Flashcards in 70. Biliary tract disease Deck (33):
1
primary biliary cirhosis - antibodies
antimitochondrial
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autoimmune heaptitis type 1 - antibodies
anti smoth muscle
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Biliary tract disease associated with ulcerative colitis
primary sclerosing cholangitis
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biliary tract disease types
1. Primary sclerosing cholangitis
2. Primary biliary cirrhosis
3. Secondary biliary cirrhosis
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Biliary tract disease - clinical presentation
1. pruritus
2. jaundice
3. dark urine
4. light - colored stiik
5. hepatosplenomegaly
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biliary tracr disease - labs
cholestatic pattern of LFTs:
1. increased CB
2. increased cholesterol
3. increased ALP
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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
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appearance ( histology and gross)
histology --> concentric "onion skin" bile duct fibrosis ( intrahepatic and extrahepatic ducts ) --> alternating strictures and dilation "beading" on ERCP and MRCP
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ERCP?
MRCP
Endoscopic Retrgrade Cholangiopancreatography
Magnetic Resonance CHolangiopancreatography
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Primary sclerosing cholangitis is associated with
1. UC
2. p-anca
3. high Ig M
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Primary sclerosing cholangitis can lead secondary to
1. biliary cirrhosis
2. cholangiocarcinoma
3. gallbladder cancer
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primary biliary cirrhosis - mechanism
anti-mitochondrial antibodies --> autoimmune reaction --> lymphocytic infiltrate + granulomas --> destraction intralobular bile ducts
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primary biliary cirrhosis associated with
1. antimitochondrial antibodies
2. increased IgM
3. other autoimmune conditions ( eg. Sjogren, CREST, Hashimoto, RA, celiac disease)
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primary biliary cirrhosis can lead to
cirrhosis
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Secondary biliary cirrhosis -mechanism
extrahepatic biliary obstruction ( gallstones , biliary stuctures , pancreatic CA ) --> high pressure in intrahepatic ducts --> injury / fibrosis and bie stasis
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Secondary biliary cirrhosis can lead to
1. cirrhosis
2. ascending cholangitis
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• Is primary sclerosing cholangitis intrahepatic, extrahepatic, or both?
Both (the entire biliary tree is affected)
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• 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)
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• 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
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• 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)
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• 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)
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• What autoimmune disease is classically associated with primary sclerosing cholangitis?
I
ΙBD (especially in young men) (more specifically, ulcerative colitis)
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• Which antibody abnormalities are classically seen in patients with primary biliary cirrhosis?
Positive anti-mitochondrial antibodies, elevated IgM antibodies
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• 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
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• 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)
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• 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)
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• 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)
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• 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
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• What class of immunoglobulin is elevated in primary sclerosing cholangitis?
Immunoglobulin M
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• What causes secondary biliary cirrhosis?
Extrahepatic biliary obstruction leading to increased pressure in the intrahepatic ducts, with fibrosis and stasis of bile
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• 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
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• Name some disease states associated with primary biliary cirrhosis.
Autoimmune conditions such as CREST, Sjögren syndrome, rheumatoid arthritis, celiac disease
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