Primary Sclerosing Cholangitis Flashcards
1
Q
What is Primary Sclerosing Cholangitis?
A
- Progressive cholestasis with bile duct inflammation and strictures
2
Q
What will chronic bile obstruction finally lead to?
A
- hepatitis > fibrosis > cirrhosis
3
Q
What are the causes of PSC?
A
- High association c UC
4
Q
What are the RF for PSC?
A
- Male
- Aged 30-40
- Ulcerative Colitis
- Family History
5
Q
What genes are associated with PSC?
A
- HLA-A1
- B8
- DR3
6
Q
What is the presentation of PSC?
A
- Jaundice
- Chronic right upper quadrant pain
- Pruritus
- Fatigue
- Hepatomegaly
7
Q
What antibodies are associated c PSC?
A
- Anti Neutrophil Cytoplasmic Antibody (ANCA)
- Antinuclear Antibodies (ANA)
- Anticardiolipin antibodies (aCL)
8
Q
What test would you order for PSC?
A
- LFT
- High ALP first
- then high bilirubin
- gamma GT
- IgM high
- ANA, ANCA, SMA high
- MRCP (definitive)
- Liver biopsy
- ERCP
- ‘beaded’ strictures
9
Q
What would liver biopsy show for PSC?
A
- Fibrous
- Obliterative cholangitis
10
Q
What are the cx of PSC?
A
- Acute bacterial cholangitis
- Cholangiocarcinoma develops in 10-20% of cases
- Colorectal cancer
- Cirrhosis and liver failure
- Biliary strictures
- Fat soluble vitamin deficiencies
11
Q
How would you Mx PSC?
A
- Liver transplant -80% survival a 5 years
- ERCP - dilate and stent strictures
- Ursodeoxycholic acid
- Colestryamine - bile acid sequestrate
- Monitor cx
