Skip to main content

Advertisement

SpringerLink
Log in

Validation of scoring system for diagnosis of autoimmune hepatitis

  • Liver: Infectious, Inflammatory, And Metabolic Disorders
  • Published:
Digestive Diseases and Sciences Aims and scope Submit manuscript

Abstract

To assess the validity of a scoring system developed by the International Autoimmune Hepatitis Group for the definite diagnosis of autoimmune hepatitis, 119 patients with autoimmune hepatitis by standard clinical criteria and 131 patients with other chronic liver diseases were evaluated. Each patient was graded on 35 items in 13 clinical categories. Ninety-seven patients diagnosed as having autoimmune hepatitis by conventional criteria (82%) had a definite diagnosis by the scoring system and 22 patients (18%) had a probable diagnosis. Of these patients, those with definite diagnoses had significant clinical differences from those with probable diagnoses. Only two patients with other chronic liver disease (2%) had scores sufficient for a definite diagnosis of autoimmune hepatitis. Probable diagnoses, however, were common in other conditions (33%). Failure to adequately downgrade for cholestatic features contributed to these uncertain diagnoses. Scoring for treatment response downgraded the diagnosis in 17 of 93 patients with a definite diagnosis before therapy (18%) and upgraded the diagnosis in 6 of 14 others (43%). We conclude that the scoring system is specific for the definite diagnosis of autoimmune hepatitis, and it complements standard clinical criteria by establishing the strength of the diagnosis and defining distinctive subgroups within the diagnostic category. Refinements are necessary to reduce the frequency of probable diagnoses. Diagnoses at presentation can be commonly modified by scoring treatment response.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Similar content being viewed by others

References

  1. Czaja AJ: Natural history, clinical features, and treatment of autoimmune hepatitis. Semin Liver Dis 4:1–12, 1984

    PubMed  Google Scholar 

  2. Czaja AJ: Autoimmune hepatitis: Current therapeutic concepts. Clin Immunother 1:413–429, 1994

    Google Scholar 

  3. Czaja AJ: Diagnosis and treatment of chronic hepatitis. Compr Ther 10:58–63, 1984

    Google Scholar 

  4. Czaja AJ: Autoimmune chronic active hepatitis—a specific entity? The negative argument. J Gastro-Hepatol 5:343–351, 1990

    Google Scholar 

  5. Kenny RP, Czaja AJ, Ludwig J, Dickson ER: Frequency and significance of antimitochondrial antibodies in severe chronic active hepatitis. Dig Dis Sci 31:705–711, 1986

    PubMed  Google Scholar 

  6. Czaja AJ, Carpenter HA, Manns MP: Antibodies to soluble liver antigen, P450IID6, and mitochondrial complexes in chronic hepatitis. Gastroenterology 105:1522–1528, 1993

    PubMed  Google Scholar 

  7. Esteban JI, Esteban R, Viladomiu L, Lopez-Talavera JC, Gonzalez A, Hernandez JM, Roget M, Vargas V, Guardia J, Houghton M, Choo Q-L, Kuo G: Hepatitis C virus antibodies among risk groups in Spain. Lancet 2:294–297, 1989

    PubMed  Google Scholar 

  8. Magrin S, Craxi A, Fiorentino G, Fabiano C, Provenzano G, Pinzello GB, Palzzo U, Almasio P, Pagliaro L: Is autoimmune chronic active hepatitis a HCV-related disease? J Hepatol 13:56–60, 1991

    PubMed  Google Scholar 

  9. McFarlane IG, Smith HM, Johnson PJ, Bray GP, Vergani D, Williams R: Hepatitis C virus antibodies in chronic active hepatitis: Pathogenetic factor or false-positive result? Lancet 335:754–757, 1990

    PubMed  Google Scholar 

  10. Czaja AJ, Taswell HF, Rakela J, Schimek C: Frequency and significance of antibody to hepatitis C virus in severe corticosteroid-treated autoimmune chronic active hepatitis. Mayo Clin Proc 66:572–582, 1991

