Diagnostic Value and Utility of the Simplified International Autoimmune Hepatitis Group (IAIHG) Criteria for Autoimmune Hepatitis in Korea

한국인 자가면역성 간염을 진단하기 위한 Simplified Scoring Criteria의 가치 및 유용성

  • Lee, Yun-Nah (Digestive Disease Center, Department of Internal Medicine, Soonchunhyang University College of Medicine) ;
  • Kim, Young-Seok (Digestive Disease Center, Department of Internal Medicine, Soonchunhyang University College of Medicine) ;
  • Kim, Sang-Gyune (Digestive Disease Center, Department of Internal Medicine, Soonchunhyang University College of Medicine) ;
  • Lim, Jae-Hee (Digestive Disease Center, Department of Internal Medicine, Soonchunhyang University College of Medicine) ;
  • Jeong, Soung-Won (Digestive Disease Center, Department of Internal Medicine, Soonchunhyang University College of Medicine) ;
  • Jang, Jae-Young (Digestive Disease Center, Department of Internal Medicine, Soonchunhyang University College of Medicine) ;
  • Lee, Se-Hwan (Digestive Disease Center, Department of Internal Medicine, Soonchunhyang University College of Medicine) ;
  • Kim, Hong-Soo (Digestive Disease Center, Department of Internal Medicine, Soonchunhyang University College of Medicine) ;
  • Kim, Boo-Sung (Digestive Disease Center, Department of Internal Medicine, Soonchunhyang University College of Medicine) ;
  • Kim, Hee-Kyung (Digestive Disease Center, Department of Pathology, Soonchunhyang University College of Medicine)
  • 이윤나 (순천향대학교 의과대학 내과학교실 소화기연구소) ;
  • 김영석 (순천향대학교 의과대학 내과학교실 소화기연구소) ;
  • 김상균 (순천향대학교 의과대학 내과학교실 소화기연구소) ;
  • 임재희 (순천향대학교 의과대학 내과학교실 소화기연구소) ;
  • 정승원 (순천향대학교 의과대학 내과학교실 소화기연구소) ;
  • 장재영 (순천향대학교 의과대학 내과학교실 소화기연구소) ;
  • 이세환 (순천향대학교 의과대학 내과학교실 소화기연구소) ;
  • 김홍수 (순천향대학교 의과대학 내과학교실 소화기연구소) ;
  • 김부성 (순천향대학교 의과대학 내과학교실 소화기연구소) ;
  • 김희경 (순천향대학교 의과대학 병리과학교실)
  • Published : 2011.09.01

Abstract

Background/Aims: The diagnostic criteria for autoimmune hepatitis (AIH) were created and revised by the International Autoimmune Hepatitis Group (IAIHG) in 1999. Simplified scoring criteria based on four clinical components were recently proposed. The aim of this study was to assess the diagnostic value and usefulness of these simplified criteria in Korea. Methods: We applied the simplified scoring criteria to 22 AIH patients diagnosed according to the original revised scoring criteria proposed in 1999. Furthermore, in order to compare the predictive power of these two sets of diagnostic criteria, we included 84 patients with liver diseases [toxic hepatitis (n = 50), nonalcoholic fatty liver disease (n = 18), primary biliary cirrhosis (PBC) (n = 11), and PBC/AIH overlap syndrome (n = 5)] other than AIH. Results: Twenty (90.9%) patients with AIH and five (100%) with PBC/AIH overlap syndrome were diagnosed with AIH according to the simplified scoring criteria. Three (27.3%) patients with PBC were false-positive for AIH according to the simplified scoring criteria. Those patients diagnosed according to the simplified scoring criteria showed an increased frequency of ANA and/or SMA of ${\geq}$ 1:80 (p = 0.491) and an increased frequency of serum IgG levels at or above the upper normal limit compared to patients with PBC (p = 0.006). The sensitivity and specificity of the simplified scoring criteria for the diagnosis of AIH were 90.9 and 96.2%, respectively. Conclusions: The simplified scoring criteria offer a reliable and simple method for excluding AIH; however, these criteria may have limitations in the diagnosis of patients with atypical features, especially those with low autoantibody and IgG levels.

