FeaturePrimary Sclerosing Cholangitis (PSC)Primary Biliary Cholangitis (PBC)Autoimmune Hepatitis (AIH)
PathoInflammation/fibrosis of intra- & extrahepatic bile ductsAutoimmune destruction of intrahepatic bile ductsAutoimmune destruction of hepatocytes
Epi / Assoc.M > F (2:1), <40s
Assoc: Ulcerative Colitis (IBD) (>80%)
F >> M (9:1), 40-60s
Assoc: Sjögren’s, other autoimmune dz
F > M (3:1), bimodal (young/middle-aged)
Assoc: Other autoimmune dz
LabsCholestatic: ↑↑ ALP, ↑ GGTCholestatic: ↑↑ ALP, ↑ GGTHepatocellular: ↑↑↑ AST/ALT (>1000s common), ↑ IgG
Serology(+) p-ANCA(+) AMA (Anti-Mitochondrial Ab)(+) ANA, (+) ASMA (Anti-Smooth Muscle Ab)
Dx / HistoMRCP/ERCP: “Beads on a string”
Histo: “Onion skinning” periductal fibrosis
Normal imaging
Histo: Florid duct lesion (lymphocytic cholangitis, granulomas)
Normal/nonspecific imaging
Histo: Interface hepatitis, plasma cells
TxSymptomatic Tx; Liver transplant (definitive)Ursodeoxycholic acid (UDCA)Corticosteroids, Azathioprine
Key RiskCholangiocarcinoma, Colorectal Cancer (w/ UC)Osteoporosis, Cirrhosis, HCCCirrhosis, Acute Liver Failure