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.