Biliary Disorders B&B Flashcards
what are the clinical features (3) and treatment for biliary atresia?
biliary atresia = idiopathic biliary obstruction in neonates (ducts do not form or degenerate)
causes jaundice + dark urine + pale stools (“acholic”)
tx = Kasai procedure: create conduit for bile draining using small intestine
describe the pathology of primary biliary cirrhosis
T-cell mediated granulomatous inflammation causes biliary cirrhosis (WITHOUT extra-hepatic obstruction such as stone) within the interlobular bile ducts of the liver
AUTOIMMUNE disorder !
describe the clinical presentation (2) of primary biliary cirrhosis
autoimmune T-cell mediated granulomatous inflammation within the interlobular bile ducts of the liver
more common in women, presents with fatigue + pruritus (BEFORE jaundice) because bile acids leak into serum and skin
ITCHING!! which is often worse at NIGHT
Pt is a 27yo F presenting with complaint of fatigue and unrelenting itchiness which is worse at night. Skin examination is unremarkable, and pt says lotions have been ineffective. PMH is notable for Sjogren’s. A biopsy is taken from a certain tissue, which shows granulomatous inflammation. From where was this tissue taken, and what is the pathology of her itchiness?
primary biliary cirrhosis: autoimmune T-cell mediated granulomatous inflammation within the interlobular bile ducts of the liver
more common in women, presents with fatigue + pruritus (BEFORE jaundice) because bile acids leak into serum and skin
ITCHING!! which is often worse at NIGHT
Pt is a 27yo F presenting with complaint of fatigue and unrelenting itchiness which is worse at night. Skin examination is unremarkable, and pt says lotions have been ineffective. PMH is notable for Sjogren’s. A biopsy is taken from a certain tissue, which shows granulomatous inflammation. The pt is ANA positive. Which 2 other key labs do you expect to be abnormal?
dx = primary biliary cirrhosis: autoimmune T-cell mediated granulomatous inflammation within the interlobular bile ducts of the liver
more common in women, presents with fatigue + pruritus (BEFORE jaundice) which is worse at night
labs show HIGH alkaline phosphatase + anti-mitochondrial Abs (hallmark)
increase in bilirubin (causing jaundice) occurs late and has poor prognosis
what will labs show in primary biliary cirrhosis? (4)
autoimmune T-cell mediated granulomatous inflammation within the interlobular bile ducts of the liver
labs show:
1. anti-mitochondrial Abs - hallmark
2. HIGH alkaline phosphatase (may see elevated AST/ALT also)
3. anti-nuclear Abs (ANA) (~70%)
4. HIGH lipids (>1000) - due to obstruction of biliary flow, cause xanxthomas
elevated bilirubin (causing jaundice) occurs late and has poor prognosis
anti-mitochondrial antibodies =
primary biliary cirrhosis: autoimmune T-cell mediated granulomatous inflammation within the interlobular bile ducts of the liver
labs show:
1. anti-mitochondrial Abs - hallmark
2. HIGH alkaline phosphatase (may see elevated AST/ALT also)
3. anti-nuclear Abs (ANA) (~70%)
4. HIGH lipids (>1000) - due to obstruction of biliary flow, cause xanxthomas
elevated bilirubin (causing jaundice) occurs late and has poor prognosis
Pt is 33yo F presenting with complaint of fatigue and skin itchiness. Skin exam is notable for xanthomas. Labs show total cholesterol is >1000 and LFTs show elevation of alkaline phosphatase. Imaging is negative for biliary obstruction. What would liver biopsy show, and what is the tx?
dx = primary biliary cirrhosis: autoimmune T-cell mediated granulomatous inflammation within the interlobular bile ducts of the liver
more common in women, presents with fatigue + pruritus
labs show HIGH alkaline phosphatase + anti-mitochondrial Abs (hallmark) + HIGH lipids (causes xanthomas)
tx = liver transplant or ursodeoxycholic acid - similar to bile acids but less toxic to hepatocytes
what pharmacological treatment is available for primary biliary cirrhosis?
autoimmune T-cell mediated granulomatous inflammation within the interlobular bile ducts of the liver
labs show HIGH alkaline phosphatase + anti-mitochondrial Abs (hallmark)
tx = liver transplant or ursodeoxycholic acid - similar to bile acids but less toxic to hepatocytes
with which autoimmune disorder is primary biliary cirrhosis most commonly associated?
