Primary sclerosing cholangitis Flashcards

1
Q

Define primary sclerosing cholangitis.

A

Chronic cholestatic liver disease characterised by progressive inflammatory fibrosis and obliteration of intrahepatic and extrahepatic bile ducts.

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2
Q

What is the aetiology of PSC?

A

Unknown

It has auto-antibodies BUT does not behave like autoimmune disease as it is male predominant (2:1) and does not respond to immunosuppressive treatment

Link with IBD could be related to translocation of lymphocytes in colon via enterohepatic circulation

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3
Q

What is the pathophysiology of the bile ducts in PSC?

A
  • Inflammation of medium and large bile ducts
  • periductal fibrosis and strictures →
  • eventually obliteration or smaller ducts and cholestasis
  • This predisposes to jaundice, pruritus and episodes of bacterial cholangitis and progression to biliary cirrhosis
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4
Q

How common is PSC and when does it present?

A

2-7/100,000

Usually presents between age 25-40yrs

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5
Q

What are the symptoms of PSC?

A

Clinical features range from asymptomatic (50%) to end-stage liver disease.

  • Fatigue
  • Abdominal pain RUQ/epigastrium
  • Pruritus - intermittent generalised
  • Liver disease - jaundice, steatorrhoea, haematemesis, encephalopathy, ascites
  • IBD - UC or Crohn’s in 80%
  • Weight loss - from fat malabsorption
  • Episodes of fever and rigors - caused by acute cholangitis, but less common
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6
Q

What are the risk factors for PSC? How common is IBD in PSC?

A
  • Male (2:1)
  • Age 40-50yrs
  • IBD - 80% of patients with PSC have IBD, but 10% of IBD patients have PSC
  • FH
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7
Q

What are the signs of PSC on examination?

A

May have no signs

In more advanced disease:

  • jaundice
  • hepatosplenomegaly
  • spider naevi
  • palmar erythema
  • ascites
  • encephalopathy
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8
Q

What investigations would you do in PSC?

A

Bloods:

  • LFT - cholestatic pattern

No auto-antibodies specific to PSC but may have ANA, ANCA, anti-SMA, RF.

Later: thrombocytopenia and clotting abnormalities

Other tests of exclusion: immunoglobulin levels, urine copper, ceruloplasmin

Imaging

  • USS - good initial test but not best for looking at biliary tree
  • Abdo CT - thickening of ducts, saccular intrahepatic duct dilation, lymphadenopathy.
  • MRCP - enables non-invasive imaging of biliary tree. ERCP is done if MRCP is not diagnostic.

Invasive

  • Colonoscopy
  • Liver biopsy - supports diagnosis but rarely diagnostic. Shows fibro-obliterative cholangitis, periductal fibrosis and inflammation.

NB: high risk of cholangiocarcinoma.

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9
Q

What are the complications of PSC?

A
  • cholangitis
  • biliary cirrhosis
  • cholangiocarcinoma
  • portal hypertension
  • metabolic bone disease
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10
Q

How do you manage PSC?

A

Early disease - observation + pruritus relief (e.g. colestyramine)

Vitamin D + calcium, bisphosphonates + HRT - protect against hepatic osteodystrophy

Liver transplantation - but may get graft recurrence in 10-20%

May have overlap with other autoimmune conditions for which you would immunosuppress but this for PSC alone does not work.

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11
Q

What should you monitor for in PSC?

A
  • Progression of cirrhosis with LFTs
  • Bone mineral density - hepatic osteodystrophy
  • Fat- soluble vitamin testing
  • CT/MRI for HCC every 6 months
  • IBD colonoscopy for colorectal cancer
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12
Q

What is the prognosis with PSC?

A

Results in progressive hepatic fibrosis, and ultimately cirrhosis and end-stage liver disease for most patients.

Reduced life expectancy - death from cholangiocarcinoma especially.

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