목적: 자가면역성 간염(AIH)을 진단하기 위한 revised original scoring criteria는 지나치게 번거로워 임상에서의 적용이 쉽지 않았다. 이러한 단점을 보안하기 위해 2008년 Hennes 등 [19]에 의해 simplified scoring criteria가 발표되었다. 이에 저자등은 이 criteria를 한국인에게 적용하였을 경우, 진단적 가치 및 유용성을 평가하고자 연구를 시행하였다. 방법: Original revised scoring criteria에 의해 AIH이 진단된 22예와 자가면역 간염/원발성담즙 간경변증(autoimmune hepatitis/primary biliary cirrhosis, AIH/PBC) 중복증후군을 진단받은 5예, 그리고 대조군으로 독성간염 50예, 비알코올성 지방간(NAFLD) 18예, 원발성담즙 간경변증(PBC) 11예를 후향적으로 분석하였다. 결과: AIH 중 20명(90.9%)과 AIH/PBC 중복 증후군 5명 모두가 simplified scoring criteria에 의해 AIH로 진단되었다. 그리고 PBC 중 3명인 27.3%에서 새로운 진단기준을 적용하였을 경우 위양성을 보였다. AIH 진단에 대한 simplified scoring criteria의 민감도와 특이도는 각각 90.9%와 96.2%였다. 결론: 한국인에게 simplified scoring criteria는 중복증후군을 포함한 자가면역성 간염을 진단하는 데 유용하게 사용될 수 있을 것으로 생각한다.

