Criteria

The diagnostic scoring system of the International Autoimmune Hepatitis Group was created by an international panel in 1993, revised in 1999, and simplified in 2008.[1][39][40]​​ The original revised scoring system has greater sensitivity for autoimmune hepatitis (AIH) compared to the simplified scoring system (100% vs. 95%), whereas the simplified scoring system has superior specificity (90% vs. 73%) and accuracy (92% vs. 82%), using clinical judgment as the gold standard. The revised diagnostic scoring system is preferable for patients with complex or unusual features, whereas the simplified scoring system is most accurate for typical patients. Guidelines published by the European Association for the Study of the Liver caveat that while the simplified criteria are user-friendly and a good tool for daily clinical practice, without a diagnostic ‘gold standard’ clinicians must regard any diagnostic score only as an aid to diagnosis of AIH and the criteria should be used alongside clinical judgment.[26]​​

Diagnostic criteria for autoimmune hepatitis. Based on recommendations of the International Autoimmune Hepatitis Group[39]

Requisites

  1. No genetic liver disease

    • Definite: normal alpha-1-antitrypsin phenotype, normal serum ceruloplasmin, iron, and ferritin levels

    • Probable: partial alpha-1-antitrypsin deficiency, non-specific serum copper, ceruloplasmin, iron, and/or ferritin abnormalities.

  2. No active viral infection

    • Definite: no markers of current infection with hepatitis A, B, and C viruses

    • Probable: no markers of current infection with hepatitis A, B, and C viruses.

  3. No toxic alcohol injury

    • Definite: daily alcohol less than 25 g/day and no recent use of hepatotoxic drugs

    • Probable: daily alcohol less than 50 g/day and no recent use of hepatotoxic drugs.

  4. Laboratory features

    • Definite: predominant serum aminotransferase abnormality. Globulin, gamma-globulin, or immunoglobulin (Ig) G level of 1.5 times normal or greater

    • Probable: predominant serum aminotransferase abnormality. Hypergammaglobulinaemia of any degree.

  5. Auto-antibodies

    • Definite: antinuclear antibodies (ANAs), smooth muscle antibodies (SMAs), or anti-liver kidney microsomal-1 (anti-LKM-1) antibodies of 1:80 or greater in adults and 1:20 or greater in children; no anti-mitochondrial antibodies (AMAs)

    • Probable: ANA, SMA, or anti-LKM-1 antibodies of 1:40 or greater in adults or other auto-antibodies (perinuclear anti-neutrophil cytoplasmic antibodies [pANCA], anti-soluble liver antigens (anti-SLA), or liver/pancreas, anti-actin, liver cytosol 1 antigen antibodies [anti-LC1], asialoglycoprotein receptor antibodies [anti-ASGPR]).

Histological findings

  • Definite and probable: interface hepatitis. No biliary lesions, granulomas, or prominent changes suggestive of another disease.

Revised original scoring system for the diagnosis of autoimmune hepatitis. Based on recommendations of the International Autoimmune Hepatitis Group[40]

Parameters/features (score)

  • Sex

    • Female: +2

    • Male: 0

  • Alkaline phosphatase: aspartate transaminase (or alanine transaminase) ratio (degree of elevation above upper limit of normal)

    • <1.5: +2

    • 1.5 to 3.0: 0

    • >3.0: -2

  • Serum gamma-globulins or IgG (times above upper limit of normal)

    • >2.0: +3

    • 1.5 to 2.0: +2

    • 1.0 to 1.5: +1

    • <1.0: 0

  • ANAs, SMAs, or LKM-1 antibodies (determined by indirect immunofluorescence on rodent tissues or HEp-2 cells)

    • >1:80: +3

    • 1:80: +2

    • 1:40: +1

    • <1:40: 0

  • AMA positive: -4

  • Hepatitis viral markers (IgM anti-hepatitis A virus, HBsAg, IgM anti-hepatitis B antibodies, hepatitis C antibody, and hepatitis C virus RNA)

    • Positive: -3

    • Negative: +3

  • Recent or current use of hepatotoxic drugs

    • Yes: -4

    • No: +1

  • Human leukocyte antigens

    • DR3 or DR4

  • Other autoimmune disease(s)

    • Thyroiditis, colitis, others: +2

  • Other markers

    • Anti-soluble liver antigen, anti-actin, anti-LC1, perinuclear anti-neutrophil cytoplasmic antibodies (pANCA): +2

  • Average alcohol intake

    • <25 g/day: +2

    • >60 g/day: -2

  • Liver histology

    • Interface hepatitis: +3

    • Plasmacytic: +1

    • Rosettes of liver cells: +1

    • None of the above: -5

    • Biliary changes: -3

    • Other changes: -3

  • Response to therapy

    • Complete (remission): +2

    • Relapse: +3

Interpretation of aggregate scores

  • Pre-treatment: definite AIH, >15; probable AIH, 10 to 15

  • Post-treatment: definite AIH, >17; probable AIH, 12 to 17.

Simplified criteria for the diagnosis of autoimmune hepatitis[40]

Where score ≥6 indicates probably AIH; score ≥7 definite AIH.

Parameter (score)

  • ANA or SMA ≥1:40:1

  • ANA or SMA ≥1:80 OR anti-LKM ≥1:40 OR anti-SLA positive: 2

  • IgG level

    • >Upper limit of normal: 1

    • >1.1 times upper limit of normal: 2

  • Liver histology

    • Compatible with AIH: 1

    • Typical of AIH: 2

  • Absence of viral disease: 2.

Clinical criteria[1][26][36]

Patients are described as having severe disease if they present with any of the following:

  • Serum aminotransferase levels greater than 10-fold the upper limit of normal

  • Serum aminotransferase levels 5-fold the upper limit of normal with a serum gamma-globulin level at least twice the upper limit of normal

  • Bridging necrosis or multi acinar necrosis on liver histology.

The American Association for the Study of Liver Diseases and the European Association for the Study of the Liver define acute severe AIH as:

  • Jaundice

  • International normalised ratio >1.5 to <2

  • No encephalopathy

  • No previously recognised liver disease.

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