Flashcards in 70. Biliary tract disease Deck (33):
primary biliary cirhosis - antibodies
autoimmune heaptitis type 1 - antibodies
anti smoth muscle
Biliary tract disease associated with ulcerative colitis
primary sclerosing cholangitis
biliary tract disease types
1. Primary sclerosing cholangitis
2. Primary biliary cirrhosis
3. Secondary biliary cirrhosis
Biliary tract disease - clinical presentation
3. dark urine
4. light - colored stiik
biliary tracr disease - labs
cholestatic pattern of LFTs:
1. increased CB
2. increased cholesterol
3. increased ALP
types and epidemiology ( what type of patients
1. Primary sclerosing cholangitis --> middle aged men with IBD (UC)
2. Primary billiary cirrhosis --> middle aged women
3. Secondary biliary cirhosis--> pateints with known obstructive lesions ( gallstone, biliary strictures, pancreatic carcinoma
appearance ( histology and gross)
histology --> concentric "onion skin" bile duct fibrosis ( intrahepatic and extrahepatic ducts ) --> alternating strictures and dilation "beading" on ERCP and MRCP
Endoscopic Retrgrade Cholangiopancreatography
Magnetic Resonance CHolangiopancreatography
Primary sclerosing cholangitis is associated with
3. high Ig M
Primary sclerosing cholangitis can lead secondary to
1. biliary cirrhosis
3. gallbladder cancer
primary biliary cirrhosis - mechanism
anti-mitochondrial antibodies --> autoimmune reaction --> lymphocytic infiltrate + granulomas --> destraction intralobular bile ducts
primary biliary cirrhosis associated with
1. antimitochondrial antibodies
2. increased IgM
3. other autoimmune conditions ( eg. Sjogren, CREST, Hashimoto, RA, celiac disease)
primary biliary cirrhosis can lead to
Secondary biliary cirrhosis -mechanism
extrahepatic biliary obstruction ( gallstones , biliary stuctures , pancreatic CA ) --> high pressure in intrahepatic ducts --> injury / fibrosis and bie stasis
Secondary biliary cirrhosis can lead to
2. ascending cholangitis
• Is primary sclerosing cholangitis intrahepatic, extrahepatic, or both?
Both (the entire biliary tree is affected)
• What is the underlying change in primary sclerosing cholangitis?
Concentric "onion skin" fibrosis of unknown cause (leading to alternating strictures and dilation of intra-/extrahepatic ducts)
• A 39-year-old woman has a liver biopsy. What findings would suggest a diagnosis of primary biliary cirrhosis?
Lymphocytic infiltrate, granulomas, destruction of intralobular bile ducts
• A man with jaundice and dark urine undergoes both ERCP and MRCP. What finding(s) suggest(s) primary sclerosing cholangitis?
Alternating strictures, dilation of the bile ducts (also called beading)
• A patient has jaundice, light stools, pruritus, dark urine, and hepatosplenomegaly. What organ is most likely affected?
The biliary tracts (this is a common presentation for both primary and secondary biliary cirrhosis)
• What autoimmune disease is classically associated with primary sclerosing cholangitis?
ΙBD (especially in young men) (more specifically, ulcerative colitis)
• Which antibody abnormalities are classically seen in patients with primary biliary cirrhosis?
Positive anti-mitochondrial antibodies, elevated IgM antibodies
• How can secondary biliary cirrhosis result from primary sclerosing cholangitis?
PSC can cause biliary strictures, which cause extrahepatic biliary obstruction, leading to secondary biliary cirrhosis
• A patient has secondary biliary cirrhosis. What do you expect his conjugated bilirubin, cholesterol, and alkaline phosphatase levels to be?
All increased (a cholestatic pattern)
• A woman is positive for antimitochondrial antibodies. What will his conjugated bilirubin, cholesterol, and alkaline phosphatase levels be?
All will be increased (the patient has primary biliary cirrhosis, and a cholestatic pattern is seen)
• A man has a biliary disease associated with ulcerative colitis. What are conjugated bilirubin, cholesterol, and alkaline phosphatase levels?
All will be increased (the patient has primary sclerosing cholangitis, and a cholestatic pattern is seen)
• A patient with CREST syndrome and celiac disease presents with jaundice. What biliary tract disease does she most likely have?
Primary biliary cirrhosis (classically associated with other autoimmune conditions
• What class of immunoglobulin is elevated in primary sclerosing cholangitis?
• What causes secondary biliary cirrhosis?
Extrahepatic biliary obstruction leading to increased pressure in the intrahepatic ducts, with fibrosis and stasis of bile
• A patient with secondary biliary cirrhosis presents with fever and abdominal pain. What complication must now be considered?
Ascending cholangitis, which is a bacterial infection facilitated by biliary stasis
• Name some disease states associated with primary biliary cirrhosis.
Autoimmune conditions such as CREST, Sjögren syndrome, rheumatoid arthritis, celiac disease