Primary Biliary Cholangitis Flashcards
_______ bile ducts are damaged by chronic autoimmune ______ inflammation causing cholestasis (destruction of bile ducts) which may lead to fibrosis, cirrhosis and portal hypertension.
Interlobular bile ducts are damaged by chronic autoimmune granulomatous inflammation causing cholestasis (destrction of bile ducts) which may lead to fibrosis, cirrhosis and portal hypertension.
what causes it?
anti-mitochondrial antibodies (AMA) are found in almost all patients and the mitochondiral protein antigen M2 is involved
Autoantigens M2-E2 E3 subunits of pyruvate dehydrogenase complex - E2 in the inner leaflet of the mitochondrial membrane triggers ____
AMA
It it _-cell mediated, ___ cells become reactive to ___ target. There is a loss of tolerance for the ___ mitochondrial protein.
It it T-cell mediated, CD4 cells become reactive to M2 target. There is a loss of tolerance for the M2 mitochondrial protein.
what pathology is diagnostic of PBC?
can see a collection of macrophages eating away at the side of bile duct
more cmmon in males?
no females
what is the clinical presentation?
- usually assymptomatic but can have
- Fatigue
- Itch without rash
- Xanthelomas
- jaundice
what increases your risk of PBC?
Family history, may UTIs, smoking, past pregnancy, other autoimmune, increased use of hair dye and nail polish
how is a diagnosis made?
2 of
- Positive AMA
- Cholestatic LFTs
– Liver biopsy - look for granulomas around bile ducts ± cirrhosis
Generally don’t need to do a biopsy if have positive mitochondrial but sometimes the boundaries between disease are unclear
what is the treatment ?
Urseo deoxycholic acid
This reduces the inflammation and reduces secondary bile damage
associated with an increased risk of ______
hepatocarcinoma
