Primary Sclerosing Cholangitis Flashcards

1
Q

What is primary sclerosing cholangitis?

A

a condition where the intrahepatic and extrahepatic bile ducts become inflamed and damaged, developing strictures that obstruct the flow of bile out of the liver and into the intestines

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2
Q

What are the consequences of chronic bile obstruction? (3)

A

Hepatitis
Fibrosis
Cirrhosis

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3
Q

What is primary sclerosing cholangitis strongly linked with?

A

UC

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4
Q

What are the key risk factors of primary sclerosing cholangitis? (4)

A

• Male
• Aged 30-40
• Ulcerative colitis
• Family history

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5
Q

What are some features of primary sclerosing cholangitis? (6)

A

• Abdominal pain in the right upper quadrant
• Pruritus (itching)
• Fatigue
• Jaundice
• Hepatomegaly
• Splenomegaly

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6
Q

What is seen on LFTs in primary sclerosing cholangitis?

A

Raised ALP
Bilirubin raised later in disease

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7
Q

What is the diagnostic imaging in primary sclerosing cholangitis?

A

MRCP
Shows bile strictures

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8
Q

Why would colonoscopy be performed in primary sclerosing cholangitis?

A

To assess for UC

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9
Q

What can be used to treat strictures in primary sclerosing cholangitis?

A

ERCP
Structures are dilated
Stents can be put in
Antibiotics are given at same time to reduce risk of infection from ERCP

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10
Q

How is an ERCP performed?

A

This involves inserting an endoscope down the oesophagus, past the stomach, to the duodenum and the opening of the common bile duct (the sphincter of Oddi). This gives the operator access to the biliary system

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11
Q

What is the management of advanced primary sclerosing cholangitis?

A

Liver transplant

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12
Q

What is used to manage symptoms of Pruritis in primary sclerosing cholangitis?

A

Colestyramine

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13
Q

What does colestyramine do?

A

a bile acid sequestrant that reduces intestinal absorption of bile acids

Used to manage Pruritis

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14
Q

What are some complications of primary sclerosing cholangitis? (7)

A

• Biliary strictures
• Acute bacterial cholangitis
• Cholangiocarcinoma develops in 10-20% of cases
• Cirrhosis and the related complications (e.g., portal hypertension and oesophageal varices)
• Fat-soluble vitamin deficiency (A, D, E and K)
• Osteoporosis
• Colorectal cancer in patients with ulcerative colitis

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15
Q

What cancer is linked to primary sclerosing cholangitis?

A

Cholangiocarcinoma

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16
Q

What is IgG4-related sclerosing cholangitis?

A

IgG4-related sclerosing cholangitis is similar to primary sclerosing cholangitis. Elevated IgG4 levels in the blood are the distinguishing feature.

17
Q

What is the treatment of IgG4-related sclerosing cholangitis?

A

Steroids

18
Q

What is IgG4-related sclerosing cholangitis associated with?

A

Autoimmune pancreatitis