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    Patent Pending

    Revised Original Score for Autoimmune Hepatitis (AIH)

    Revised version of the original scoring system for the diagnosis of autoimmune hepatitis.
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    When to Use
    Why Use

    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.

    Male
    0
    Female
    +2
    <1.5
    +2
    1.5-3.0
    0
    >3.0
    -2
    >2.0
    +3
    1.5-2.0
    +2
    1.0-1.5
    +1
    <1.0
    0
    >1:80
    +3
    1:80
    +2
    1:40
    +1
    <1:40
    0
    Seropositivity for other defined autoantibodies
    +2
    HLA-DR3 or HLA-DR4
    +1
    None of the above
    0
    Positive
    -4
    Negative
    0
    Positive
    -3
    Negative
    +3
    Yes
    -4
    No
    +1
    <25 g/day
    +2
    25-60 g/day
    0
    >60 g/day
    -2
    Yes
    +3
    No
    0
    Yes
    +1
    No
    0
    Yes
    +1
    No
    0
    Yes
    -3
    No
    0
    Yes
    -3
    No
    0
    Yes
    +2
    No
    0
    Complete
    +2
    Relapse
    +3

    Result:

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    Next Steps
    Evidence
    Creator Insights

    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.

    Dr. Fernando Alvarez

    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

    About the Creator
    Dr. Fernando Alvarez