Primary sclerosing cholangitis, primary biliary cirrhosis Flashcards
Define primary sclerosing cholangitis
Bile duct injury ( intra and extrahepatic) characterized by inflammation, fibrosis, and strictures
In what population is primary sclerosing cholangitis most frequently seen?
Men, usually before 45
Primary sclerosing cholangitis is an ______ disease
autoimmune
thought that an inflammatory/ infectious trigger initiates (bacteria from portal circulation??)
p-ANCA
______ attack on the bile duct leads to scarring and strictures in PSC
T cell
75% of people with PSC have ________
ulcerative colitis
Describe the clinical presentation of PSC
asymptomatic in 10% of people
fatigue, abdominal pain, weight loss, pruritis, episodic jaundice, rarely acute cholangitis
How is PSC diagnosed?
Alkaline phosphatase 2x normal
Elevated transaminases, bilirubin, prothrombin time
low albumin
ERCP is gold standard for diagnosis: multifocal strictures with intervening dilated ducts (beaded appearance)
onion ring appearance on liver biopsy
Describe the prognosis of PSC
Slow progression to secondary biliary cirrhosis, portal HTN, liver failure
12 yr median survival
increased risk of cholangiocarcinoma
Describe treatment of PSC
supportive- no intervention is successful in slowing disease progression (including immune suppression) Endoscopic procedures to remove stones, stent strictures liver transplant (new liver will get PSC)
_______ is the most common form of cholangiocarcinoma
Klatskin tumor (at bifurcation)
List causes that have been associated with cholangiocarcinoma
PSC, gallstones, liver flukes, cysts, toxic exposure
Describe the presentation of cholangiocarcinoma
non-specific weight loss, anorexia, abdominal pain, failure to thrive
new onset or worsening LFT elevation, jaundice
What are serum tumor markers for cholangiocarcinoma
CEA and CA 19-9
Cholangiocarcinoma is extremely difficult to diagnose particularly with longstanding _______
PSC
What is the prognosis for cholangiocarcinoma?
Extremely poor- 3 years if tumor is resectable, 1 year if not
Define primary biliary cirrhosis
Progressive chronic cholestatic autoimmune disease that affects the small microscopic intrahepatic bile ducts
What population is most commonly affected by primary biliary cirrhosis?
women»men
6th or 7th decade
What immunologic abnormalities are seen in PBC?
T-cell mediated autoimmune response against bile duct epithelial cells
presence of antimitochondrial antibodies (AMA), IgM hypergammaglobulinemia, circulating immune complexes
Describe the pathophysiology of PBC
Chronic damage/destruction of small ducts causes obstruction of bile flow and cholestasis»>hepatocyte injury, scarring + accumulation of bile acids
Possible cirrhosis and liver failure
What are symptoms of PBC?
Very often asymptomatic at diagnosis
fatigue, pruritis worse at night, jaundice
What is the underlying cause of the bone disease seen in PBC?
Bone disease – osteoporosis and osteomalacia secondary to Vit D malabsorption
Describe the metabolic sequelae of PBC
Weight loss, steatorrhea, bone disease, vitamin deficiencies – bile secretory failure and low intestinal bile salt levels
Describe the liver sequelae of PBC
Later stages- cirrhosis, portal hypertension
Ascites
Esophageal varices and bleeding
Increased risk of hepatocellular carcinoma
How is PBC diagnosed?
Elevated ALKP, bilirubin
Positive AMA, hypergammaglobulinemia
Liver biopsy shows patchy destruction of interlobular bile ducts with mononuclear infiltrate and possible granulomas
