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Year Two - Gastroenterology System > Primary Biliary Cholangitis > Flashcards

Primary Biliary Cholangitis Flashcards

1
Q

What is another term for primary biliary cholangitis?

A

Primary biliary cirrhosis

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2
Q

What is primary biliary cholangitis?

A

It is defined as a chronic condition in which the immune system targets the interlobular bile ducts within the liver

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3
Q

What is another term for the interlobular bile ducts?

A

Canals of Herring

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4
Q

What is the pathophysiological consequence of primary biliary cholangitis?

A

This leads to progressive obstruction of bile outflow, referred to as cholestasis

This back-pressure of bile obstruction leads to fibrosis, cirrhosis and liver failure

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5
Q

What are the four risk factors of primary biliary cholangitis?

A

Female Gender

Middle Aged

Rheumatoid Conditions

Autoimmune Conditions

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6
Q

What three rheumatoid conditions are associated with primary biliary cholangitis?

A

Sjorgen’s syndrome

Systemic sclerosis

Rheumatoid arthritis

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7
Q

What two autoimmune conditions are associated with primary biliary cholangitis?

A

Thyroid disease

Coeliac disease

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8
Q

What are the seven clinical features associated with primary biliary cholangitis?

A

Pruritis

Jaundice

Xanthoma

Xanthelasmas

Right Upper Quadrant Pain

Steatorrhoea

Hepatosplenomegaly

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9
Q

How is pruritus a clinical feature of primary biliary cholangitis?

A

The cholestasis results in an increased level of bile acids in the blood

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10
Q

How is jaundice a clinical feature of primary biliary cholangitis?

A

The cholestasis results in an increased level of bilirubin in the blood

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11
Q

What is xanthoma?

A

It is defined as the nodular deposition of cholesterol in the skin/tendons

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12
Q

What is xanthelasma?

A

It is defined as a subtype of xanthoma, in which there is the deposition of cholesterol in the skin - usually around the eyelids

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13
Q

How are xanthoma and xanthelasma clinical features of primary biliary cholangitis?

A

The cholestasis results in an increased level of cholesterol in the blood

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14
Q

How is steatorrhoea a clinical features of primary biliary cholangitis?

A

The cholestasis results in a lack of bile acids in the gastrointestinal tract, which aid the digestion of fats

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15
Q

What three investigations are used to diagnose primary biliary cholangitis?

A

Blood Tests

Ultrasound Scan

Liver Biopsy

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16
Q

What six blood test results indicate primary biliary cholangitis?

A

Increased ALP Levels

Increased Bilirubin Levels

Increased Serum IgM Levels

Increased ESR Levels

Anti-Mitochondrial Antibodies (AMA) M2 Subtype

Smooth Muscle Antibodies (SMA)

17
Q

Which antibody is most commonly associated with primary biliary cholangitis?

A

Anti-Mitochondrial Antibodies (AMA) M2 Subtype

18
Q

How is an ultrasound scan used to investigate primary biliary cholangitis?

A

It is used to exclude an extrahepatic biliary obstruction related cause

19
Q

How is a liver biopsy used to investigate primary biliary cholangitis?

A

It is used to confirm a diagnosis of primary biliary cholangitis and to stage the disease

20
Q

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

A

Ursodeoxycholic Acid

Cholestyramine

Fat Soluble Vitamin Supplementation

21
Q

What is the first line pharmacological management option of primary biliary cholangitis?

A

Ursodeoxycholic Acid

22
Q

What is the mechanism of action of ursodeoxycholic acid?

A

It reduces the intestinal absorption of cholesterol

23
Q

How is ursodeoxycholic acid used to manage primary biliary cholangitis?

A

It slows disease progression and provides symptomatic relief

24
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

25
Q

How is cholestyramine used to manage primary biliary cholangitis?

A

It relieves pruritis features

26
Q

What surgical management option is used in primary biliary cholangitis?

A

Liver transplant

27
Q

When are liver transplants used to manage primary biliary cholangitis?

A

When the complication development of end stage liver disease occurs

OR

When bilirubin levels > 100

28
Q

What are the seven complications of primary biliary cholangitis?

A

Liver Cirrhosis

Portal Hypertension

Hepatocellular Carcinoma

Osteoporosis

Osteomalacia

Distal Renal Tubular Acidosis

Hypothyroidism

29
Q

What rule can be used to remember the key features of primary biliary cholangitis?

A

M rule

igM

anti-Mitochondria antibodies, M2 subtype

Middle aged females

Year Two - Gastroenterology System (33 decks)

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