Primary Biliary Cholangitis Flashcards
which antibodies are implicated?
how many pts have these antibodies?
Anti-microbial antibodies
90%
primary biliary cholangitis used to be called what?
primary biliary cirrhosis
describe pathophysiology of primary biliary cholangitis?
Inflammation & lymphocytes destroy intrahepatic bile ducts - leads to cholestasis –> bile builds in liver –> bile leaks into circulation –> liver more inflamed + damaged –> cirrhosis, fibrosis.
why is there cholestasis?
what happens to the bile in the liver then?
intrahepatic bile ducts being destroyed
leaks out
why is there jaundice?
bilirubin in bile leaks out
why does cholestasis damage liver even more?
bile has toxic materials, leak out
what is the main pigment in bile?
bilirubin
what is the main excretory method for cholesterol?
via bile
why might here be hypercholesterolaemia in this condition?
what signs may be seen?
cholesterol in bile leaks out
xanthelasma + xanthomata
which vitamins need supplementing?
why these vitamins?
ADEK
fat-soluble vitamins
what happens to fat absorption in late disease?
why?
fat malabsorption
less bile going to gut
what is classic presentation?
MIDDLE-AGED FEMALE WITH ITCHING AND FATIGUE.
which other autoimmune condition is associated with primary biliary cholangitis?
- the most common one
classic features of this disease?
-2
Sjogren’s syndrome
Dry eyes + dry mouth
which age is a risk factor?
45-60 years old
which gender is a risk factor?
Female
which lifestyle factor is a risk factor?
Smoking
which type of infection is a risk factor?
Chronic UTI
two most common symptoms?
Pruritus
Fatigue
list all symptoms?
-8
Pruritus Fatigue Weight loss Dry eyes + dry mouth Obstructive jaundice symptoms - Yellow skin, pale stools, dark urine
which sign might be seen on the face area in late disease?
-2
Yellow sclera
xanthelasma
which sign might you be able to palpate in the abdominal area?
hepatosplenomegaly
which sign might you see in fingers specifically?
Clubbing
which signs might be seen in the skin?
-3
causes of this?
Skin hyperpigmentation - high melanin
xanthelasma - cholesterol builds up in skin
jaundice - high bilirubin
1st line Ix?
if this is negative what is the second line Ix?
Antimicrobial antibodies (AMA)
Antinuclear antibodies (ANA)
LFT results?
↑↑↑ALP ↑↑↑GGT ↑Bilirubin
↑AST ↑ALT
why would you do US?
exclude gallstones
1st line Mx?
-3 things
Ursodeoxycholic acid + ADEK supplement + cholestyramine
what do you give to stop pruritus?
what group of drugs does this belong in?
basic effect of this drug?
Cholestyramine
bile acid sequestrant
↓bile acids and ↓cholesterol
pt in end stage disease and drugs have not worked.
2nd line Mx?
Liver transplant
which secondary bile acid is given as a treatment for primary biliary cholangitis?
how does this drug help?
Ursodeoxycholic acid
stops apoptosis in the bile ducts
pt has primary biliary cholangitis.
what happens to there cholesterol levels as disease progresses?
increased cholesterol
pt has primary biliary cholangitis.
which type of jaundice might occur in later disease stages?
symptoms of this?
-3
obstructive jaundice
Yellow skin, pale stools, dark urine
describe pathophysiology of primary biliary cholangitis?
Inflammation & lymphocytes destroy intrahepatic bile ducts - leads to cholestasis –> bile builds in liver –> bile leaks into circulation –> liver more inflamed + damaged –> cirrhosis, fibrosis.
which serious complication can happen with primary biliary cholangitis that causes ascites and bleeding?
portal HTN
in portal HTN where does the bleeding happen?
variceal bleeding
