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Digestive Diseases and Sciences

, Volume 51, Issue 11, pp 1978–1984 | Cite as

Type 1 Autoimmune Hepatitis in Taiwan: Diagnosis Using the Revised Criteria of the International Autoimmune Hepatitis Group

  • Lok-Beng KoayEmail author
  • Ching-Yih Lin
  • Sun-Lung Tsai
  • Chuan Lee
  • Ching-Nan Lin
  • Ming-Juen Sheu
  • Hsing-Tao Kuo
  • Chi-Shu Sun
Original Paper

Abstract

Autoimmune hepatitis (AIH) is rare in Asian countries compared to the West, and an exceptionally low prevalence was noted previously in Taiwan. Using the revised criteria of the IAIHG, 48 cases of AIH patients were diagnosed. All patients were consecutively diagnosed over a period of 5 years. Detailed medical histories including disease onset, hepatitis B and C, alcohol, drugs, blood transfusion, and family history of autoimmune disease were recorded. Clinical manifestations, result of steroid therapy, outcome, and survival rate were investigated and analyzed. Clinical data on AIH patients with cirrhosis and without cirrhosis were compared and analyzed for their outcome. The statistical methods used were Fisher's exact test, Wilcoxon rank sum test, and Kaplan-Meier curve. Forty-eight patients were diagnosed as AIH type 1, with a median age of 58 years and a female:male ratio of 37:11. The most common clinical features at presentation were fatigue, jaundice, and anorexia. Ninety-eight percent of patients were ANA positive, and most of the patients showed elevated values of AST, ALT, serum globulin, and bilirubin. A substantial proportion of patients presented with poor liver function at entry and 35% of patients had liver cirrhosis, with relatively prolonged PT (P=0.001) and poorer outcome (P=0.005) compared to the noncirrhotics. As a whole there was a favorable treatment response and the overall survival rate was 85%. We conclude that the incidence of AIH in Taiwan is much higher than previously presumed and AIH type 1 is the predominant type of the disease. Although a substantial proportion of AIH patients presented with poor hepatic function at entry, as a whole there was a favorable clinical outcome.

Keywords

Autoimmune hepatitis Antinuclear antibodies Liver cirrhosis Autoimmune hepatitis type 1 

