG). Autoimmune hepatitis: From current knowledge and clinical practice to future research agenda. Liver Int. 2018 Jan. 38(1):15-22. [Medline].
Vinnitskaya EV, Abdulkhakov SR, Abdurakhmanov DT, et al. Important problems in the diagnosis and treatment of primary sclerosing cholangitis (based on the Russian consensus on diagnosis and treatment autoimmune hepatitis. Moscow, 2018). Ter Arkh. 2019 Mar 17. 91(2):9-15. [Medline].
Valota M, Thienemann F, Misselwitz B. False-positive serologies for acute hepatitis A and autoimmune hepatitis in a patient with acute Epstein-Barr virus infection. BMJ Case Rep. 2019 May 10. 12(5):[Medline].
Tables
David C Wolf, MD, FACP, FACG, AGAF, FAASLD Medical Director of Liver Transplantation, Westchester Medical Center; Professor of Clinical Medicine, Division of Gastroenterology and Hepatobiliary Diseases, Department of Medicine, New York Medical College
David C Wolf, MD, FACP, FACG, AGAF, FAASLD is a member of the following medical societies: American Association for the Study of Liver Diseases, American College of Gastroenterology, American College of Physicians, American Gastroenterological Association
Disclosure: Serve(d) as a speaker or a member of a speakers bureau for: Salix; Gilead; Abbvie; Intercept; Merck.
Unnithan V Raghuraman, MD, FACG, FACP, FRCP Consulting Staff, Department of Gastroenterology, St John Medical Center
Unnithan V Raghuraman, MD, FACG, FACP, FRCP is a member of the following medical societies: American Association for the Study of Liver Diseases, American College of Gastroenterology, American College of Physicians, American Gastroenterological Association, American Society for Gastrointestinal Endoscopy
Disclosure: Nothing to disclose.
BS Anand, MD Professor, Department of Internal Medicine, Division of Gastroenterology, Baylor College of Medicine
BS Anand, MD is a member of the following medical societies: American Association for the Study of Liver Diseases, American College of Gastroenterology, American Gastroenterological Association, American Society for Gastrointestinal Endoscopy
Disclosure: Nothing to disclose.
BS Anand, MD Professor, Department of Internal Medicine, Division of Gastroenterology, Baylor College of Medicine
BS Anand, MD is a member of the following medical societies: American Association for the Study of Liver Diseases, American College of Gastroenterology, American Gastroenterological Association, and American Society for Gastrointestinal Endoscopy
Disclosure: Nothing to disclose.
Robert Baldassano, MD Director, Center for Pediatric Inflammatory Bowel Disease, Children's Hospital of Philadelphia; Professor, Department of Pediatrics, Division of Gastroenterology and Nutrition, University of Pennsylvania School of Medicine
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.
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.
Mohammad F El-Baba, MD Associate 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.
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.
Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference
Disclosure: Medscape Salary Employment
Mary L Windle, PharmD, Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference
Disclosure: Nothing to disclose.