70. Biliary tract disease Flashcards Preview

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Flashcards in 70. Biliary tract disease Deck (33):
1

primary biliary cirhosis - antibodies

antimitochondrial

2

autoimmune heaptitis type 1 - antibodies

anti smoth muscle

3

Biliary tract disease associated with ulcerative colitis

primary sclerosing cholangitis

4

biliary tract disease types

1. Primary sclerosing cholangitis
2. Primary biliary cirrhosis
3. Secondary biliary cirrhosis

5

Biliary tract disease - clinical presentation

1. pruritus
2. jaundice
3. dark urine
4. light - colored stiik
5. hepatosplenomegaly

6

biliary tracr disease - labs

cholestatic pattern of LFTs:
1. increased CB
2. increased cholesterol
3. increased ALP

7

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

8

appearance ( histology and gross)

histology --> concentric "onion skin" bile duct fibrosis ( intrahepatic and extrahepatic ducts ) --> alternating strictures and dilation "beading" on ERCP and MRCP

9

ERCP?
MRCP

Endoscopic Retrgrade Cholangiopancreatography
Magnetic Resonance CHolangiopancreatography

10

Primary sclerosing cholangitis is associated with

1. UC
2. p-anca
3. high Ig M

11

Primary sclerosing cholangitis can lead secondary to

1. biliary cirrhosis
2. cholangiocarcinoma
3. gallbladder cancer

12

primary biliary cirrhosis - mechanism

anti-mitochondrial antibodies --> autoimmune reaction --> lymphocytic infiltrate + granulomas --> destraction intralobular bile ducts

13

primary biliary cirrhosis associated with

1. antimitochondrial antibodies
2. increased IgM
3. other autoimmune conditions ( eg. Sjogren, CREST, Hashimoto, RA, celiac disease)

14

primary biliary cirrhosis can lead to

cirrhosis

15

Secondary biliary cirrhosis -mechanism

extrahepatic biliary obstruction ( gallstones , biliary stuctures , pancreatic CA ) --> high pressure in intrahepatic ducts --> injury / fibrosis and bie stasis

16

Secondary biliary cirrhosis can lead to

1. cirrhosis
2. ascending cholangitis

17

• Is primary sclerosing cholangitis intrahepatic, extrahepatic, or both?

Both (the entire biliary tree is affected)

18

• 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)

19

• 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

20

• 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)

21

• 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)

22

• What autoimmune disease is classically associated with primary sclerosing cholangitis?
I

ΙBD (especially in young men) (more specifically, ulcerative colitis)

23

• Which antibody abnormalities are classically seen in patients with primary biliary cirrhosis?

Positive anti-mitochondrial antibodies, elevated IgM antibodies

24

• 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

25

• 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)

26

• 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)

27

• 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)

28

• 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

29

• What class of immunoglobulin is elevated in primary sclerosing cholangitis?

Immunoglobulin M

30

• What causes secondary biliary cirrhosis?

Extrahepatic biliary obstruction leading to increased pressure in the intrahepatic ducts, with fibrosis and stasis of bile

31

• 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

32

• Name some disease states associated with primary biliary cirrhosis.

Autoimmune conditions such as CREST, Sjögren syndrome, rheumatoid arthritis, celiac disease

33

• A man with primary sclerosing cholangitis presents with a right upper quadrant mass. After HCC, what is the next most concerning etiology?

Cholangiocarcinoma, a potential complication of primary sclerosing cholangitis

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