Revised Original Score for Autoimmune Hepatitis (AIH)

Revised Original Score for AIH

Sex

ALP:AST (or ALT) Ratio

Serum Globulins or IgG (above ULN)

ANA, SMA, or Anti-LKM1 Titer

AMA Positive

Hepatitis Viral Markers

Recent Hepatotoxic Drug Use

Average Alcohol Intake

Liver Histology

Other Autoimmune Disease (patient or 1st-degree relative)

Additional Markers (anti-SLA/LP, anti-actin, anti-LC1, pANCA)

HLA DR3 or DR4

Treatment Response

Pre-treatment Score: –

Awaiting input

Select all 13 criteria.

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

Why Use

AIH is multifactorial, complex, and typically has many competing diagnoses. The score offers a data driven, consensus-based path to help guide treatment.

When to Use

Patients with established hepatitis and available liver biopsy results, but unclear diagnosis.

Formula

Addition of the selected points: Variable Points Sex Male 0 Female 2 ALP : AST or ALP : ALT ratio <1.5 2 1.5-3.0 0 >3.0 -2 Serum globulins or IgG, times above upper limit of normal >2.0 3 1.5-2.0 2 1.0-1.5 1 <1.0 0 Antinuclear antibody (ANA), smooth muscle antibody (SMA), or liver/kidney microsomal type 1 (LKM-1) antibody >1:80 3 1:80 2 1:40 1 <1:40 0 Anti-mitochondrial antibody (AMA) Positive -4 Negative 0 Hepatitis viral markers 1 Positive -3 Negative +3 Recent or current use of hepatotoxic drugs Yes -4 No 1 Average alcohol intake <25 g/day 2 25-60 g/day 0 >60 g/day -2 Liver histology Interface hepatitis 3 Predominantly lymphoplasmacytic infiltrate 1 Rosetting of liver cells 1 No interface hepatitis, not predominantly lymphoplasmacytic infiltrate, and no rosetting of liver cells -5 Biliary changes (bile duct changes typical of primary biliary cirrhosis or primary scleroisng cholangitis, and/or periportal ductal reaction with copper-associated protein accumulation) -3 Other changes (any other prominent features suggesting different etiology) -3 Other autoimmune disease(s) (in patient or 1st degree relatives) 2 No 0 Yes 2 Optional additional parameters 3 Seropositivity for other defined autoantibodies 4 2 HLA-DR3 or HLA-DR4 1 Response to therapy Complete 2 Relapse 3 Footnotes: 1 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. 2 In patient or 1st degree relatives. 3 Assign points only in patients seronegative for ANA, SMA and LKM-1. 4 e.g. pANCA, anti-LCl, anti-SLA, anti-ASGPR, anti-LP and anti-sulfatide.

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.

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.

More Information

Interpretation: AIH Score Pre- or post treatment Diagnosis >15 Pre-treatment Definite AIH 10-15 Probable AIH >17 Post-treatment Definite AIH 12-17 Probable AIH