Autoimmune Liver Diseases
| Feature | Primary Sclerosing Cholangitis (PSC) | Primary Biliary Cholangitis (PBC) | Autoimmune Hepatitis (AIH) |
|---|---|---|---|
| Patho | Inflammation/fibrosis of intra- & extrahepatic bile ducts | Autoimmune destruction of intrahepatic bile ducts | Autoimmune 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 |
| Labs | Cholestatic: ↑↑ ALP, ↑ GGT | Cholestatic: ↑↑ ALP, ↑ GGT | Hepatocellular: ↑↑↑ AST/ALT (>1000s common), ↑ IgG |
| Serology | (+) p-ANCA | (+) AMA (Anti-Mitochondrial Ab) | (+) ANA, (+) ASMA (Anti-Smooth Muscle Ab) |
| Dx / Histo | MRCP/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 |
| Tx | Symptomatic Tx; Liver transplant (definitive) | Ursodeoxycholic acid (UDCA) | Corticosteroids, Azathioprine |
| Key Risk | Cholangiocarcinoma, Colorectal Cancer (w/ UC) | Osteoporosis, Cirrhosis, HCC | Cirrhosis, Acute Liver Failure |
