Primary biliary cholangitis & Primary sclerosing cholangitis Flashcards
describe what type of disease primary biliary cholangitis(PBC) and primary sclerosing cholangitis(PSC) are
autoimmune liver diseases
describe the disease pathway for PBC
chronic portal inflammation, fibrosis, cirrhosis
what part of the liver does primary biliary cholangitis(PBC) affect
small bile ducts
what group of people is PBC most prevalent in
middle-age women, (90% females)
what are the symptoms/signs associated with PBC
fatigue, itch without rash, xanthomas, can be asymptomatic
what investigations are done to diagnose PBC
+ve AMA, cholestatic LFTs, liver biopsy
what are the treatment options for PBC
urseo deoxycholic acid, obeticholic acid, liver transplant in some cases
describe the different outcomes of PBC
most won’t develop symptoms in lifetime, majority with symptoms don’t develop liver failure, not all with liver failure are able to receive transplant
describe what primary sclerosing cholangitis is
chronic inflammatory process effecting medium and large intra- and extra-hepatic bile ducts
what does PSC lead to
fibrosis, duct destruction, jaundice
what disease is PSC associated with
ulcerative colitis(IBD)
what disease does PSC increase the risk of developing
malignancy in bile ducts and colon
what gender is mostly effected by PSC
males
what is seen clinically with PSC
recurrent cholangitis
what does cholangitis mean
inflammation of the bile ducts