References

  1. 1.
    Alvarez F, Berg PA, Bianchi FB, Bianchi L, Burroughs AK, Cancado EL, Chapman RW, Cooksley WGE, Czaja AJ, Desmet VJ, Donaldson PT, Eddleston ALWF, Fainboim L, Heathcote J, Homberg J-C, Hoofnagle JH, Kakumu S, Krawitt EL, Mackay IR, MacSween RNM, Maddrey WC, Manns MP, McFarlane IG, Meyer zum Buschenfelde K-H, Mieli-Vergani G, Nakanuma Y, Nishioka M, Penner E, Porta G, Portmann BC, Reed WD, Rodes J, Schalm SW, Scheuer PJ, Schrumpf E, Seki T, Toda G, Tsuji T, Tygstrup N, Vergani D, Zeniya M (1999) International Autoimmune Hepatitis Group Report: review of criteria for diagnosis of autoimmune hepatitis. J Hepatol 31:929–938PubMedCrossRefGoogle Scholar
  2. 2.
    International Working Party (1995) Terminology of chronic hepatitis. Am J Gastroenterol 90:181–189Google Scholar
  3. 3.
    Czaja AJ (2005) Current concepts in autoimmune hepatitis. Ann Hepatol 4(1):6–24PubMedGoogle Scholar
  4. 4.
    Boberg KM, Adland E, Jahnsen J, Raknerud N, Stiris M, Bell H (1998) Incidence and prevalence of primary biliary cirrhosis, primary sclerosing cholangitis, and autoimmune hepatitis in a Norwegian population. Scand J Gastroenterol 33:99–103PubMedCrossRefGoogle Scholar
  5. 5.
    Toda G, Zeniya M, Watanabe F, Imawari M, Kiyosawa K, Nishioka M, Tsuji T, Omata M (1997) Present status of autoimmune hepatitis in Japan-correlating the characteristics with international criteria in an area with a high rate of HCV infection. J Hepatol 26:1207–1212PubMedCrossRefGoogle Scholar
  6. 6.
    Lee YM, Teo EK, NG TM, Khor C, Fock KM (2001) Autoimmune hepatitis in Singapore: A rare syndrome affecting middle-aged women. J Gastroenterol Hepatol 16:1384–138PubMedCrossRefGoogle Scholar
  7. 7.
    Gupta R, Agarwal SR, Jain M, Malhotra V, Sarin SK (2001) Autoimmune hepatitis in the Indian subcontinent: 7 years experience. J Gastroenterol Hepatol 16:1144–1148PubMedCrossRefGoogle Scholar
  8. 8.
    Koay LB, Sun CS, Hsu SI, Chen JJ, Lin CY (2001) Type 1 autoimmune hepatitis micmicking crytogenic hepatitis. Gastroenterol J Taiwan 18:132–138Google Scholar
  9. 9.
    Huang HC, Huang YS, Wu JC, Tsay SH, Huo TI, Wang YJ, Lo JC, Chen CY, Li CP, Chang FY, Lee SD (2002) Characteristics of autoimmune hepatitis in Taiwan: the 11 year experiences of a medical center. Chinese Med J (Taipei) 65:563–569Google Scholar
  10. 10.
    Qiu DK, Ma X (2003) Relationship between human leukocyte antigen-DRBI and autoimmune hepatitis type 1 in Chinese patients. J. Gastroenterol Hepatol 18:63–67PubMedCrossRefGoogle Scholar
  11. 11.
    Johnson PJ, Mcfarlane IG (1993) Meeting report: International Autoimmune Hepatitis Group. Hepatology 18:998–1005PubMedGoogle Scholar
  12. 12.
    Czaja AJ, Carpenter HA (1996) Validation of scoring system for diagnosis of autoimmune hepatitis. Dig Dis Sci 41:305–314PubMedCrossRefGoogle Scholar
  13. 13.
    Nishioka M, Morshed SA, McFarlane IG (1998) Geographical variation in the frequency and characteristics of autoimmune liver diseases. In: Krawitt EL, Wiesner RH, Nishioka M (eds) Autoimmune liver diseases, 2nd ed. Elsevier, Amsterdam, pp 413–424Google Scholar
  14. 14.
    Boberg KM (2002) Prevalence and epidemiology of autoimmune hepatitis. Clin Liver Dis 6:347–359Google Scholar
  15. 15.
    Sherlock S, Dooley J (1993) Chronic autoimmune hepatitis. In: Diseases of the liver and biliary system. Blackwell Science, Oxford, pp 299–306Google Scholar
  16. 16.
    Parker DR, Kingham JGC (1997) Type 1 autoimmune hepatitis is primarily a disease of later life. Q J Med 90:289–296Google Scholar
  17. 17.
    Ferrari R, Pappas G, Agostinelli D, Muratori P, Muratori L, Lenzi M, Verucchi G, Cassani E, Chiodo F, Bianchi FB (2004) Type 1 autoimmune hepatitis: patterns of clinical presentation and differential diagnosis of the ‘acute’ type. Q J Med 97(7):407–417Google Scholar
  18. 18.
    Krawitt EL (1996) Autoimmune hepatitis. N Eng J Med 334:897–903CrossRefGoogle Scholar
  19. 19.
    Czaja AJ, Mann MP (1995) The validity and importance of subtypes of autoimmune hepatitis: a point of view. Am J Gastroenterol 90:1206–1211PubMedGoogle Scholar
  20. 20.
