TABLE 1.
Diagnostic features of the overlap syndromes of autoimmune hepatitis (AIH)
| Overlap syndrome | Laboratory features | Serological features | Histological features | Cholangiographic findings |
|---|---|---|---|---|
| AIH-PBC | Consistent with Paris criteria*(19,30) Mild forms may have AP ≤2×ULN (2,6) |
AMA positive (2) | Interface hepatitis (30) Destructive cholangitis (florid duct lesions) (30) |
Normal (6) |
| AIH-PSC | AST/ALT>ULN (2) γ-globulin and IgG >ULN (2) AP or GGT>ULN (2) |
AMA negative (2) | Interface hepatitis (34) Ductopenia (34) Portal edema or fibrous (34) Obliterative fibrous cholangitis (rare) (34) |
Bile duct strictures(2,18,20,72) |
| AIH-cholestatic syndrome | AST/ALT>ULN (2) γ-globulin and IgG >ULN (2) AP or GGT >ULN (2) |
AMA negative (2) | Interface hepatitis (34) Destructive cholangitis or bile duct loss (11,27) |
Normal (2,11,27) |
Numbers in parentheses refer to references.
*
Paris criteria endorsed by the European Association for the Study of the Liver require interface hepatitis and either serum alanine aminotransferase (ALT) level ≥5-fold upper limit of normal range (ULN), serum immunoglobulin G (IgG) level ≥2-fold ULN or smooth muscle antibodies, and two of three features of primary biliary cirrhosis (PBC) including serum alkaline phosphatase (AP) level ≥2-fold ULN or serum gamma glutamyl transferase (GGT) ≥5-fold ULN, antimitochondrial antibodies (AMA) and destructive cholangitis. AST Serum aspartate aminotransferase level; PSC Primary sclerosing cholangitis