Autoimmune Hepatitis/Cholangiopathies

Question 1

What are the autoimmune cholangiopathies?

Answer 1

• primary biliary cirrhosis
• primary sclerosing cholangitis

Question 2

What are characteristic features of primary biliary cirrhosis?

(epidemiology, associated conditions, affected ducts, serology/radiology, histologic appearance)

Answer 2

• female predominant (90%)
• frequently with other autoimmune conditions
• anti-mitochondrial Ab/AMA (90%)
• florid duct lesion
• small-medium intrahepatic ducts

Question 3

What are characteristic features of primary sclerosing cholangitis?

(epidemiology, associated conditions, affected ducts, serology/radiology, histologic appearance)

Answer 3

• male predominant (70%)
• frequently with IBD, particularly UC
• segemental fibrosis w/ intervening dilation on MRCP/ERCP -> “beads on a string”
• inflammatory damage to large intra and extrahepatic ducts
• fibrotic obliteration of small-medium intrahepatic ducts
• periductal fibrosis -> “onion-skinning”

Question 4

What is the presentation of PBC and PSC?

Answer 4

PBC:

• pruritis
• jaundice
• xanthelasma

PSC:

• pruritis
• jaundice

Question 5

What are complications of PBC and PSC?

Answer 5

PBC:

-cirrhosis

PSC:

-cholangiocarcinoma

Question 6

What features are used as the primary diagnostic features in PBC and PSC?

Answer 6

PBC:

• biopsy -> flurid duct lesion
• no findings on imaging

PSC:

• ERCP/MRCP -> “beading”
• biopsy shows “onion skinning” but is not necessary for diagnosis

Question 7

What is the treatment of PBC and PSC?

Answer 7

PBC:

• ursodeoxycholate
• cholestyramine for pruritits
• liver transplant in advanced stages

PSC:

• no therapy exists
• cholestyramine for pruritits
• liver transplant in advanced stages