Primary sclerosing cholangitis Flashcards
What is Primary Sclerosing Cholangitis (PSC) and how does it affect the liver?
PSC is a condition where both the intrahepatic and extrahepatic bile ducts become inflamed and develop strictures, obstructing bile flow from the liver. This leads to liver inflammation (hepatitis), fibrosis, and cirrhosis over time.
What are the potential causes and risk factors of Primary Sclerosing Cholangitis?
The exact cause is unclear, but PSC is thought to result from genetic and environmental factors. Key risk factors include:
* Male gender
* Age 30-40
* Ulcerative colitis (strong association, present in ~70% of cases)
* Family history
* Less commonly associated with Crohn’s disease.
What symptoms and signs may be present in Primary Sclerosing Cholangitis?
Symptoms may include:
* Abdominal pain (right upper quadrant)
* Pruritus (itching)
* Fatigue
* Jaundice
* Hepatomegaly
* Splenomegaly
Many patients may be asymptomatic at diagnosis, and the disease is often first discovered through abnormal liver function tests.
How is Primary Sclerosing Cholangitis diagnosed?
Diagnosis can involve:
* Liver function tests: Elevated alkaline phosphatase (early sign), later raised liver enzymes and bilirubin.
* Autoantibodies: May include p-ANCA, ANA, anti-SMA.
* Magnetic Resonance Cholangiopancreatography (MRCP): Shows bile duct strictures.
* Colonoscopy: To assess for ulcerative colitis.
* Liver biopsy: Rarely needed but may be done if diagnostic uncertainty exists.
What is the management approach for Primary Sclerosing Cholangitis?
Management may include:
* Endoscopic Retrograde Cholangiopancreatography (ERCP): To treat dominant strictures.
* Liver transplant: Considered for advanced disease, with around 80% survival at five years post-transplant.
* Colestyramine: For pruritus caused by bile acid buildup.
* Fat-soluble vitamin replacement: A, D, E, and K.
* Monitoring for complications: Regular screening for cholangiocarcinoma, cirrhosis, and oesophageal varices.
What complications and associated conditions can arise from Primary Sclerosing Cholangitis?
Complications may include:
* Biliary strictures
* Acute bacterial cholangitis
* Cholangiocarcinoma: Develops in 10-20% of cases
* Cirrhosis and associated complications like portal hypertension and oesophageal varices
* Fat-soluble vitamin deficiency (A, D, E, K)
* Osteoporosis
* Colorectal cancer in patients with ulcerative colitis.
How is IgG4-related sclerosing cholangitis different from Primary Sclerosing Cholangitis?
IgG4-related sclerosing cholangitis is distinguished by elevated IgG4 levels in the blood and responds well to steroid treatment. It is also associated with autoimmune pancreatitis.
