Path-Cholestatic & Autoimmune Flashcards
which cholestatic liver diseases lead to obstruction of large caliber extrahepatic ducts?
choledocholithiasis, pancreatic cancer
which cholestatic liver diseases involve intermediate caliber intra- and extrahepatic ducts?
cholangiocarcinoma, primary sclerosing cholangitis
which cholestatic liver diseases involve small caliber intrahepatic bile ducts?
sarcoidosis, primary biliary cirrhosis
which cholestatic and hepatic diseases are of autoimmune etiology?
primary sclerosing cholangitis (PSC), primary biliary cirrhosis (PBC), and autoimmune hepatitis (AIH)
which cholestatic diseases are a result of mechanical obstruction of the biliary tree?
choledocholithiasis, pancreatic carcinoma, and cholangiocarinoma
name the bile ducts from smallest to largest in order of branching
interlobular, septal, segmental, right & left hepatic, common hepatic (+ cystic), common bile
cholestasis is defined as? (3 parts)
defective excretion of bile from liver, bile pigment retained in hepatocytes, inspissated bile plugs dilated canaliculi
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
what engulfs pigmented debris from damaged hepatocytes?
centrilobular Kupffer cells
what type of stain would help you visualize the bile duct epithelium (to see ductular rxn)?
cytokeratin 7
which portion of the biliary tree is usually compressed in cancer of the pancreatic head? Blocked by choledocholithiasis?
common bile duct
RUQ pain, jaundice, fever with elevated serum bili and elevated WBC are likely?
the development of ascending cholangitis secondary to choledocholithiasis
what does ascending cholangitis look like on histo?
neutrophils seen in the wall and lumen of interlobular bile ducts
if a gallstone becomes inpacted at the ampulla of Vater, reflux of bile enters the ________ duct, causing sever acute ___________
pancreatic, pancreatitis
a malignant tumor arising from the epithelium of the common hepatic duct is most likely?
cholangiocarcinoma
cholangiocarcinoma results in obstruction of an _____ caliber duct, with _____ of smaller ducts of the intrahepatic biliary tree
intermediate or large; dilation
cholangiocarcinomas are (benign/malignant) _____ differentiated ____carcinomas that grow (quickly/slowly) and are usually (advanced/early) at time of detection
malignant, moderately, adeno-, slowly, advanced
what is the only cure for cholangiocarcinoma?
surgical resection
what are risk factors for cholangiocarcinoma?
PSC, bile duct cysts, infection with liver flukes
how do you distinguish cholangiocarcinoma from metastatic adenocarcinoma?
centrally, neoplastic glands are more sparsely distributed within a dense hyalinized stroma
alternating strictures and ballooning dilation of the biliary tree is seen in?
primary sclerosing cholangitis
areas of intrahepatic bile duct stricture can be visualized histologically by?
concentric (“onion skin”) periductal fibrosis of intermediate caliber intrahepatic ducts
bile ducts track with _____ of the same size
arteries
an artery that has lost its paired bile duct is referred to as?
a “widowed artery”
persistent periductal fibrosis may lead to?
obliteration of duct, replaced by nodule of scar tissue
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
____ is a common etiology of PCS, while PCS may cause?
IBD (esp UC); cholangiocarcinoma, cirrhosis, portal HTN, liver failure
it is best to do a ____ biopsy to diagnose PSC early
wedge biopsy (not a needle biopsy)
primary biliary cirrhosis is caused by?
an autoimmune attack on intrahepatic bile ducts by cytotoxic T-lymphocytes
which gender is more commonly affected by PBC?
women
PBC labs typically show?
increased AP, GGT, bili as disease progresses
useful serologic studies for PBC include?
anti-mito antibody (AMA) present in virtually all patients, IgM variably increased
histologic features of PBC
lymphocytes infiltrate and damage interlobular bile ducts, leading to irregular, distorted lumen with swelling and increased eosinophilia of cytoplasm
what other histologic feature may be present in PBC?
granulomas
describe the architectural pattern of biliary cirrhosis?
residual liver parenchyma have a bizarre jigsaw puzzle-piece like appearance
sarcoidosis of the liver results in abnormal liver enzymes and a (large/small) liver
elevated; large
lab findings of sarcoidosis of the liver
elevated AP, AST/ALT normal or mild elevation, ACE elevated (also elevated in PBC)
what is associated with the elevated ACE in PBC and sarcoidosis?
seen in granulomatous diseases
clinical sx of liver sarcoidosis are predominantly ____, resulting from?
cholestatic, obstruction of interlobular bile ductules by granulomas
what must be excluded before treating for sarcoidosis?
infection, bc tx for sarcoidosis is immune suppression
the injury pattern in autoimmune hepatitis is similar to viral hepatitis, showing both ____ and ____ injury patterns
lobular, interface
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
risk factors for autoimmune hepatitis
being a young woman, other autoimmune disorders
lab findings of autoimmune hepatitis
high ALT/AST, positive for autoimmune antibodies, hypergammaglobulinemia, viral NEGATIVE
treatment for AIH
immunosuppression
what is the clinical course for AIH?
highly varied; some see slow progression to ESLD, others show fulminant dz leading to acute liver failure
