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    Simplified Autoimmune Hepatitis (AIH) Score

    Diagnoses autoimmune hepatitis.
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    INSTRUCTIONS

    Use in patients with histological evidence of hepatitis. Do not use in patients with normal histology.

    When to Use
    Pearls/Pitfalls
    Why Use

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

    The Simplified Autoimmune Hepatitis (AIH) Score was developed to simplify AIH diagnosis and reduce false positive diagnoses.

    AIH is multifactorial, complex, and typically has many competing diagnoses. The score offers a data driven, consensus-based path to help guide treatment and is likely to be better than clinical judgment alone.

    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. AIH scoring neatly solves this problem by defining the diagnosis. ‘Likely’ scores appear highly reliable.

    Management

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

    Critical Actions

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

    Formula

    Addition of the selected points:

    Variable

    0 points

    1 point

    2 points

    ANA or SMA

    <1:40

    ≥1:40

    ≥1:80*

    LKM1

    <1:40

    --

    ≥1:40*

    SLA

    Negative

    --

    Positive*

    IgG

    Normal

    ≥Upper limit of normal

    ≥1.1× upper limit of normal

    Liver histology**

    --

    Compatible with AIH

    Typical of AIH

    Viral hepatitis

    Present

    --

    Absent


    *Maximum 2 points total (e.g. if ANA or SMA ≥1:80 and SLA is positive, only 2 points are assigned, not 4).

    **If histology is normal, AIH is not the diagnosis.

    AIH, autoimmune hepatitis; ANA, antinuclear antibody; LKM1, Liver Kidney Microsome type 1; SLA, soluble liver antigen; SMA, smooth muscle antibody; IgG, immunoglobulin G.

    Facts & Figures

    Interpretation:

    Simplified AIH Score

    Diagnosis

    ≤5

    Possible AIH (though further testing should be done)

    6

    Probable AIH

    ≥7

    Likely AIH

    Evidence Appraisal

    The Simplified AIH score has been tested in a number of studies, including by comparison to its progenitor, the conventional score. Validations are typically in referral populations using database and chart review. The score is intended to improve specificity and reduce false positives over the conventional score, particularly in the ‘probable AIH’ category.

    Dr. Ansgar W. Lohse

    From the Creator

    Why did you develop the Simplified AIH Score? Was there a particular clinical experience or patient encounter that inspired you to create this tool for clinicians?

    The diagnosis of AIH is difficult, and the disease is often overlooked. The International Autoimmune Hepatitis Group had developed a score useful for comparison of patient groups in scientific papers, but it was too cumbersome for everyday use. In addition, it was only reliable post-hoc, i.e., when the response to therapy could be evaluated. What was needed was a score to help decide if a trial of immunosuppression should be undertaken.

    What recommendations do you have for doctors once they have applied the Simplified AIH Score? Are there any adjustments or updates you would make to the score based on new data or practice changes?

    1. The score only works,if you have a liver biopsy result, and good liver histopathologists are not always available. The minimum requirement is the histological demonstration of Inflammation, i.e., hepatitis.

    2. In very acute AIH, the histological picture may look exactly like drug-induced liver injury (immunoallergic hepatitis) with centrilobular necroses, and not yet periportal hepatitis. At the same time, in very acute AIH, IgG levels and autoantibodies may not yet be present. In these patients, a trial of prednisolone monotherapy is justified (see the EASL Clinical Practice Guidelines on AIH).

    What pearls, pitfalls and/or tips do you have for users of the Simplified AIH Score? Do you know of cases when it has been applied, interpreted, or used inappropriately?

    The simplified AIH Score was developed using immunofluorescence testing for ANA and SMA autoantibodies, which is the international standard. However, in the U.S. and some other countries, ELISA testing for these autoantibodies is widely used. The score is not validated for these assays, and should therefore be used with caution.

    About the Creator

    Ansgar W. Lohse, MD, is Chairman of the Department of Medicine at the University Medical Center Hamburg-Eppendorf in Hamburg, Germany. He specializes in gastroenterology and infectious disease. Professor Lohse has authored over 300 peer-reviewed research papers in clinical and basic science hepatology.

    To view Dr. Ansgar W. Lohse's publications, visit PubMed