Primary Sclerosing Cholangitis Flashcards

1
Q

What is primary sclerosing cholangitis?

A

It is is defined as a chronic condition in which the intrahepatic or extrahepatic ducts become strictured and fibrotic

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2
Q

What is the pathophysiological consequence of primary sclerosing cholangitis?

A

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

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3
Q

What is the underlying aetiology of primary sclerosing cholangitis?

A

Unknown

However, there is likely to be a combination of genetic, autoimmune, intestinal microbiome and environmental factors involved

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4
Q

What are the four risk factors of primary sclerosing cholangitis?

A

Male Gender

Middle Aged

Family History

Ulcerative Colitis

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5
Q

What are the four clinical features associated with primary sclerosing cholangitis?

A

Pruritis

Jaundice

Right Upper Quadrant Pain

Hepatosplenomegaly

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6
Q

What are the three investigations used to diagnose primary sclerosing cholangitis?

A

Blood Tests

Magnetic Resonance Cholangiopancreatography (MRCP)

Endoscopic Retrograde Cholangiopancreatography (ERCP)

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7
Q

What seven blood test results indicate primary sclerosing cholangitis?

A

Increased ALP Levels

Increased ALT Levels

Increased AST Levels

Increased Bilirubin Levels

Antineutrophil Cytoplasmin Antibody (p-ANCA)

Antinuclear Antibodies (ANA)

Anticardiolipin Antibodies (aCL)

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8
Q

How are autoantibodies used to diagnose primary sclerosing cholangitis?

A

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

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9
Q

What is the gold standard investigation used to diagnose primary sclerosing cholangitis?

A

MRCP

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10
Q

What is MRCP?

A

An MRI scan of the liver, bile ducts and pancreas

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11
Q

What are the two features of primary sclerosing cholangitis on MRCP?

A

Bile duct lesions

Bile duct strictures

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12
Q

What is ERCP?

A

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

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13
Q

What are the two features of primary sclerosing cholangitis on ERCP?

A

Bile duct lesions

Bile duct strictures

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14
Q

What are the three pharmacological management options of primary sclerosing cholangitis?

A

Ursodeoxycholic Acid

Cholestyramine

Fat Soluble Vitamin Supplementation

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15
Q

What is the mechanism of action of ursodeoxycholic acid?

A

It reduces the intestinal absorption of cholesterol

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16
Q

How is ursodeoxycholic acid used to manage primary sclerosing cholangitis?

A

It slows disease progression and provides symptomatic relief

17
Q

What is the mechanism of action of cholestyramine?

A

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

18
Q

How is cholestyramine used to manage primary sclerosing cholangitis?

A

It relieves pruritis features

19
Q

What are the two surgical management options are used in primary sclerosing cholangitis?

A

Endoscopic Retrograde Cholangiopancreatography (ERCP)

Liver Transplant

20
Q

How are ERCPs used to manage primary sclerosing cholangitis?

A

They can be used to dilate and stent the bile ducts

This improves bile duct flow and provides symptomatic relief

21
Q

What is the only definitive management option of primary sclerosing cholangitis?

A

Liver transplant

22
Q

What are the six complications of primary sclerosing cholangitis?

A

Liver Cirrhosis

Liver Failure

Portal Hypertension

Acute Bacterial Cholangitis

Cholangiocarcinoma

Colorectal Cancer

23
Q

What complication is not associated with primary sclerosing cholangitis - unlike primary biliary cholangitis?

A

Hepatocellular carcinoma