Liver disease Characteristics of clinical manifestations and biochemical indicators Histological characteristics of liver
AIH PBC[23] PBS Overlap syndrome (AIH-PBC/PBS) 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 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.