PBC + PSC Flashcards
What is primary biliary cholangitis?
Organ specific autoimmune disease
What is the pathogenesis of PBC?
Granulomatous inflammation involving bile ducts (body attacks bile ducts)
Loss of intrahepatic bile ducts
Progression to cirrhosis
What is the presentation of PBC?
Pruritus
Fatigue
How is PBC diagnosed?
Anti-mitochondrial auto-antibodies in serum (positive)
IgM elevated
Raised serum alkaline phosphatase
Ultrasound: excludes extrahepatic cholestasis
What are the treatment options for PBC?
UDCA = treatment of choice
Liver transplant
What are the complications of PBC?
Liver cirrhosis
Who does PBC more commonly affect: males or females?
Mainly affects females (9:1)
What is PSC?
Progressive cholestasis with bile duct inflammation and stricture formation
What is pathogenesis underlying PSC?
Chronic inflammation and fibrous obliteration of bile ducts
Loss of intrahepatic bile ducts (intra and extrahepatic ducts involved)
Progression to cirrhosis
What is the presentation of PSC?
Associated with inflammatory bowel disease (usually UC)
Pruritus ± fatigue
If advanced, ascending cholangitis, cirrhosis and hepatic failure
Signs: recurrent cholangitis, jaundice
How is PSC diagnosed?
MRCP: test of choice
pANCA positive
What are the treatment options for PSC?
Liver transplant (mainstay for end-stage disease) Biliary stents
What are the complications of PSC?
Cirrhosis
Increased risk of development of cholangio-carcinoma
Cancers:
- Bile duct, gallbladder, liver and colon cancers are more common so do yearly colonoscopy + ultrasound
- Consider cholecystectomy for gallbladder polyps
- If patients has PSC and IBD (usually UC), this combination is associated with increased increased risk of colorectal malignancy
Is PSC more common in males or females?
Male predominant
