№ 22Gastroenterology14 min read
Primary biliary cirrhosis and primary sclerosing cholangitis
1. Big picture
Primary biliary cirrhosis is now called primary biliary cholangitis (PBC) because many patients are diagnosed before cirrhosis develops. Primary sclerosing cholangitis (PSC) is a different chronic cholestatic disease.
The examiner usually wants you to separate them quickly:
| Feature | PBC | PSC |
|---|---|---|
| Typical patient | Middle-aged woman | Young/middle-aged man |
| Main duct affected | Small intrahepatic bile ducts | Intrahepatic and/or extrahepatic bile ducts |
| Key antibody | Antimitochondrial antibody (AMA), ↑ immunoglobulin M (IgM) | Perinuclear antineutrophil cytoplasmic antibody (p-ANCA) may be positive |
| Strong association | Sjögren syndrome, systemic sclerosis, autoimmune thyroid disease | Ulcerative colitis / inflammatory bowel disease |
| Key investigation | Cholestatic labs + AMA | Magnetic resonance cholangiopancreatography (MRCP): multifocal strictures/beading |
| Main treatment | Ursodeoxycholic acid (UDCA) | No reliably curative drug; treat strictures/cholangitis; transplant if advanced |
| Main cancer risk | Hepatocellular carcinoma mainly if cirrhotic/high-risk | Cholangiocarcinoma + colorectal cancer if PSC-IBD |
PBC is the AMA-positive itchy middle-aged woman. PSC is the UC-associated beaded bile duct disease in a man with cholangiocarcinoma risk.
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