Primary Biliary Cholangitis Flashcards

1
Q

define primary biliary cholangitis?

A

chronic disease of small intrahepatic bile ducts-> characterised by progressive bile duct damage ( and eventual loss) occurring in context of chronic portal tract inflammation

fibrosis develops as consequence of original insulit

secondary effects of bile acids retained in liver=> CIRRHOSIS

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2
Q

outline the risk factors for primary biliary cholangitis?

A

female sex
age between 45-60 years
family history of PBC/autoimmune disease
smoking

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3
Q

outline the aetiology of PBC?

A

unknown

likely to be autoimmune
characterised by chronic autoimmune granulomatous inflammation-> leads to fibrosis, cirrhosis and portal, hypertension

genetic and environmental factors involved

An environmental trigger may cause bile duct epithelial injury, which then leads to a T-cell mediated autoimmune response directed against bile duct epithelial cells

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4
Q

summarise the epidemiology of PBC?

A

mainly affects middle aged women ( 9:1 female: male)

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5
Q

what are the presenting symptoms of PBC?

A

Fatigue

weight loss

pruritis

family history of autoimmune disease

history of hypercholestralaemia - cholestasis

May present with a complication of liver decompensation (e.g. jaundice, ascites, variceal haemorrhage)

May present with symptoms of associated conditions (e.g. Sjogren’s syndrome, arthritis, Raynaud’s phenomenon)

postural dizziness/ blackouts

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6
Q

what are the signs of PBC on physical examination

A
  • Jaundice
  • Skin pigmentation
  • Scratch marks
  • Xanthomas (secondary to hypercholesterolaemia which is a feature of cholestasis)
  • Hepatosplenomegaly
  • Ascites
  • Signs of chronic liver disease
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7
Q

what are the appropriate investigations for PBC?

A

bloods

ultrasound

liver biopsy

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8
Q

describe bloods in PBC?

A

LFT

  • High ALP and GGT
  • bilirubin may be high or normal
  • ALT and AST are normal initially-> but will increase as disease progresses and cirrhosis develops
  • low albumin
  • high prothrombin time

clotting
- prolongation of pT

typical features of PBC

  • antimitochondrial antibodies ( typical feature of PBC) - AMAs are hallmarks of PBC
  • high IgM
  • high cholesterol
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9
Q

summarise typical presentation of PBC?

A

lethargy and pruritus female with minor increase in AST/gamma-GT and increase of alkaline phosphatase; hypercholesterolaemia, can be associated with Sjogren’s, Rheumatoid arthritis etc

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10
Q

what are the typical features of PBC?

A

▪ Antimitochondrial antibodies (typical feature of PBC) – AMAs are the hallmarks of PBC!!

▪ High IgM

High Cholesterol

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11
Q

outline the treatment for PBC?

A

Once diagnosis made treatment is important to prevent progression, with ursodeoxycholic acid (this is just exogenous bile salts).

Cholestyramine-> alleviate pruritus but must be given at least 2 hours apart from ursodeoxycholic acid.

patient may ultimately need transplantation

SIDE NOTE: Statins are not generally used in PBC.
Ursodeoxycholic acid lowers lipids and statins can obviously affect liver function therefore they do not form part of the initial treament.

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12
Q

what is used instead of statins in PBC?

A

ursodeoxycholic acid-> lowers lipids

statins can affect liver function-> don’t form part of initial treatment

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13
Q

why is an ultrasound a useful investigation for PBC

A

exclude extrinsicallyahepatic biliary obstruction ( gallstones)

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14
Q

What do we look for on a liver biopsy in PBC?

A

show chronic inflammatory cells and granulomas around the intrahepatic bile ducts, destruction of bile ducts, fibrosis and regenerating nodules of hepatocytes

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