| Requirements | Definite Diagnostic Criteria | Criteria for Probable Diagnosis |
|---|---|---|
| Absence of liver disease of genetic origin | Normal phenotype for alpha 1 antitrypsin. Normal serum levels of ceruloplasmin, iron, and ferritin | Partial deficiency of alpha 1 antitrypsin. Non-specific abnormalities in the levels of ceruloplasmin, iron, and/or ferritin |
| Absence of active viral infection | Absence of markers for active A, B, or C virus hepatitis | Absence of markers for active A, B, or C virus hepatitis |
| Absence of toxic lesions or lesions caused by alcohol | Daily alcohol intake <25 g/d and no recent use of hepatotoxic drugs | Daily alcohol intake <50 g/d and no recent use of hepatotoxic drugs |
| Laboratory findings | Increase ≥1.5 fold of serum aminotransferases, levels of globulins, gammaglobulins or immunoglobulin G | Increase in serum aminotransferases. Any degree of hypergammaglobulinemia |
| Autoantibodies | ANA, SMA, or anti-LKM1 titers ≥ 80 for adults and ≥ 20 for children. Absence of AMA | ANA, SMA, or anti-LKM1 ≥ 40 in adults or the presence of other autoantibodies* |
| Histologic findings | Interface hepatitis. No biliar lesions, granulomas of changes suggesting other liver diseases | Interface hepatitis. No biliar lesions, granulomas of changes suggesting other liver diseases |
Including perinuclear anti-neutrophil cytoplasm antibodies (p-ANCA) and antigens which are generally not exposed such as the soluble liver antigens/liver-pancreas antigen (anti-SLA/LP), actin and type 1 liver cytosolic protein antibody (anti-LC1), and anti-asialoglycoprotein receptor antibody. Abbreviations. ANA: antinuclear antibodies, SMA: anti-smooth muscle antibodies, anti-LKM1: anti-type 1 liver and kidney microsome antibodies, AMA: anti-mitochondrial antibodies.
From: Chapter 31, Autoimmune hepatitis
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