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AIH
PBC[23]
PBS
Overlap syndrome (AIH-PBC/PBS)
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Various clinical manifestations
Characterized by hypergammaglobulinemia, positive circulating
autoantibodies, histologic interface hepatitis, and liver function
impairment[14][15].
Type 1: ANA and/or anti-SMA: positive, type 2: anti-LKM-1 and/or
anti-LC-1: positive[22].
Fatigue, pruritus, skin pigmentation.
Typical signs of cirrhosis, portal hypertension, and cholestasis.
ALP: increased and lasted for more than six months, γ-GTP: increased,
and total bilirubin: typically normal.
AMA: positive.
Chronic cholestatic liver disease, inflammation, and fibrosis lead to
multifocal biliary stricture and progression to end-stage liver
disease[23].
ALP: increase
Circulating autoantibody: Positive
One of the subgroups of AIH mainly manifested in hepatitis and
cholestasis serum liver tests and has the histological characteristics
of AIH, PBC, or PSC[18,19].
AST, ALT, ALP, GGT: increase
AIH-PBC: AMA+ANA and/or ASMA
AIH-PBS: P-ANCA±ANA and/or ASMA
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Interface hepatitis with lymphoplasmacytic infiltrates,
hepatocellular rosette, emperipolesis,
hepatocyte swelling or pyknotic necrosis[21].
Florid duct lesion,
Epithelioid granulomas,
Ductopaenia,
Feathery degeneration of liver cytoplasm.
Onion skin fibrosis: inflammatory infiltration of intrahepatic and
extrahepatic bile duct wall, with occlusive concentric peritubular loose
fibrosis,
Bile duct scar: degeneration and atrophy of bile duct cells, resulting
in stricture and occlusion[24].
AIH-PBC: Interface hepatitis + ductopenia
AIH-PBS: Interface hepatitis + ductal impairment.
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