Makindo Medical Notes

Related Subjects: |Chronic liver disease |Cirrhosis |Alkaline phosphatase (ALP) |Liver Function Tests |Ascites Assessment and Management |Budd-Chiari syndrome |Autoimmune Hepatitis |Primary Biliary Cirrhosis |Primary Sclerosing Cholangitis |Wilson disease |Hereditary Haemochromatosis |Alpha-1 Antitrypsin (AAT) deficiency |Non alcoholic steatohepatitis (NASH) |Spontaneous Bacterial Peritonitis |Alcoholism and Alcoholic Liver Disease

In advanced Primary Sclerosing Cholangitis (PSC), liver transplantation is the only curative option. Survival post-transplant is generally good, though PSC may recur in the graft in 20–25% of cases.

📖 About

• PSC is a chronic, progressive cholestatic liver disease characterised by fibrosis, inflammation, and obliteration of intrahepatic and extrahepatic bile ducts.
• It is the most common indication for liver transplantation in young men in the UK.
• Strongly associated with Ulcerative Colitis (seen in ~75% of PSC cases).
• Up to 10–15% of patients develop cholangiocarcinoma.

🦠 Aetiology

• Exact cause unknown; considered an autoimmune-mediated disease.
• ~50–75% of cases associated with Inflammatory Bowel Disease (predominantly UC).
• Can be classified as:
  • Primary (idiopathic, most common)
  • Secondary (due to surgical injury, infection, or ischaemia of bile ducts)

🔍 Clinical Features

• Often presents in a patient with known IBD.
• Jaundice, pruritus, malaise, and weight loss are common.
• Recurrent episodes of cholangitis (fever, RUQ pain, jaundice).
• Progression leads to cirrhosis and features of portal hypertension.

🧪 Investigations

• LFTs:
  • Isolated ↑ ALP and GGT early on
  • Mild ↑ AST/ALT
  • ↑ Bilirubin in later stages
• Serology:
  • pANCA positive (~2/3) but non-specific
  • ANA and SMA may be seen
  • AMA → suggests PBC, not PSC
• Imaging:
  • MRCP = investigation of choice: “beaded bile ducts” (alternating strictures & dilatations)
  • USS to exclude gallstones/other causes
  • ERCP if intervention required
• Histology: Liver biopsy may show classic “onion-skin periductal fibrosis”.

📊 Staging

• I: Portal inflammation only
• II: Hepatitis + portal fibrosis
• III: Bridging fibrosis
• IV: Biliary cirrhosis with nodularity

💊 Management

• No curative medical therapy exists.
• Supportive: Nutritional support, fat-soluble vitamin replacement (A, D, E, K).
• Pruritus: Cholestyramine first-line; rifampicin or naltrexone if refractory.
• Endoscopic: Stenting/balloon dilatation for dominant strictures.
• Ursodeoxycholic acid: Improves LFTs, but no proven survival benefit.
• Transplantation: Definitive therapy; 5-yr survival ~80% post-LT. Annual colonoscopy recommended in those with IBD due to ↑ colorectal cancer risk.

⚠️ Complications

• Cholangiocarcinoma (10–15%).
• Colorectal carcinoma risk markedly ↑ in PSC + UC patients.
• Biliary strictures, recurrent cholangitis, cirrhosis, and portal hypertension.

📚 References

• Cholangitis: Diagnosis, Treatment and Prognosis