MDCalc

Revised Original Score for Autoimmune Hepatitis (AIH)

Revised version of the original scoring system for the diagnosis of autoimmune hepatitis.

Sex
ALP:AST or ALP:ALT ratio
Serum globulins or IgG
Times above upper limit of normal
ANA, SMA, or LKM-1 antibody
Optional additional parameters
If ANA, SMA, LKM-1 are negative
AMA
Hepatitis viral markers
Hepatitis A, B and C viruses (i.e., positive/negative for IgM anti-HAV, HBsAg, IgM anti-HBc, anti-HCV and HCV-RNA); if all are negative and viral etiology is still suspected, CMV/EBV testing may be relevant
Recent or current use of hepatotoxic drugs
Average alcohol intake
Liver histology: interface hepatitis
Liver histology: predominantly lymphoplasmacytic infiltrate
Liver histology: rosetting of liver cells
If no interface hepatitis, not predominantly lymphoplasmacytic infiltrate, and no rosetting of liver cells, then subtract 5 points
Liver histology: biliary changes
Bile duct changes typical of primary biliary cirrhosis or primary scleroisng cholangitis, and/or periportal ductal reaction with copper-associated protein accumulation
Liver histology: other changes
Any other prominent features suggesting different etiology
Other autoimmune disease(s)
In patient or 1st degree relatives
Response to therapy

Result:

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Advice

AIH is a complex diagnosis partly because there is no anatomic criterion (i.e. ’gold’) standard. The revised (1999), conventional AIH score solves this problem by defining the diagnosis, though some would say the recently developed, more specific, simplified version offers a preferred alternative.

Management

We are unaware of validated management algorithms using the AIH score.

Critical Actions

Overlap with multiple conditions is always a concern to consider, despite score findings, and ‘definite’ scores are considerably more predictive than ‘probable’ scores.

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