    PubMed  Google Scholar 

  11. Mitchel LS, Jeffers LJ, Reddy KR, Cheinquer H, Coelho-Little E, Moreda R, Parker T, Silva M, Li XM, deMedina M, Coelho-Borges S, Hill M, Altman R, Manns MP, Schiff ER: Detection of hepatitis C virus antibody by first and second generation assays and polymerase chain reaction in patients with autoimmune chronic active hepatitis types I, II, and III. Am J Gastroenterol 88:1027–1034, 1993

    PubMed  Google Scholar 

  12. Czaja AJ, Magrin S, Fabiano C, Fiorentino G, Diquattro O, Craxi A, Pagliaro L: Hepatitis C virus infection as a determinant of behavior in type 1 autoimmune hepatitis. Dig Dis Sci 40:33–40, 1995

    PubMed  Google Scholar 

  13. Ludwig J, Czaja AJ, Dickson ER, LaRusso NF, Wiesner RH: Manifestations of nonsuppurative cholangitis in chronic hepatobiliary disease: Morphologic spectrum, clinical correlations and terminology. Liver 4:105–116, 1984

    PubMed  Google Scholar 

  14. Bach N, Thung SN, Schaffner F: The histological features of chronic hepatitis C and autoimmune chronic hepatitis: A comparative analysis. Hepatology 15:572–577, 1992

    PubMed  Google Scholar 

  15. Czaja AJ, Carpenter HA: Sensitivity, specificity and predictability of biopsy interpretations in chronic hepatitis. Gastroenterology 105:1824–1832, 1993

    PubMed  Google Scholar 

  16. Cooksley WG, Powell LW, Kerr JF, Bhathal PS: Cholestasis in active chronic hepatitis. Am J Dig Dis 17:495–504, 1972

    PubMed  Google Scholar 

  17. Crapper RM, Bhathal PS, Mackay IR, Frazer IH: “Acute” autoimmune hepatitis. Digestion 34:216–225, 1986

    PubMed  Google Scholar 

  18. Czaja AJ, Rakela J, Ludwig J: Features reflective of early prognosis in corticosteroid-treated severe autoimmune chronic active hepatitis. Gastroenterology 95:448–453, 1988

    PubMed  Google Scholar 

  19. Amontree JS, Stuart TD, Bredfeldt JE: Autoimmune chronic active hepatitis masquerading as acute hepatitis. J Clin Gastroenterol 11:303–307, 1989

    PubMed  Google Scholar 

  20. Nikias GA, Batts KP, Czaja AJ: The nature and prognostic implications of autoimmune hepatitis with an acute presentation. J Hepatol 21:866–871, 1994

    PubMed  Google Scholar 

  21. Czaja AJ, Nishioka M, Morshed SA, Hachiya T: Patterns of nuclear immunofluorescence and reactivities to recombinant nuclear antigens in autoimmune hepatitis. Gastroenterology 107:200–207, 1994

    PubMed  Google Scholar 

  22. Manns MP, Johnson EF, Griffin KJ, Tan EM, Sullivan KF: Major antigen of liver kidney microsomal autoantibodies in idiopathic autoimmune hepatitis is cytochrome P450db1. J Clin Invest 83:1066–1072, 1989

    PubMed  Google Scholar 

  23. Czaja AJ, Manns MP, Homburger HA: Frequency and significance of antibodies to liver/kidney microsome type 1 in adults with chronic active hepatitis. Gastroenterology 103:1290–1295, 1992

    PubMed  Google Scholar 

  24. Yamamoto AM, Cresteil D, Homberg JC, Alvarerz F: Characterization of the anti-liver-kidney microsome antibody (anti-LKM1) from hepatitis C virus-positive and -negative sera. Gastroenterology 104:1762–1767, 1993

    PubMed  Google Scholar 

  25. Treichel U, Poralla T, Hess G, Manns M, Meyer zum Buschefelde K-H: Autoantibodies to human asialoglycoprotein receptor in autoimmune-type chronic hepatitis. Hepatology 11:606–612, 1990

