Primary Biliary Cholangitis Flashcards

1
Q

which antibodies are implicated?

how many pts have these antibodies?

A

Anti-microbial antibodies

90%

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2
Q

primary biliary cholangitis used to be called what?

A

primary biliary cirrhosis

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3
Q

describe pathophysiology of primary biliary cholangitis?

A

Inflammation & lymphocytes destroy intrahepatic bile ducts - leads to cholestasis –> bile builds in liver –> bile leaks into circulation –> liver more inflamed + damaged –> cirrhosis, fibrosis.

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4
Q

why is there cholestasis?

what happens to the bile in the liver then?

A

intrahepatic bile ducts being destroyed

leaks out

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5
Q

why is there jaundice?

A

bilirubin in bile leaks out

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6
Q

why does cholestasis damage liver even more?

A

bile has toxic materials, leak out

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7
Q

what is the main pigment in bile?

A

bilirubin

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8
Q

what is the main excretory method for cholesterol?

A

via bile

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9
Q

why might here be hypercholesterolaemia in this condition?

what signs may be seen?

A

cholesterol in bile leaks out

xanthelasma + xanthomata

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10
Q

which vitamins need supplementing?

why these vitamins?

A

ADEK

fat-soluble vitamins

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11
Q

what happens to fat absorption in late disease?

why?

A

fat malabsorption

less bile going to gut

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12
Q

what is classic presentation?

A

MIDDLE-AGED FEMALE WITH ITCHING AND FATIGUE.

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13
Q

which other autoimmune condition is associated with primary biliary cholangitis?
- the most common one

classic features of this disease?
-2

A

Sjogren’s syndrome

Dry eyes + dry mouth

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14
Q

which age is a risk factor?

A

45-60 years old

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15
Q

which gender is a risk factor?

A

Female

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16
Q

which lifestyle factor is a risk factor?

A

Smoking

17
Q

which type of infection is a risk factor?

A

Chronic UTI

18
Q

two most common symptoms?

A

Pruritus

Fatigue

19
Q

list all symptoms?

-8

A
Pruritus 
Fatigue 
Weight loss 
Dry eyes + dry mouth
Obstructive jaundice symptoms - Yellow skin, pale stools, dark urine
20
Q

which sign might be seen on the face area in late disease?

-2

A

Yellow sclera

xanthelasma

21
Q

which sign might you be able to palpate in the abdominal area?

A

hepatosplenomegaly

22
Q

which sign might you see in fingers specifically?

A

Clubbing

23
Q

which signs might be seen in the skin?
-3

causes of this?

A

Skin hyperpigmentation - high melanin
xanthelasma - cholesterol builds up in skin
jaundice - high bilirubin

24
Q

1st line Ix?

if this is negative what is the second line Ix?

A

Antimicrobial antibodies (AMA)

Antinuclear antibodies (ANA)

25
Q

LFT results?

A

↑↑↑ALP ↑↑↑GGT ↑Bilirubin

↑AST ↑ALT

26
Q

why would you do US?

A

exclude gallstones

27
Q

1st line Mx?

-3 things

A

Ursodeoxycholic acid + ADEK supplement + cholestyramine

28
Q

what do you give to stop pruritus?

what group of drugs does this belong in?

basic effect of this drug?

A

Cholestyramine

bile acid sequestrant

↓bile acids and ↓cholesterol

29
Q

pt in end stage disease and drugs have not worked.

2nd line Mx?

A

Liver transplant

30
Q

which secondary bile acid is given as a treatment for primary biliary cholangitis?

how does this drug help?

A

Ursodeoxycholic acid

stops apoptosis in the bile ducts

31
Q

pt has primary biliary cholangitis.

what happens to there cholesterol levels as disease progresses?

A

increased cholesterol

32
Q

pt has primary biliary cholangitis.

which type of jaundice might occur in later disease stages?

symptoms of this?
-3

A

obstructive jaundice

Yellow skin, pale stools, dark urine

33
Q

describe pathophysiology of primary biliary cholangitis?

A

Inflammation & lymphocytes destroy intrahepatic bile ducts - leads to cholestasis –> bile builds in liver –> bile leaks into circulation –> liver more inflamed + damaged –> cirrhosis, fibrosis.

34
Q

which serious complication can happen with primary biliary cholangitis that causes ascites and bleeding?

A

portal HTN

35
Q

in portal HTN where does the bleeding happen?

A

variceal bleeding