autoimmune T-cell mediated granulomatous inflammation within the interlobular bile ducts of the liver
more common in women, presents with fatigue + pruritus
associated most commonly with Sjogren’s (dry eyes + dry mouth)
describe the pathology of primary sclerosing cholangitis
autoimmune inflammation causes fibrosis + strictures of biliary tree involving intra + extra-hepatic bile ducts —> RUQ pain + jaundice
90% associated with ulcerative colitis
with which condition is primary sclerosing cholangitis almost always associated?
autoimmune inflammation causes fibrosis + strictures of biliary tree involving intra + extra-hepatic bile ducts —> RUQ pain + jaundice
90% associated with ulcerative colitis (+p-ANCA)
what do labs show in primary sclerosing cholangitis? (3)
autoimmune inflammation causes fibrosis + strictures of biliary tree involving intra + extra-hepatic bile ducts; 90% associated with ulcerative colitis
labs show:
1. cholestasis (elevated Alk phos and conjugated bilirubin)
2. elevated IgM (just go with it)
3. positive p-ANCA (this makes sense bc this is also seen in UC)
describe the histology findings of primary sclerosing cholangitis
autoimmune inflammation causes fibrosis + strictures of biliary tree involving intra + extra-hepatic bile ducts —> RUQ pain + jaundice
histology shows ”onion-skinning” periductal fibrosis
90% associated with ulcerative colitis
how is primary sclerosing cholangitis diagnosed (confirmatory)?
autoimmune inflammation causes fibrosis + strictures of biliary tree involving intra + extra-hepatic bile ducts —> RUQ pain + jaundice; 90% associated with ulcerative colitis (+p-ANCA)
confirmatory dx via cholangiogram (ERCP or MRCP) which shows beading due to alternating biliary strictures and dilations
what annual screening should patients with primary sclerosing cholangitis obtain?
autoimmune inflammation causes fibrosis + strictures of biliary tree involving intra + extra-hepatic bile ducts; 90% associated with ulcerative colitis
tx = endoscopic stenting of strictures or liver transplant + annual screening for cholangiocarcinoma (bc of chronic inflammation)
Pt is a 41yo F presenting with RUQ pain and fatigue. PMH is notable for ulcerative colitis. PE is notable for jaundice. Labs are notable for elevated IgM and positive for p-ANCA. What is the dx?
primary sclerosing cholangitis: autoimmune inflammation causes fibrosis + strictures of biliary tree involving intra + extra-hepatic bile ducts —> RUQ pain + jaundice
90% associated with ulcerative colitis
Pt is a 41yo F presenting with RUQ pain and fatigue. PMH is notable for ulcerative colitis. PE is notable for jaundice. A cholangiogram is performed, which shows beading of the intra- and extra-hepatic bile ducts. What would a biopsy of these ducts show? What annual screening should this patient receive?
primary sclerosing cholangitis: autoimmune inflammation causes fibrosis + strictures of biliary tree involving intra + extra-hepatic bile ducts; 90% associated with ulcerative colitis
histology shows ”onion-skinning” periductal fibrosis
annual screening for cholangiocarcinoma (bc of chronic inflammation)
contrast the following features of primary biliary cirrhosis vs primary sclerosing cholangitis:
a. localization of damage
b. autoimmune association
c. presenting symptoms
d. Abs
primary biliary cirrhosis:
a. intrahepatic bile ducts, via T-cell mediated granulomatous inflammation
b. associated with Sjogren’s
c. presents with itching
d. anti-mitochondrial Abs
primary sclerosing cholangitis:
a. intra+extra hepatic bile ducts, via strictures
b. associated with ulcerative colitis
c. presents with RUQ pain + jaundice
d. elevated IgM and +p-ANCA
what is the cellular origin of cholangiocarcinoma? what is the risk factor for development?
rare cancer of bile duct epithelial cells —> symptoms from bile duct obstruction
increased risk with chronic biliary inflammation (primary sclerosing cholangitis, clonorchis sinensis/ Chinese liver fluke)
which 2 conditions are key risk factors for cholangiocarcinoma?
rare cancer of bile duct epithelial cells —> symptoms from bile duct obstruction
increased risk with chronic biliary inflammation:
1. primary sclerosing cholangitis
2. clonorchis sinensis (Chinese liver fluke)
