Revised Original Score for Autoimmune Hepatitis (AIH)
Patients with established hepatitis and available liver biopsy results, but unclear diagnosis.
AIH is multifactorial, complex, and typically has many competing diagnoses. The score offers a data driven, consensus-based path to help guide treatment.
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.
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 markers1 |
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 parameters3 |
Seropositivity for other defined autoantibodies4 |
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.
Facts & Figures
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 |
Evidence Appraisal
The conventional AIH score has been tested by numerous groups, typically in referral populations using database and chart review validations. With many complex inputs the score codifies an accepted and user friendly approach to diagnosis, but has been criticized for low specificity and thus false positives, particularly in the ‘probable AIH’ category.
About the Creator
Fernando Alvarez, MD, is the Chief of the Division of Gastroenterology, Hepatology & Nutrition at CHU Sainte-Justine, University of Montreal. He studied medicine in his home country of Argentina, where he focused on hepatology and pediatrics. He currently practices pediatric hepatology, and he researches topics including autoimmune hepatitis, viral hepatitis, and liver immunity.
To view Dr. Fernando Alvarez's publications, visit PubMed