Path-Cholestatic & Autoimmune

Question 1

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

Answer 1

choledocholithiasis, pancreatic cancer

Question 2

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

Answer 2

cholangiocarcinoma, primary sclerosing cholangitis

Question 3

which cholestatic liver diseases involve small caliber intrahepatic bile ducts?

Answer 3

sarcoidosis, primary biliary cirrhosis

Question 4

which cholestatic and hepatic diseases are of autoimmune etiology?

Answer 4

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

Question 5

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

Answer 5

choledocholithiasis, pancreatic carcinoma, and cholangiocarinoma

Question 6

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

Answer 6

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

Question 7

cholestasis is defined as? (3 parts)

Answer 7

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

Question 8

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

Answer 8

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

Question 9

what engulfs pigmented debris from damaged hepatocytes?

Answer 9

centrilobular Kupffer cells

Question 10

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

Answer 10

cytokeratin 7

Question 11

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

Answer 11

common bile duct

Question 12

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

Answer 12

the development of ascending cholangitis secondary to choledocholithiasis

Question 13

what does ascending cholangitis look like on histo?

Answer 13

neutrophils seen in the wall and lumen of interlobular bile ducts

Question 14

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

Answer 14

pancreatic, pancreatitis

Question 15

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

Answer 15

cholangiocarcinoma

Question 16

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

Answer 16

intermediate or large; dilation

Question 17

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

Answer 17

malignant, moderately, adeno-, slowly, advanced

Question 18

what is the only cure for cholangiocarcinoma?

Answer 18

surgical resection

Question 19

what are risk factors for cholangiocarcinoma?

Answer 19

PSC, bile duct cysts, infection with liver flukes

Question 20

how do you distinguish cholangiocarcinoma from metastatic adenocarcinoma?

Answer 20

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

Question 21

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

Answer 21

primary sclerosing cholangitis

Question 22

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

Answer 22

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

Question 23

bile ducts track with _____ of the same size

Answer 23

arteries

Question 24

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

Answer 24

a “widowed artery”

Question 25

persistent periductal fibrosis may lead to?

Answer 25

obliteration of duct, replaced by nodule of scar tissue

Question 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 ______

Answer 26

chronic, biliary tree, ascending cholangitis, dysplasia

Question 27

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

Answer 27

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

Question 28

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

Answer 28

wedge biopsy (not a needle biopsy)

Question 29

primary biliary cirrhosis is caused by?

Answer 29

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

Question 30

which gender is more commonly affected by PBC?

Answer 30

women

Question 31

PBC labs typically show?

Answer 31

increased AP, GGT, bili as disease progresses

Question 32

useful serologic studies for PBC include?

Answer 32

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

Question 33

histologic features of PBC

Answer 33

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

Question 34

what other histologic feature may be present in PBC?

Answer 34

granulomas

Question 35

describe the architectural pattern of biliary cirrhosis?

Answer 35

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

Question 36

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

Answer 36

elevated; large

Question 37

lab findings of sarcoidosis of the liver

Answer 37

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

Question 38

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

Answer 38

seen in granulomatous diseases

Question 39

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

Answer 39

cholestatic, obstruction of interlobular bile ductules by granulomas

Question 40

what must be excluded before treating for sarcoidosis?

Answer 40

infection, bc tx for sarcoidosis is immune suppression

Question 41

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

Answer 41

lobular, interface

Question 42

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

Answer 42

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

Question 43

risk factors for autoimmune hepatitis

Answer 43

being a young woman, other autoimmune disorders

Question 44

lab findings of autoimmune hepatitis

Answer 44

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

Question 45

treatment for AIH

Answer 45

immunosuppression

Question 46

what is the clinical course for AIH?

Answer 46

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