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Diagnostic Criteria and Scoring System for Autoimmune Hepatitis

AP: AST (or ALT)
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AP: AST (or ALT)
>3
<1.5
γ-globulin or IgG levels above normal values
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γ-globulin or IgG levels above normal values
>2.0
1.5-2.0
1.0-1.5
<1.0
ANA, SMA, or anti-LKM-1 titer
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ANA, SMA, or anti-LKM-1 titer
>1:80
1:80
1:40
<1:40
Viral Markers
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Viral Markers
Positive
Negative
Medication
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Medication
Yes
No
Alcohol
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Alcohol
<25g/d
>60g/d
Histological Findings
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Histological Findings
Interface Hepatitis
Plasma Cell Infiltration
Rosette Formation
No Above Lesions
Biliary Changes
No Lesions
Treatment Response
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Treatment Response
Effective
Relapse
Score:

Result Interpretation

Diagnostic Criteria Established by the International AIH Working Group:
Features Confirmed Diagnosis Suspected
Histology Moderate to severe interface hepatitis, with or without lobular hepatitis or central-portal bridging necrosis, but without biliary changes or obvious changes indicating other causes. Same as confirmed cases.
Serum Biochemistry Various serum transaminase abnormalities, especially without significant elevation of serum alkaline phosphatase. Normal serum α1-antitrypsin, copper, and ceruloplasmin levels. Same as confirmed cases, but with abnormal serum copper or ceruloplasmin levels, related tests should exclude Wilson's disease.
Serum Immunoglobulins Total serum globulin or γ-globulin or IgG levels > 1.5 × UNL. Various serum globulins or γ-globulin or IgG levels above the normal upper limit.
Serum Autoantibodies Serum ANA, SMA, or anti-LKM-1 antibody titers > 1:80. Low titers in children (especially anti-LKM-1 antibodies) are significant. Serum AMA negative. Same as confirmed cases, but titers ≥ 1:40. Negative for this type of serum antibody but positive for other serum antibodies.
Viral Markers Serum markers for hepatitis A, B, and C virus infections negative. Same as confirmed cases.
Other Causes Average alcohol consumption < 25g/d. No recent history of hepatotoxic drug use. Alcohol consumption < 50g/d and no recent history of hepatotoxic drug use. Indications of significant alcohol consumption or recent use of hepatotoxic drugs, such as persistent liver damage after quitting alcohol or stopping drugs.

1. Diagnostic Criteria:

*Other serum antibodies: including perinuclear ANCA, anti-soluble liver antigen antibodies/liver-pancreatic antigen antibodies, anti-actin antibodies, anti-liver cytosol type I antibodies, and anti-sialoglycoprotein receptor antibodies.

2. Scoring System:

Pre-treatment score: confirmed cases > 15; suspected cases 10-15.

Post-treatment score: confirmed cases > 17; suspected cases 12-17.

In 1993 and 1999, the International AIH Working Group established the diagnostic criteria and scoring system for AIH. Clinical diagnostic criteria meet the needs for clear diagnosis or exclusion of AIH in most cases. The original scoring system serves as a research tool, allowing comparability in clinical trials and is also applicable to difficult-to-meet suspected cases. The original scoring system grades treatment response and can show different scores before and after treatment.

The sensitivity for a pre-treatment score of 10 points is 100%, with a specificity of 73%, and a diagnostic accuracy of 67%. A pre-treatment score of 15 points indicates "diagnosed AIH" with a sensitivity of 95%, specificity of 97%, and diagnostic accuracy of 94%. Retrospective studies on pediatric AIH cases support the application value of the originally established scoring system.