Keywords

References

  1. McFarlane IG. Autoimmune hepatitis: diagnostic criteria, subclassifications, and clinical features. Clin Liver Dis 2002;6:605-621. https://doi.org/10.1016/S1089-3261(02)00019-3
  2. Waldenstrom J. Liver, blood proteins and nutritive protein. Dtsch Z Verdau Stoffwechselkr 1953;9:113-119.
  3. Boberg KM, Aadland E, Jahnsen J, Raknerud N, Stiris M, Bell H. Incidence and prevalence of primary biliary cirrhosis, primary sclerosing cholangitis, and autoimmune hepatitis in a Norwegian population. Scand J Gastroenterol 1998;33:99-103. https://doi.org/10.1080/00365529850166284
  4. Wiesner RH, Demetris AJ, Belle SH, et al. Acute hepatic allograft rejection: incidence, risk factors, and impact on outcome. Hepatology 1998;28:638-645. https://doi.org/10.1002/hep.510280306
  5. Czaja AJ, dos Santos RM, Porto A, Santrach PJ, Moore SB. Immune phenotype of chronic liver disease. Dig Dis Sci 1998;43:2149-2155. https://doi.org/10.1023/A:1018836004279
  6. Gregorio GV, Portmann B, Reid F, et al. Autoimmune hepatitis in childhood: a 20-year experience. Hepatology 1997;25:541-547. https://doi.org/10.1002/hep.510250308
  7. Nikias GA, Batts KP, Czaja AJ. The nature and prognostic implications of autoimmune hepatitis with an acute presentation. J Hepatol 1994;21:866-871. https://doi.org/10.1016/S0168-8278(94)80251-3
  8. Kessler WR, Cummings OW, Eckert G, Chalasani N, Lumeng L, Kwo PY. Fulminant hepatic failure as the initial presentation of acute autoimmune hepatitis. Clin Gastroenterol Hepatol 2004;2:625-631. https://doi.org/10.1016/S1542-3565(04)00246-0
  9. Kogan J, Safadi R, Ashur Y, Shouval D, Ilan Y. Prognosis of symptomatic versus asymptomatic autoimmune hepatitis: a study of 68 patients. J Clin Gastroenterol 2002;35:75-81. https://doi.org/10.1097/00004836-200207000-00016
  10. Feld JJ, Dinh H, Arenovich T, Marcus VA, Wanless IR, Heathcote EJ. Autoimmune hepatitis: effect of symptoms and cirrhosis on natural history and outcome. Hepatology 2005;42:53-62.
  11. Soloway RD, Summerskill WH, Baggenstoss AH, et al. Clinical, biochemical, and histological remission of severe chronic active liver disease: a controlled study of treatments and early prognosis. Gastroenterology 1972;63:820-833.
  12. Czaja AJ. Auto-immune chronic active hepatitis: a specific entity? the negative argument. J Gastroenterol Hepatol 1990;5:343-351. https://doi.org/10.1111/j.1440-1746.1990.tb01636.x
  13. Cassani F, Muratori L, Manotti P, et al. Serum autoantibodies and the diagnosis of type-1 autoimmune hepatitis in Italy: a reappraisal at the light of hepatitis C virus infection. Gut 1992;33:1260-1263. https://doi.org/10.1136/gut.33.9.1260
  14. Czaja AJ, Carpenter HA, Santrach PJ, Moore SB. Immunologic features and HLA association in chronic viral hepatitis. Gastroenterology 1995;108:157-164. https://doi.org/10.1016/0016-5085(95)90020-9
  15. Czaja AJ, Homburger HA. Autoantibodies in liver disease. Gastroenterology 2001;120:239-249. https://doi.org/10.1053/gast.2001.20223
  16. Johnson PJ, McFarlane IG. Meeting report: International Autoimmune Hepatitis Group. Hepatology 1993;18:998-1005. https://doi.org/10.1002/hep.1840180435
  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. https://doi.org/10.1016/S0168-8278(99)80297-9
  18. Papamichalis PA, Zachou K, Koukoulis GK, 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. https://doi.org/10.1186/1740-2557-4-3
  19. Hennes EM, Zeniya M, Czaja AJ, et al. Simplified criteria for the diagnosis of autoimmune hepatitis. Hepatology 2008;48:169-176. https://doi.org/10.1002/hep.22322
  20. Chung HV, Riley M, Ho JK, et al. Retrospective review of pediatric and adult autoimmune hepatitis in two quaternary care centeres in British Columbia: increased prevalence seen in British Columbia's First Nations Community. Can J Gastroenterol 2007;21:565-568.
  21. Lim KN, Casanova RL, Boyer TD, Bruno CJ. Autoimmune hepatitis in African Americans: presenting features and response to therapy. Am J Gastroenterol 2001;96:3390-3394. https://doi.org/10.1111/j.1572-0241.2001.05272.x
  22. Chazouilleres O, Wendum D, Serfaty L, Montembault S, Rosmorduc O, Poupon R. Primary biliary cirrhosis-autoimmune hepatitis overlap syndrome: clinical features and response to therapy. Hepatology 1998;28:296-301. https://doi.org/10.1002/hep.510280203
  23. Czaja AJ. Performance parameters of the diagnostic scoring systems for autoimmune hepatitis. Hepatology 2008;48:1540-1548. https://doi.org/10.1002/hep.22513
  24. Benichou C. Criteria of drug-induced liver disorders: report of an international consensus meeting. J Hepatol 1990;11:272-276. https://doi.org/10.1016/0168-8278(90)90124-A
  25. Clark JM, Brancati FL, Diehl AM. Nonalcoholic fatty liver disease. Gastroenterology 2002;122:1649-1657. https://doi.org/10.1053/gast.2002.33573
  26. Jung SR, Lee HC, Park YH, et al. Validation of International Autoimmune Hepatitis Group scoring system for diagnosis of type 1 autoimmune hepatitis in Korea. Korean J Hepatol 2002;8:35-43.
  27. van Buuren HR, van Hoogstraten HJE, Terkivatan T, Schalm SW, Vleggaar FP. High prevalence of autoimmune hepatitis among patients with primary sclerosing cholangitis. J Hepatol 2000;33:543-548. https://doi.org/10.1016/S0168-8278(00)80005-7
  28. Omagari K, Masuda J, Kato Y, et al. Re-analysis of clinical features of 89 patients with autoimmune hepatitis using the revised scoring system proposed by the International Autoimmune Hepatitis Group. Intern Med 2000;39:1008-1012. https://doi.org/10.2169/internalmedicine.39.1008
  29. Kaya M, Angulo P, Lindor KD. Overlap of autoimmune hepatitis and primary sclerosing cholangitis: an evaluation of a modified scoring system. J Hepatol 2000;33:537-542. https://doi.org/10.1016/S0168-8278(00)80004-5
  30. Czaja A, Carpenter HA. Validation of a scoring system for diagnosis of autoimmune hepatitis. Dig Dis Sci 1996;41:305-314. https://doi.org/10.1007/BF02093820
  31. Al-Chalabi T, Portmann BC, Bernal W, McFarlane IG, Heneghan MA. Autoimmune hepatitis overlap syndromes: an evaluation of treatment response, long term-outcome and survival. Aliment Pharmacol Ther 2008;28:209-220. https://doi.org/10.1111/j.1365-2036.2008.03722.x
  32. Yeoman AD, Westbrook RH, Al-Chalabi T, et al. Diagnostic value and utility of the simplified International Autoimmune Hepatitis Group (IAIHG) criteria in acute and chronic liver disease. Hepatology 2009;50:538-545.
  33. Manns MP, Vogel A. Autoimmune hepatitis, from mechanism to therapy. Hepatology 2006;43(2 Suppl 1):S132-S144.
  34. Strassburg CP, Manns MP. Autoantibodies and autoantigens in autoimmune hepatitis. Semin Liver Dis 2002;22:339-352. https://doi.org/10.1055/s-2002-35704
  35. Adams LA, Lindor KD, Angulo P. The prevalence of autoantibodies and autoimmune hepatitis in patients with nonalcoholic fatty liver disease. Am J Gastroenterol 2004;99:1316-1320. https://doi.org/10.1111/j.1572-0241.2004.30444.x
  36. Kuiper EM, Zondervan PE, van Buuren HR. Paris criteria are effective in diagnosis of primary biliary cirrhosis and autoimmune hepatitis overlap syndrome. Clin Gastroenterol Hepatol 2010;8:530-534. https://doi.org/10.1016/j.cgh.2010.03.004