PBC Flashcards
What is the cause?
Unknown environmental triggers and genetic predisposition leads to loss of immune self tolerance of mitochondrial antibodies
Knowing the cause what is therefore the hallmark feature of PBC?
Raised AMA
More likely in what sex?
Female (9:1)
What risk factors are there for PBC?
FMH, many UTIs, smoking, past pregnancy, other autoimmune diseases, high use of nail polish/hair dye
What is the typical age at presentation?
~50
Symptoms include?
Often none, lethargy, sleepiness, pruritus, jaundice
What are the signs of PBC?
Skin pigmentation, xantholasma, xanthomata, hepatomegaly, splenomegaly, capital medusae
Complications of PBC include?
Cirrhosis, osetoporosis, malabsorption of fat soluble vitamins, coagulapathy, HCC (screen twice per annum)
What tests should you do?
LFTs, AMA, other autoantibodies, USS, may do biopsy
What might you see from the LFTs?
Raised: ALP, gamma glutyl transaminase, AST, ALT,
In late disease: raised bilirubin, low albumin, raised PTT, raised cholesterol
What differentials might you consider?
Sarcoidosis, TB, parasites, drug reaction
What is the management for primary biliary sclerosis?
Colestyramine, ursodeoxycholic acid, regular monitoring
Key: liver transplant
What is the prognosis for PBC once jaundice has developed?
Less than 2 years if no transplant is received
What is PBC?
Interlobular bile duct destruction by autoimmune granulomatous inflammation causing cholestasis which can then lead to hepatic fibrosis and cirrhosis as well as portal hypertension
