PSC Flashcards

1
Q

What is PSC?

A

progressive liver disease with inflammatory fibrosis + obliteration of intrahepatic + extrahepatic bile ducts

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2
Q

Describe the aetiology of PSC

A

UNKNOWN
Possible immune + genetic predisposition with environmental triggers

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3
Q

List 3 conditions associated with PSC

A

UC
Crohns (much less than UC)
HIV

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4
Q

Explain the relationship between PSC and UC

A

80% of PSC pts have UC
4% UC pts have PSC

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5
Q

Describe the epidemiology of PSC

A

M > F (2:1)
25-40y

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6
Q

List 5 S/S of PSC

A

Pruritis
Jaundice
Pale stool, dark urine
RUQ pain
Fatigue

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7
Q

List 3 late signs of PSC

A

Hepatosplenomegaly
Portal hypertension
Liver failure

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8
Q

What should you look for in the PMH if PSC is suspected?

A

UC

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9
Q

Bloods for PSC?

A

FBC
LFTs, Albumin
Lipid profile
Autoantibodies
Immunoglobulins

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10
Q

What is seen on LFTs in PSC?

A

High ALP
High GGT
Norm/ high BR
Mild transaminitis/ norm ALT + AST

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11
Q

Describe the serology found in PSC

A

pANCA (80%)
ANA (75%)
IgG high

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12
Q

Why is imaging performed in PSC?

A

r/o biliary obstruction
confirm dx with MRCP

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13
Q

Which is the confirmatory investigation of choice in PSC?

A

MRCP (if no biliary obstruction)

(ERCP if obstruction)

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14
Q

What is seen on MRCP in PSC?

A

multifocal intrahepatic + extrahepatic bile duct strictures

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15
Q

Why perform liver biopsy in PSC?

A

Not routinely performed in workup
Performed in small duct/ AI hepatitis overlap syndrome

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16
Q

What may be seen on biopsy in PSC?

A

concentric periductal “onion skin” fibrosis

17
Q

Which ducts are affected in PSC?

A

INTRAHEPATIC

EXTRAHEPATIC

18
Q

Describe management of PSC

A

Mainly supportive
No medications prevent progression

19
Q

Describe pharmacological management of PSC

A

Ursodeoxycholic acid

20
Q

What interventional management may be performed in PSC? What are the indications for this?

A

ERCP dilation +/- stenting
To relieve Sx caused by stenosis, e.g., pruritus +/or cholangitis
To exclude cholangiocarcinoma

21
Q

List 4 complications of PSC

A

Steatorrhea + fat soluble vitamin deficiency
Cirrhosis
Bacterial cholangitis
Malignancy

22
Q

Which malignancies may arise in PSC?

A

Cholangiocarcinoma
Gallbladder carcinoma
Colorectal cancer

Screen annually with abdo US/ MRI + colonoscopy

23
Q

What is the curative treatment for PSC?

A

Liver transplant, but high rate recurrence