    PubMed  Google Scholar 

  26. Poralla T, Treichel U, Lohr H, Fleischer B: The asialoglycoprotein receptor as target structure in autoimmune liver diseases. Semin Liver Dis 11:215–222, 1991

    PubMed  Google Scholar 

  27. Manns M, Gerken G, Kyriatsoulis A, Staritz M, Meyer zum Buschenfelde K-H: Characterization of a new subgroup of autoimmune chronic active hepatitis by autoantibodies against a soluble liver antigen. Lancet 1:292–294, 1987

    PubMed  Google Scholar 

  28. Wachter B, Kyriatsoulis A, Lohse AW, Gerken G, Meyer zum Buschenfelde K-H, Manns M: Characterization of liver cytokeratin as a major target antigen of anti-SLA antibodies. J Hepatol 11:232–239, 1990

    PubMed  Google Scholar 

  29. Stechemesser E, Klein R, Berg PA: Characterization and clinical relevance of liver-pancreas antibodies in autoimmune hepatitis. Hepatology 18:1–9, 1993

    PubMed  Google Scholar 

  30. Martini E, Abuaf N, Cavalli F, Durand V, Johanet C, Homberg J-C: Antibody to liver cytosol (anti-LC1) in patients with autoimmune chronic active hepatitis type 2. Hepatology 8:1662–1666, 1988

    PubMed  Google Scholar 

  31. Abuaf N, Johanet C, Chretien P, Martini E, Soulier E, Laperche S, Homberg JC: Characterization of the liver cytosol antigen type 1 reacting with autoantibodies in chronic active hepatitis. Hepatology 6:892–898, 1992

    Google Scholar 

  32. Rabinovitz M, Demetris AJ, Bou-Abboud CF, Van Thiel DH: Simultaneous occurrence of primary sclerosing cholangitis and autoimmune chronic active hepatitis in a patient with ulcerative colitis. Dig Dis Sci 37:1606–1611, 1992

    PubMed  Google Scholar 

  33. Perdigoto R, Carpenter HA, Czaja AJ: Frequency and significance of chronic ulcerative colitis in severe corticosteroid-treated autoimmune hepatitis. J Hepatol 14:325–331, 1992

    PubMed  Google Scholar 

  34. Czaja AJ, Hay JE, Rakela J: Clinical features and prognostic implications of severe corticosteroid-treated cryptogenic chronic active hepatitis. Mayo Clin Proc 65:23–30, 1990

    PubMed  Google Scholar 

  35. Czaja AJ, Carpenter HA, Santrach PJ, Moore SB, Homburger HA: The nature and prognosis of severe cryptogenic chronic active hepatitis. Gastroenterology 104:1755–1761, 1993

    PubMed  Google Scholar 

  36. Czaja AJ: Chronic active hepatitis: The challenge for a new nomenclature. Ann Intern Med 119:510–517, 1993

    PubMed  Google Scholar 

  37. Johnson PJ, McFarlane IG, Alvarez F, Bianchi FB, Bianchi L, Burroughs A, Chapman RW, Czaja AJ, Desmet V, Eddleston ALWF, Gerber MA, Hoofnagle JH, Kakumu S, MacSween RNM, Maddrey WC, Manns MP, Meyer zum Buschenfelde K-H, Mieli-Vergani G, Portmann BC, Reed WD, Schalm SW, Scheuer PJ, Toda G, Tsuji T, Tygstrup N, Vergani G, Zeniya M: Meeting report. International Autoimmune Hepatitis Group. Hepatology 18:998–1005, 1993

    PubMed  Google Scholar 

  38. Desmet VJ, Gerber M, Hoofnagle JH, Manns M, Scheuer PJ: Classification of chronic hepatitis: Diagnosis, grading and staging. Hepatology 19:1513–1520, 1994

