357 Ch 356. Autoimmune Hepatitis (AIH), Primary Biliary Cholangitis (PBC), and Primary Sclerosing Cholangitis (PSC)

Autoimmune liver diseases 三大：(1) AIH (autoimmune hepatitis) — chronic hepatocellular inflammation, female predominance, ANA + ASMA + ↑ IgG, prednisone + azathioprine 標準；(2) PBC (primary biliary cholangitis, formerly cirrhosis) — chronic destructive bile duct inflammation, ANA + AMA (anti-mitochondrial antibody, 95%), ursodeoxycholic acid (UDCA) 13-15 mg/kg first-line + obeticholic acid (FDA 2016) for inadequate response；(3) PSC (primary sclerosing cholangitis) — chronic fibrosing inflammation of intrahepatic + extrahepatic bile ducts, strong UC association (80%), MRCP “beading”, pANCA, no effective medical therapy except UDCA modest, ↑ cholangiocarcinoma risk (10-15%)；overlap syndromes (AIH + PBC, AIH + PSC) common；2024-2025 emerging：elafibranor (Iqirvo) PPAR α/δ FDA 2024 for PBC, seladelpar (Livdelzi) PPAR δ FDA 2024 for PBC；IgG4-related cholangitis mimics PSC; liver transplant for end-stage all three。