Path-Cholestatic & Autoimmune Flashcards Preview

Gastrointestinal > Path-Cholestatic & Autoimmune > Flashcards

Flashcards in Path-Cholestatic & Autoimmune Deck (46):
1

which cholestatic liver diseases lead to obstruction of large caliber extrahepatic ducts?

choledocholithiasis, pancreatic cancer

2

which cholestatic liver diseases involve intermediate caliber intra- and extrahepatic ducts?

cholangiocarcinoma, primary sclerosing cholangitis

3

which cholestatic liver diseases involve small caliber intrahepatic bile ducts?

sarcoidosis, primary biliary cirrhosis

4

which cholestatic and hepatic diseases are of autoimmune etiology?

primary sclerosing cholangitis (PSC), primary biliary cirrhosis (PBC), and autoimmune hepatitis (AIH)

5

which cholestatic diseases are a result of mechanical obstruction of the biliary tree?

choledocholithiasis, pancreatic carcinoma, and cholangiocarinoma

6

name the bile ducts from smallest to largest in order of branching

interlobular, septal, segmental, right & left hepatic, common hepatic (+ cystic), common bile

7

cholestasis is defined as? (3 parts)

defective excretion of bile from liver, bile pigment retained in hepatocytes, inspissated bile plugs dilated canaliculi

8

name 5 characteristic pathological findings seen in acute obstruction of large caliber bile ducts (choledocholithiasis, gallstones)

edematous expansion of portal areas, ductular reaction (prolif of multiple small caliber bile ductules adjacent to limiting plate), distended canaliculi, hepatocyte ballooning and green discoloration (from bile pigment), and retained bile salts that cause toxic injury

9

what engulfs pigmented debris from damaged hepatocytes?

centrilobular Kupffer cells

10

what type of stain would help you visualize the bile duct epithelium (to see ductular rxn)?

cytokeratin 7

11

which portion of the biliary tree is usually compressed in cancer of the pancreatic head? Blocked by choledocholithiasis?

common bile duct

12

RUQ pain, jaundice, fever with elevated serum bili and elevated WBC are likely?

the development of ascending cholangitis secondary to choledocholithiasis

13

what does ascending cholangitis look like on histo?

neutrophils seen in the wall and lumen of interlobular bile ducts

14

if a gallstone becomes inpacted at the ampulla of Vater, reflux of bile enters the ________ duct, causing sever acute ___________

pancreatic, pancreatitis

15

a malignant tumor arising from the epithelium of the common hepatic duct is most likely?

cholangiocarcinoma

16

cholangiocarcinoma results in obstruction of an _____ caliber duct, with _____ of smaller ducts of the intrahepatic biliary tree

intermediate or large; dilation

17

cholangiocarcinomas are (benign/malignant) _____ differentiated ____carcinomas that grow (quickly/slowly) and are usually (advanced/early) at time of detection

malignant, moderately, adeno-, slowly, advanced

18

what is the only cure for cholangiocarcinoma?

surgical resection

19

what are risk factors for cholangiocarcinoma?

PSC, bile duct cysts, infection with liver flukes

20

how do you distinguish cholangiocarcinoma from metastatic adenocarcinoma?

centrally, neoplastic glands are more sparsely distributed within a dense hyalinized stroma

21

alternating strictures and ballooning dilation of the biliary tree is seen in?

primary sclerosing cholangitis

22

areas of intrahepatic bile duct stricture can be visualized histologically by?

concentric ("onion skin") periductal fibrosis of intermediate caliber intrahepatic ducts

23

bile ducts track with _____ of the same size

arteries

24

an artery that has lost its paired bile duct is referred to as?

a "widowed artery"

25

persistent periductal fibrosis may lead to?

obliteration of duct, replaced by nodule of scar tissue

26

primary sclerosing cholangitis is a (chronic/acute), inflammatory, fibrosing disease of the ______ with segmental narrowing, beading, and pruning, repeated bouts of ____, and increased risk for bile duct ______

chronic, biliary tree, ascending cholangitis, dysplasia

27

____ is a common etiology of PCS, while PCS may cause?

IBD (esp UC); cholangiocarcinoma, cirrhosis, portal HTN, liver failure

28

it is best to do a ____ biopsy to diagnose PSC early

wedge biopsy (not a needle biopsy)

29

primary biliary cirrhosis is caused by?

an autoimmune attack on intrahepatic bile ducts by cytotoxic T-lymphocytes

30

which gender is more commonly affected by PBC?

women

31

PBC labs typically show?

increased AP, GGT, bili as disease progresses

32

useful serologic studies for PBC include?

anti-mito antibody (AMA) present in virtually all patients, IgM variably increased

33

histologic features of PBC

lymphocytes infiltrate and damage interlobular bile ducts, leading to irregular, distorted lumen with swelling and increased eosinophilia of cytoplasm

34

what other histologic feature may be present in PBC?

granulomas

35

describe the architectural pattern of biliary cirrhosis?

residual liver parenchyma have a bizarre jigsaw puzzle-piece like appearance

36

sarcoidosis of the liver results in abnormal liver enzymes and a (large/small) liver

elevated; large

37

lab findings of sarcoidosis of the liver

elevated AP, AST/ALT normal or mild elevation, ACE elevated (also elevated in PBC)

38

what is associated with the elevated ACE in PBC and sarcoidosis?

seen in granulomatous diseases

39

clinical sx of liver sarcoidosis are predominantly ____, resulting from?

cholestatic, obstruction of interlobular bile ductules by granulomas

40

what must be excluded before treating for sarcoidosis?

infection, bc tx for sarcoidosis is immune suppression

41

the injury pattern in autoimmune hepatitis is similar to viral hepatitis, showing both ____ and ____ injury patterns

lobular, interface

42

what features, in addition to lobular and interface acidophil bodies and inflammatory cells, are seen in autoimmune hepatitis specifically?

plasma cells makes up the major component of inflammatory cells; untreated AIH leads to lots of acidophil bodies; perivenular plasma cells and accompanying acidophil bodies are common

43

risk factors for autoimmune hepatitis

being a young woman, other autoimmune disorders

44

lab findings of autoimmune hepatitis

high ALT/AST, positive for autoimmune antibodies, hypergammaglobulinemia, viral NEGATIVE

45

treatment for AIH

immunosuppression

46

what is the clinical course for AIH?

highly varied; some see slow progression to ESLD, others show fulminant dz leading to acute liver failure