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cc: gastro > Primary sclerosing cholangitis > Flashcards

Primary sclerosing cholangitis Flashcards

1
Q

What is the end point of primary sclerosing cholangitis (PSC)?

A

End-stage liver disease

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2
Q

What characterises PSC?

A

Progressive inflammation, fibrosis, and stricturing of the intrahepatic and extrahepatic bile ducts

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3
Q

How is the diagnosis of PSC best made?

A

Contrast cholangiography

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4
Q

What does PSC look like on contrast cholangiography?

A

Diffuse multifocal strictures of the bile ducts

Focal dilation of the bile ducts as well, leading to a ‘beaded’ appearance

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5
Q

T/F PSC is more common in males

A

True

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6
Q

T/F There is an increase in incidence of PSC in first degree relatives

A

True, but still low

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7
Q

Is there a link between ulcerative colitis and PSC?

A

Yes

UC has been reported in anywhere between 25 and 90% of PSC patients

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8
Q

Is there an association between PSC and any other GIT disorders?

A

Yes

Crohn’s disease (around 15%)

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9
Q

How does the epidemiology of PSC, specifically gender distribution, change when considering those that have UC?

A

Rather than being male dominant, PSC becomes equally distributed between the genders

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10
Q

What are some possibly aetiologies of PSC?

A

The link between PSC and UC suggests an autoimmune process

Could be an inflammatory reaction in the liver and bile ducts from chronic or recurrent entry of bacteria in the portal circulation

Ischaemic damage to the bile ducts may occur

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11
Q

Describe the chronological relationship between PSC and UC onset

A

PSC may develop years after colectomy for UC

UC may first present after liver transplantation has been performed for PSC

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12
Q

What is the most likely aetiology for PSC?

A

That it is a multifocal disorder, or that PSC represents multiple diseases with a similar clinical presentation

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13
Q

Is there also a relationship between PSC and CF?

A

Yes

similar radiologic and histologic pictures

Evidence of increased levels of CFTR

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14
Q

What is the natural history of PSC?

A

Progressive disorder

Leads to complications of cholestasis and hepatic failure

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15
Q

What is the median survival without liver transplantation?

A

10-12 years

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16
Q

What is a poor prognostic factor at time of diagnosis?

A

If they are already symptomatic at diagnosis, this is a very poor prognostic marker

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17
Q

What percentage of patients with UC have PSC?

A

~5%

Hence you should not screen UC patients for PSC unless LFTs deranged

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18
Q

What are some findings on physical exam in PSC?

A

Jaundice

Hepatomegaly

Splenomegaly

Excoriations

Normally exam is normal though

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19
Q

What do LFTs normally show with PSC?

A

Cholestatic patern

Elevation of serum alkaline phosphatase predominating

20
Q

What are the normal radiographic findings in PSC?

A

Bile ducts with wall thickening, dilations, and strictures

‘string of beads’ appearance

21
Q

If a PSC patient is symptomatic at the time of diagnosis, what symptom will that most commonly be?

A

Fatigue or pruritus

22
Q

How bad will pruritus be in PSC patients?

A

Can be extremely bad and debilitating

23
Q

What is the normal treatment for intractable pruritus in PSC?

A

Indication for liver transplantation

24
Q

What are bilirubin levels like in PSC patients that have pruritus?

A

Normal

25
Q

What are some autoantibodies that you can test for in PSC?

A

anticardiolipin (most important)

antinuclear

Antismooth muscle

thyroperoxidase

rheumatoid factor

26
Q

How important is autoantibody testing in PSC?

A

Not that important or useful

Mayo risk score for PSC does include anticardiolipin autoantibodies though

27
Q

What is the typical description of the stricutres of PSC?

A

Short

multifocal

Annular

Alternate with normal or mildly dilated segments

28
Q

What would long strictures in the bile ducts be concerning for?

A

Cholangiocarcinoma

29
Q

Why should you test IgG4 levels in PSC?

A

IgG4-associated disease may have a more rapidly progressive course

Appears to be less responsive to corticosteroids

30
Q

Why should you test for antimtochondrial antibodies in PSC?

A

To help exclude primary biliary cirrhosis

31
Q

How is the diagnosis of PSC typically established?

A

By the demonstration of characteristic stricturing patterns on cholangiography

32
Q

What are the three methods by which cholangiography can be obtained?

A

MRCP

ERCP

Percutaneous transhepatic cholangiography

33
Q

Which of the three cholangiography methods for the diagnosis of PSC is the best?

A

MRCP

Less invasive, with comparable diagnostic ability to ERCP

34
Q

T/F The strictures of PSC occur anywhere along the extrahepatic biliary tree only

A

F

They occur anywhere in the biliary tree, whether intra- or extra

35
Q

Is it more common to have only extra-hepatic bile duct involvement in PSC?

A

No

It’s far more common (87%) to have involvement of the entire biliary tree

36
Q

What are the most important DDxs for PSC?

A

Secondary causes of sclerosing cholangitis

IgG4-associated cholangitis/autoimmune pancreatitis

PSC-autoimmune hepatitis overlap syndrome

37
Q

What are some secondary causes of sclerosing cholangitis?

A

Chronic bacterial cholangitis

Infection or ischaemic cholangitis

Cholangiocarcinoma

Choledocholithiasis

Eosinophilic cholangitis

Portal hypertensive biliopathy

Recurrent pancreatitis

Surgical biliary trauma

38
Q

How often does IgG4 associated cholangitis occur without autoimmune pancreatitis?

A

Rarely

Usually happen at the same time

39
Q

What agent can you add to your treatment of PSC, if they have IgG4-associated cholangitis?

A

Glucocorticoids

40
Q

What are some important complications of PSC?

A

End-stage liver disease

Vit ADEK deficiency

Metabolic bone disease

Cholangiocarcinoma

Hepatocellular carcinoma in those with cirrhosis

Colorectal carcinoma in those patients with UC

41
Q

Talk about bone disease in PSC

A

Due to osteoporosis, not osteomalacia, so vit D levels, though normally low, are not causative and replenishment does not prevent progression

Concomitant bowel disease, common in PSC, does contribute normally

Glucocorticoids also increases the rate of bone loss

Has been proposed that a toxin or toxins retained because of cholestasis prevents osteoblast function

42
Q

What are the goals in management of PSC?

A

Retardation and reversal of the disease process

Management of progressive disease and its complications

43
Q

What immunosuppressive agents have proved successful in PSC?

A

None

Current trials looking at ursodeoxycholic acid

44
Q

How does ursodeoxycholic acid work?

A

Protection of cholangiocytes against cytotoxic hydrophobic bile acids

Stimulation of hepatobiliary secretion

Protection of hepatocytes against bile acid-induced apoptosis

Induction of antioxidants

45
Q

What does ursodeoxycholic acid do for PSC patients, and what does it not do?

A

Does improve liver biochemical markers

Does not result in a survival benefit or a delay in the need for liver transplant

46
Q

What is the only current definitive treatment for PSC?

A

Liver transplantation

cc: gastro (6 decks)

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