AIH_Revised_Original_Score_guideline v.1

Revised Original Score for Autoimmune Hepatitis (AIH) for the diagnosis of autoimmune hepatitis.

Maryam Razavi

maryam.razavi@cambio.se

Cambio CDS

To diagnose autoimmune hepatitis.

Use in 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, >2.0 3 times above upper limit of normal 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 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): No 0 Yes 2 Optional additional parameters Seropositivity for other defined autoantibodies4 2 HLA-DR3 or HLA-DR4 1 Response to therapy: Complete 2 Relapse 3 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

1. Alvarez F, Berg PA, Bianchi FB, Bianchi L, Burroughs AK, Cancado EL, Chapman RW, Cooksley WG, Czaja AJ, Desmet VJ, Donaldson PT, Eddleston AL, Fainboim L, Heathcote J, Homberg JC, Hoofnagle JH, Kakumu S, Krawitt EL, Mackay IR, MacSween RN, Maddrey WC, Manns MP, McFarlane IG, Meyer zum Büschenfelde KH, Zeniya M, et al. International Autoimmune Hepatitis Group Report: review of criteria for diagnosis of autoimmune hepatitis. J Hepatol. 1999 Nov;31(5):929-38. doi: 10.1016/s0168-8278(99)80297-9. PMID: 10580593. 2. Czaja AJ. Performance parameters of the diagnostic scoring systems for autoimmune hepatitis. Hepatology. 2008 Nov;48(5):1540-8. doi: 10.1002/hep.22513. PMID: 18924244.

OBSERVATION.aih_score_revised.v0, EVALUATION.aih_revised_original_score_assessment.v0