Gastro - Primary Sclerosing Cholangitis Flashcards
What is primary sclerosing cholangitis?
Condition where intrahepatic and extrahepatic bile ducts become inflamed and damaged
Strictures develop obstructing bile flow out of the liver and into the intestines
What does chronic obstruction eventually lead to?
Hepatitis, fibrosis and cirrhosis
What is the cause of primary sclerosing cholangitis?
Genetic and environmental factors
Strong association with ulcerative colitis around 70% of cases occur alongside pre-existing UC
Less commonly associated with Crohn’s
What are the key risk factors for primary sclerosing cholangitis?
Male
30-40
Ulcerative colitis
Family history
How does primary sclerosing cholangitis present?
Patients often asymptomatic and picked up on LFTs, may present with:
- RUQ pain
- Pruritis
- Fatigue
- Jaundice
- Hepatomegaly
- Splenomegaly
What investigations are used for primary sclerosing cholangitis and what do they show?
Liver function tests
- Raised ALP
MRCP
- MRI that gives detailed view of bile ducts, showing bile duct strictures
Colonoscopy
- Assess for ulcerative colitis
Liver biopsy
- Not required, can be used if diagnostic uncertainty
What are the main autoantibodies in primary sclerosing cholangitis?
Perinuclear antineutrophil cytoplasmic antibody (p-ANCA)
Antinuclear antibodies (ANA)
Anti-smooth muscle antibodies (anti-SMA)
What are autoantibodies used for in primary sclerosing cholangitis?
Not helpful in diagnosis or assessment
No antibodies particularly sensitive or specific to primary sclerosing cholangitis
How is primary sclerosing cholangitis managed surgically?
No treatments are proven to be effective
ERCP
- Can be used to treat dominant strictures
- Stents are inserted to keep ducts open
- Antibiotics given alongside to reduce risk of infection
Liver transplant
- Advanced disease, 80% 5-year survival
What are some other aspects of management of primary sclerosing cholangitis?
Colestyramine
- Pruritis symptoms
Fat-soluble vitamin replacement
Monitoring for complications
- Cholangiocarcinoma
- Cirrhosis
- Oesophageal varices
What are some complications of primary sclerosing cholangitis?
Biliary strictures
Acute bacterial cholangitis
Cholangiocarcinoma
Cirrhosis
Fat-soluble vitamin deficiency
Osteoporosis
Colorectal cancer in patients with UC
What is IgG4- related sclerosing cholangitis?
Similar to primary sclerosing cholangitis
Elevated IgG4 levels in the blood
Responds well to treatment with steroids, associated with autoimmune pancreatitis
