Primary Sclerosing Cholangitis Flashcards
What is primary sclerosing cholangitis?
It is is defined as a chronic condition in which the intrahepatic or extrahepatic ducts become strictured and fibrotic
What is the pathophysiological consequence of primary sclerosing cholangitis?
This leads to progressive obstruction of bile outflow into the intestines, referred to as cholestasis
This back-pressure of bile obstruction leads to hepatitis, fibrosis, cirrhosis and liver failure
What is the underlying aetiology of primary sclerosing cholangitis?
Unknown
However, there is likely to be a combination of genetic, autoimmune, intestinal microbiome and environmental factors involved
What are the four risk factors of primary sclerosing cholangitis?
Male Gender
Middle Aged
Family History
Ulcerative Colitis
What are the four clinical features associated with primary sclerosing cholangitis?
Pruritis
Jaundice
Right Upper Quadrant Pain
Hepatosplenomegaly
What are the three investigations used to diagnose primary sclerosing cholangitis?
Blood Tests
Magnetic Resonance Cholangiopancreatography (MRCP)
Endoscopic Retrograde Cholangiopancreatography (ERCP)
What seven blood test results indicate primary sclerosing cholangitis?
Increased ALP Levels
Increased ALT Levels
Increased AST Levels
Increased Bilirubin Levels
Antineutrophil Cytoplasmin Antibody (p-ANCA)
Antinuclear Antibodies (ANA)
Anticardiolipin Antibodies (aCL)
How are autoantibodies used to diagnose primary sclerosing cholangitis?
They are are not specific to primary sclerosing cholangitis and are therefore not helpful in diagnosis
However, they can indicate an autoimmune element to the disease that may respond to immunosuppression
What is the gold standard investigation used to diagnose primary sclerosing cholangitis?
MRCP
What is MRCP?
An MRI scan of the liver, bile ducts and pancreas
What are the two features of primary sclerosing cholangitis on MRCP?
Bile duct lesions
Bile duct strictures
What is ERCP?
It involves the insertion of an endoscope through the oesophagus, stomach and into the duodenum
In the duodenum, the endoscope is then guided through the sphincter of Oddi into the bile duct – where contrast is injected, and x-rays are then taken
What are the two features of primary sclerosing cholangitis on ERCP?
Bile duct lesions
Bile duct strictures
What are the three pharmacological management options of primary sclerosing cholangitis?
Ursodeoxycholic Acid
Cholestyramine
Fat Soluble Vitamin Supplementation
What is the mechanism of action of ursodeoxycholic acid?
It reduces the intestinal absorption of cholesterol
How is ursodeoxycholic acid used to manage primary sclerosing cholangitis?
It slows disease progression and provides symptomatic relief
What is the mechanism of action of cholestyramine?
It is a bile acid sequestrate, which binds to bile acids to prevent absorption in the gut
It therefore increases bile acid levels in the gut
How is cholestyramine used to manage primary sclerosing cholangitis?
It relieves pruritis features
What are the two surgical management options are used in primary sclerosing cholangitis?
Endoscopic Retrograde Cholangiopancreatography (ERCP)
Liver Transplant
How are ERCPs used to manage primary sclerosing cholangitis?
They can be used to dilate and stent the bile ducts
This improves bile duct flow and provides symptomatic relief
What is the only definitive management option of primary sclerosing cholangitis?
Liver transplant
What are the six complications of primary sclerosing cholangitis?
Liver Cirrhosis
Liver Failure
Portal Hypertension
Acute Bacterial Cholangitis
Cholangiocarcinoma
Colorectal Cancer
What complication is not associated with primary sclerosing cholangitis - unlike primary biliary cholangitis?
Hepatocellular carcinoma