    PubMed  Google Scholar 

  39. Ludwig J, Members of the Working Party of the World Congresses of Gastroenterology: Terminology of chronic hepatitis, hepatic allograft rejection, and nodular lesions of the liver: Summary of recommendations developed by an international working party, supported by the World Congress of Gastroenterology, Los Angeles, 1994. Am J Gastroenterol 89:S177-S181, 1994

    PubMed  Google Scholar 

  40. Czaja AJ, Davis GL, Ludwig J, Baggenstoss AH, Taswell HF: Autoimmune features as determinants of prognosis in steroid-treated chronic active hepatitis of uncertain etiology. Gastroenterology 85:713–717, 1983

    PubMed  Google Scholar 

  41. Czaja AJ, Carpenter HA, Santrach PJ, Moore SB: Genetic predispositions for the immunological features of chronic active hepatitis. Hepatology 18:816–822, 1993

    PubMed  Google Scholar 

  42. Czaja AJ, Carpenter HA, Santrach PJ, Moore SB: Significance of HLA DR4 in type 1 autoimmune hepatitis. Gastroenterology 105:1502–1507, 1993

    PubMed  Google Scholar 

  43. Summerskill WHJ, Korman MG, Ammon HV, Baggenstoss AH: Prednisone for chronic active liver disease: Dose titration, standard dose, and combination with azathioprine compared. Gut 16:876–883, 1975

    PubMed  Google Scholar 

  44. Lenzi M, Johnson PJ, McFarlane IG, Ballardini G, Smith HM, McFarlane GM, Bridger C, Vergani D, Bianchi FB, Williams R: Antibodies to hepatitis C virus in autoimmune liver disease: Evidence for geographical heterogeneity. Lancet 338:277–280, 1991

    PubMed  Google Scholar 

  45. Silva E, Sallie R, Tibbs C, McFarlane I, Johnson P, Williams R: Absence of hepatitis C virus in British patients with type 1 autoimmune chronic active hepatitis—a polymerase chain reaction and serological study. J Hepatol 19:211–215, 1993

    PubMed  Google Scholar 

  46. Nakatsuji Y, Matsumoto A, Tanaka E, Ogata H, Kiyosawa K: Detection of chronic hepatitis C virus infection by four diagnostic systems: First-generation and second-generation enzyme-linked immunosorbent assay, second-generation recombinant immunoblot assay and nested polymerase chain reaction analysis. Hepatology 16:300–305, 1992

    PubMed  Google Scholar 

  47. Zein NN, Rakela J, Persing DH: Genotype-dependent serologic reactivities in patients infected with hepatitis C virus in the United States. Mayo Clin Proc 70:449–452, 1995

    PubMed  Google Scholar 

  48. Schalm SW, Ammon HV, Summerskill WHJ: Failure of customary treatment in chronic active liver disease: Causes and management. Ann Clin Res 8:221–227, 1976

    PubMed  Google Scholar 

  49. Czaja AJ, Davis GL, Ludwig J, Taswell HF: Complete resolution of inflammatory activity following corticosteroid treatment of HBsAg-negative chronic active hepatitis. Hepatology 4:622–627, 1984

    PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. From the Division of Gastroenterology and Internal Medicine, and the Department of Laboratory Medicine and Pathology, Mayo Clinic and Mayo Foundation, Rochester, Minnesota

    Albert J. Czaja MD & Herschel A. Carpenter MD

Authors
  1. Albert J. Czaja MD
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Herschel A. Carpenter MD
    View author publications

    You can also search for this author in PubMed Google Scholar

Additional information

Presented in part at the meeting of the American Association for the Study of Liver Diseases, May 14, 1995, San Diego, California.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Czaja, A.J., Carpenter, H.A. Validation of scoring system for diagnosis of autoimmune hepatitis. Digest Dis Sci 41, 305–314 (1996). https://doi.org/10.1007/BF02093820

Download citation

  • Received:

  • Revised:

  • Accepted:

  • Issue Date:

  • DOI: https://doi.org/10.1007/BF02093820

Key words

Search

Navigation