Primary Sclerosing Cholangitis Flashcards

1
Q

What is primary sclerosing cholangitis?

A

Intrahepatic and extrahepatic ducts become fibrotic, stiff and hardened causing chronic bile obstruction

I.e. sclerosing= stiffening and hardening
cholangitis= inflammation of bile ducts

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2
Q

What condition is associated with the development of PSC?

A

Ulcerative colitis i.e. 70% of cases occurs in conjunctive with UC

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3
Q

How would someone with PSC present?

A
Jaundice 
Fatigue 
Pruritus-> scratch marks 
Chronic RUQ pain 
Fatigue 
Signs of liver cirrhosis (in late stages)-> ascites + signs of portal HTN 
Hepatomegaly 
More likely to be male
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4
Q

What are the LFTs likely to show in PSC?

A

Cholestatic picture

  • ++ ALP= first to present
    • Bilirubin= as strictures become more severe
  • ALT/AST may become more derranged as PSC progresses and causes hepatitis and liver cirrhosis
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5
Q

How is PSC diagnosed?

A

(MRCP) Magnetic resonance cholangiopancreatography

-bile duct lesions or strictures present

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6
Q

How can PSC be managed?

A

ERCP dilate and stent ducts i.e. endoscope through sphincter of Oddi to enter ampulla of Vater to insert stent into bile ducts

UDCA-> decreased cholesterol

Colestyramine to sequestrate bile acids to help with pruritus

Monitor for complications of cirrhosis and oesophageal varices

Treat IBD-> aim for remission as IBD disease state drives PSC disease state

Liver transplant is CURATIVE
-need to assess early because patients can go downhill very quickly

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7
Q

What are the possible extra-hepatic manifestations of PSC and why do they occur?

A

Pyoderma gangrenosum

Erythema nodosum

Episcleritis

Due to being associated with active bowel disease eg IBD

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8
Q

What is the use of testing for autoantibodies in PSC?

A

Not helpful in diagnosis but can indicate if there is autoimmune element to PSC which might respond to immunosuppression

Eg:

  • p-ANCA
  • ANA
  • aCL
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9
Q

Why might someone with PSC be prone to current bouts of cholangitis/acute bacterial cholangitis?

A

Strictures in bile ducts lead to stagnant bile which increases the risk of bile infection and the risk of cholangitis

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10
Q

What are the associated complications which can occur with PSC?

A

Acute bacterial cholangitis

Cholangiocarcinoma

Colorectal cancer

Cirrhosis + LF

Biliary strictures

Fat soluble vitamin D deficiencies

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