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 the later stages of Primary Sclerosing Cholangitis (PSC), liver transplantation is often an option. Survival after the operation is good, although PSC can recur in the new liver in some patients.

About

• PSC involves progressive fibrosis, inflammation, and destruction of the intrahepatic and extrahepatic bile ducts.
• Approximately 10 to 15% of PSC patients develop cholangiocarcinoma.
• There is a strong association with ulcerative colitis, with 75% of PSC patients also having this condition.

Aetiology

• PSC is associated with inflammatory bowel disease (IBD) in over 50% of cases, particularly ulcerative colitis.
• PSC can be classified as primary (idiopathic) or secondary to another disease or bile duct injury.

Clinical Features

• Progressive jaundice, often in a patient with known IBD.
• Common symptoms include malaise, pruritus (itching), and weight loss.
• Patients may experience episodes of cholangitis (fever, jaundice, and right upper quadrant pain).

Investigations

• Initially, there is an isolated elevation in Alkaline phosphatase (ALP).
• Mildly elevated AST and ALT are seen, with raised bilirubin appearing later as cholestasis develops.
• Positive antinuclear antibodies (ANA) and anti-smooth muscle antibodies may be found.
• A positive anti-mitochondrial antibody (AMA) suggests Primary Biliary Cirrhosis (PBC), not PSC.
• pANCA (perinuclear anti-neutrophil cytoplasmic antibodies) is positive in about two-thirds of PSC cases, but it is non-specific.
• There may be raised gamma globulins in blood tests.
• Ultrasound (USS) can be used to exclude gallstones and other causes of cholestasis.
• MRCP (Magnetic Resonance Cholangiopancreatography) is the imaging modality of choice and shows bile duct strictures with intervening normal areas, giving a "beaded" appearance.
• ERCP (Endoscopic Retrograde Cholangiopancreatography) may also be used but is invasive.
• Liver biopsy shows onion-skin fibrosis around bile ducts, characteristic of PSC.

Staging

• Stage I: Inflammation confined to portal tracts.
• Stage II: Hepatitis and portal fibrosis.
• Stage III: Bridging fibrosis.
• Stage IV: Biliary cirrhosis with nodules.

Management

• No treatment has been proven to slow progression. Focus is on nutritional support, including fat-soluble vitamin supplementation (A, D, E, K).
• Stenting for bile duct strictures and balloon dilatation may relieve obstructions.
• Immunosuppressive therapies (steroids, azathioprine) have been found ineffective in PSC.
• Pruritus can be managed with cholestyramine to alleviate itching.
• Ursodeoxycholic acid may improve biochemical markers such as bilirubin and ALP but has no effect on overall survival.
• Liver transplantation is the only definitive treatment for advanced PSC, often combined with a choledochojejunostomy to minimize recurrence.
• Patients with a liver transplant require an annual colonoscopy to screen for colonic cancer, especially if they have a history of ulcerative colitis.
• Median survival for PSC patients is approximately 12 years.

Complications

• Cholangiocarcinoma (bile duct cancer) occurs in 10-15% of PSC patients.
• Increased risk of colonic carcinoma in patients with PSC and ulcerative colitis.

References

• Cholangitis: Diagnosis, Treatment and Prognosis