    Homberg J-C, Abuaf N, Bernard O, Islam S, Alvarez F, Khalil SH, Poupon R, Darnis F, Levy V-G, Grippon P, Opolon P, Bernuau J, Benhamou J-P, Alagille D (1987) Chronic active hepatitis associated with anti-liver/kidney microsome type 1: a second type of “autoimmune” hepatitis. Hepatology 7:1333–1339Google Scholar
  21. 21.
    Manns MP (1997) Recent developments in autoimmune liver diseases. J Gastroenterol Hepatol 12(Suppl):s256–s271PubMedGoogle Scholar
  22. 22.
    Whittingham S, Mackay IR, Irwin J (1966) Autoimmune hepatitis: immunofluorescence reactions with cytoplasm of smooth muscle and glomerular cells. Lancet 1:1333–1335PubMedCrossRefGoogle Scholar
  23. 23.
    Czja AJ, Cassani F, Cataleta M (1966) Frequency and significance of antibodies to actin in type 1 autoimmune hepatitis. Hepatology 24:1068–1073CrossRefGoogle Scholar
  24. 24.
    Mcfarlane IG (1998) The relationship between autoimmune markers and different clinical syndromes in autoimmune hepatitis. Gut 42:599–602PubMedCrossRefGoogle Scholar
  25. 25.
    Czja AJ, Donaldson PT, Lohse AW (2002) Antibodies to soluble liver antigen/liver pancreas and HLA risk factors for type 1 autoimmune hepatitis. Am J Gastroenterol 97:413–419CrossRefGoogle Scholar
  26. 26.
    Targan SR, Landers C, Vidrich A (1995) High-titer antineutrophil cytoplasmic antibodies in type-1 autoimmune hepatitis. Gastroenterology 108:1159–1166PubMedCrossRefGoogle Scholar
  27. 27.
    Obermayer-Straub P, Strassburg CP, Manns MP (2000) Autoimmune hepatitis. J Hepatol 32(Suppl 1):181–197PubMedCrossRefGoogle Scholar
  28. 28.
    Czaja AJ, Doherty DG, Donalson PT (2002) Genetic bases of autoimmune hepatitis. Dig Dis Sci 47:2139–2150PubMedCrossRefGoogle Scholar
  29. 29.
    Donaldson PT, Doherty DG, Hayllar KM, McFarlane IG, Johnson PJ, Williams R (1991) Susceptibility to autoimmune chronic active hepatitis: human leukocyte antigens DR4 and A1-B8-DR3 are independent risk factors. Hepatology 13:701–706PubMedCrossRefGoogle Scholar
  30. 30.
    Czaja AJ, Carpenter HA, Santrach PJ, Moore SB (1993) Significance of HLA DR4 in type 1 autoimmune hepatitis. Gastroenterology 105:1502–1507PubMedGoogle Scholar
  31. 31.
    Seki T, Kiyosawa K, Inoko H, Ota M (1990) Association of autoimmune hepatitis with HLA-Bw54 and DR4 in Japanese patients. Hepatology 12:1300–1304PubMedGoogle Scholar
  32. 32.
    Czaja AJ, Strettell MDJ, Thomson LJ, Santrach PJ, Moore SB, Donaldson PT, William R (1997) Associations between alleles of the major histocompatibility complex and type 1 autoimmune hepatitis. Hepatology 25:317–323PubMedCrossRefGoogle Scholar
  33. 33.
    Czaja AJ (1996) Diagnosis and therapy of autoimmune liver disease. Med Clin North Am 80:973–994PubMedGoogle Scholar
  34. 34.
    Roberts SK, Therneau TM, Czaja AJ (1996) Prognosis of histological cirrhosis in type ∣ autoimmune hepatitis. Gastroenterology 110:848–857PubMedCrossRefGoogle Scholar
  35. 35.
    Omagari K, Kinoshita H, Kato Y, Nakata K, Kanematsu T, Kusumoto Y, Mori I (1999) Clinical features of 89 patients with autoimmune hepatitis in Nagasaki Prefecture, Japan. J Gastroenterol 34:221–226PubMedCrossRefGoogle Scholar
  36. 36.
    Czaja AJ, Carpenter HA (2004) Decreased fibrosis during corticosteroid therapy of autoimmune hepatitis. Hepatol 40:646–652CrossRefGoogle Scholar
  37. 37.
    Cotler SJ, DeLellis R, Kaplan MM (2001) Reversibility of cirrhosis in autoimmune hepatitis with corticosteroid therapy. J Clin Gasteroenterol 32:428–430CrossRefGoogle Scholar
  38. 38.
    Mohamadnejad M, Malekzadeh R, Nasseri-Moghaddam S, Hagh-Azali S, Rakhshani N, Tavangar SM, Sedaghat M, Alimohamadi SM (2005) Impact of immunosuppressive treatment on liver fibrosis in autoimmune hepatitis. Dig Dis Sci 50:547–551PubMedCrossRefGoogle Scholar

Copyright information

© Springer Science+Bisiness Media, Inc. 2006

Authors and Affiliations

  • Lok-Beng Koay
    • 1
    Email author
  • Ching-Yih Lin
    • 1
  • Sun-Lung Tsai
    • 1
  • Chuan Lee
    • 1
  • Ching-Nan Lin
    • 2
  • Ming-Juen Sheu
    • 1
  • Hsing-Tao Kuo
    • 1
  • Chi-Shu Sun
    • 1
  1. 1.Division of Gastroenterology and Hepatology, Department of Internal MedicineChi Mei Medical CenterYung Kang City, TainanR.O.C.
  2. 2.Department of PathologyChi Mei Medical CenterTainanR.O.C.

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