The revised international autoimmune hepatitis score in chronic liver diseases including autoimmune hepatitis/overlap syndromes and autoimmune hepatitis with concurrent other liver disorders

Panagiotis A Papamichalis¹^,2 , Kalliopi Zachou¹^,2 , George K Koukoulis³ , Aikaterini Veloni² , Efthimia G Karacosta² , Lampros Kypri² , Ioannis Mamaloudis² , Stella Gabeta¹ , Eirini I Rigopoulou² , Ansgar W Lohse⁴ and George N Dalekos¹^,2

¹Dept of Medicine, Research Laboratory of Internal Medicine, Medical School, University of Thessaly, 22 Papakiriazi str, Larissa 41222, Greece

²Dept. of Medicine, Academic Liver Unit, Medical School, University of Thessaly, 22 Papakiriazi str, Larissa 41222, Greece

³Dept. of Pathology, Medical School, University of Thessaly, 22 Papakiriazi str, Larissa 41222, Greece

⁴Dept. of Medicine I, University Medical Center Hamburg-Eppendorf, Hamburg, Germany

author email corresponding author email

Journal of Autoimmune Diseases 2007, 4:3doi:10.1186/1740-2557-4-3


Published:	29 June 2007

Abstract

Background

We conducted a study in order to determine the usefulness and diagnostic value of International Autoimmune Hepatitis Group (IAHG) score in non-autoimmune hepatitis (AIH) hepatic disorders as well as in AIH/overlap syndromes and in cases with coexistence of AIH and other liver diseases.

Methods

We applied the IAHG score in 423 patients with liver diseases excluding patients with AIH, AIH/overlap syndromes and AIH with concurrent other liver disease namely, patients with chronic hepatitis B (n = 109), chronic hepatitis C (n = 95), chronic hepatitis D (n = 4), alchoholic liver disease (n = 28), non-alcoholic fatty liver disease (n = 55), autoimmune cholestatic liver diseases (n = 77), liver disorders of undefined origin (n = 32) and with miscellaneous hepatic disorders (n = 23). 24 patients with AIH associated with any kind of liver disorder including 10 patients with AIH/overlap syndromes and 14 AIH with concurrent other liver disease were also investigated. 43 patients with AIH consisted the control group.

Results

The specificity of the score was 98.1% while the sensitivity in unmasking AIH in patients with either AIH/overlap syndromes or AIH with concurrent other liver diseases was only 50% and 78.6%. In the binary logistic regression model, the presence of other autoimmune diseases (p < 0.001), the total histological score (p < 0.001) and positivity for autoantibodies (p < 0.05) were identified as independent predictors for the presnce of AIH/ovea syndromes o AI with concurren other liver diseass.

Conclusion

The IAHG scoring system has very good specificity for excluding AIH in patients with chronic liver diseases but not that sensitivity in order to unmask AIH/overlap syndromes or AIH with concurrent other liver diseases. The presence of other autoimmune diseases or autoantibody markers in the absence of hepatitis viral markers should alarm physicians for the possible presence of AIH either as "pure" AIH or in association with other liver disorders (AIH/overlap syndromes or AIH with concurrent other liver diseases). Under these conditions, liver histology seems essential and it must always be included in the work up of hepatic patients.