PBC & PSC (Hepatic Duct Disease) Flashcards
What type of disease is primary biliary cholangitis/cirrhosis (PBC)
Autoimmune (T cells attack cells lining small bile ducts in liver - affects only intra hepatic ducts)
What two diseases is PBC associated with?
- Sjogren’s syndrome
- Autoimmune hepatitis (raised levels of anti-smooth muscle antibodies and ANAs, associated with Graves’ disease)
What are the symptoms of PBC?
- Pruritis
- Jaundice
- Xanthelesmata (cholesterol deposits)
- RUQ pain/discomfort
- Fatigue
- Oedema
Who typically gets PBC?
Middle aged women
How do you diagnose PBC?
Blood tests ± ultrasound
What do blood tests show in PBC?
- Anti-mitochondrial antibodies (AMAs)
- Raised ALP and GGT
What is the treatment for PBC?
Ursodeoxycholic acid (UDCA)
Which disease is primary sclerosing cholangitis (PSC) strongly linked with?
Ulcerative colitis (UC)
What causes PSC?
Cells lining the intra and/or extra hepatic ducts die causing fibrosis and tightening of the bile ducts
What do you see on histology in PSC?
Onion skin fibrosis
What is the only definitive treatment for PSC?
Liver transplant
How is PSC diagnosed?
MRCP/ERCP
What will blood tests show in PSC?
- P-ANCAs ± ANAs, SMAs (negative AMAs)
- Elevated ALP and GGT
What does PSC increase your risk of?
- Cholangiocarcinoma (and other cancers)
- Bacterial cholangitis
What are the symptoms of PSC?
- Jaundice
- RUQ pain
- Light stools and dark urine
- Hepatosplenomegaly
