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