Revised Original Score for Autoimmune Hepatitis (AIH)

Sex

ALP:AST (or ALP:ALT) ratio

Serum globulins or IgG (x upper limit of normal)

ANA, SMA, or anti-LKM1 titer

Antimitochondrial antibody (AMA)

Viral hepatitis markers

Recent hepatotoxic drug use

Average alcohol intake

Liver histology: interface hepatitis

Liver histology: predominantly lymphoplasmacytic infiltrate

Liver histology: rosetting of liver cells

Liver histology: biliary changes suggesting another diagnosis

Liver histology: other changes suggesting another diagnosis

Other autoimmune disease (patient or first-degree relative)

Seropositive for other autoantibodies (anti-SLA, actin, LC1, pANCA)

HLA DR3 or DR4 positive

Response to immunosuppressive therapy

Result-1 points

Possible AIH / diagnosis not supported (pre-treatment thresholds).

Histology subscore: -5

When to use

Complex or atypical presentations of suspected autoimmune hepatitis where the simplified score is insufficient or research-grade diagnostic certainty is needed.

Formula

Sum of weighted parameters (sex, ALP:AST ratio, globulin/IgG elevation, autoantibody titer, AMA, viral markers, hepatotoxic drugs, alcohol intake, liver histology, other autoimmune disease, other autoantibodies, HLA DR3/DR4, and treatment response). Histology: interface hepatitis (+3), lymphoplasmacytic infiltrate (+1), and rosetting (+1) are additive; -5 applies if none of these three is present; biliary changes (-3) and other features suggesting an alternative diagnosis (-3) are additional deductions. Pre-treatment: >15 definite, 10-15 probable AIH. Post-treatment (if response assessed): >17 definite, 12-17 probable AIH.

Pearls and pitfalls

Largely superseded in routine practice by the simplified IAIHG score (2008), but retains higher sensitivity for atypical presentations.

References

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(5):929-938.