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
