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gi, liver, kidney > Primary biliary cholangitis > Flashcards

Primary biliary cholangitis Flashcards

1
Q

What is PBC

A

Rare, treatable, chronic inflammatory condition that effect young people

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2
Q

What biochem suggests PBC

A

AMA + raised ALP

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3
Q

Who is more susceptible to PBC

A

Female, middle aged
Genetic, triggers from environement, hormones
Hyperferritenemia

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4
Q

Pathology of PBC

A

Immune insult
Bile duct damage
Inflammation
Fibrosis/cirrhosis

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5
Q

Symptoms of PBC

A

Asymptomatic
#Fatigue
Itch - palms o hands, soles of feet. worse at night/hot
Dry eyes + mouth - sjrogens ass
Poor memory
Signs of advanced liver disease

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6
Q

Diagnostic criteria for PBC

A

Abnormal LFTs - cholestatic, raised ALP
+AMA subtype M2 antibodies
Compatible histology - liver biopsy
2 = probable 3 = definitie

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7
Q

Investigations for PBC

A

Exclude drug, viral and metabolic causes
US - biliary obstruction
Liver biopsy

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8
Q

Signs of PBC on liver biopsy

A

Florid duct lesions - granulomas + duct obliteration (ductopenia_

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9
Q

What does ductopenia on biopsy mean for patinet

A

a high risk marker on biopsy for agressive disease - need trnasplant

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10
Q

First line treatment PBC

A

Ursodeoxycholic acid - UDCA

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11
Q

What is UDCA and how used

A

Hydrophilic bile acid
13-15mg/kg/day at night
Response defined by ALP

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12
Q

What ALP means need to up UDCA dose

A

> 200

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13
Q

Side effects of UDCA

A

Weight gain, hair thinning, GI side effects

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14
Q

Add ins with UDCA

A

Fibrates
Obeticholic acid
Liver transplantation

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15
Q

Fibrates what are and do

A

Anti cholesterol drug
improve LFTs in PBC

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16
Q

Why need to monitor fibrates and how

A

Risk of liver injury - raise ALT -> hepatits
Fortnightly LFTs and U+Es

17
Q

Do antihistamines work in cholestasis

A

Often completely ineffecive

18
Q

What check when itch

A

Ferritin - low can cause an itch

19
Q

Itch control ladder management

A

1- Bile salt sequestrants - cholestyreine or cholecefalam
2 - rifampacin
3- gabapentin
4-naltrexone
5- sertraline
6 - PUVA
7 - liver transplant

20
Q

What need to do when taking UDCA and bile sequestrants eg cholecysterine

A

Separate by 4 hours as drug interactions cancels both effects

21
Q

What need to monitor with rifampacin

A

2 weekly blood tests - acute liver injury monitor
Orange secretion warn

22
Q

What to investigate to potentially treat with fatigue

A

Anaemia
Thyroid disease
Autonomic dysfunction
OSA
itch

23
Q

Monitoring in PBC

A

Review symptoms
DEXA every 5 years
Cirrhosis evidence - US, fibroscan, bloods (low albumin, platelets, jaundice)
HCC + varices screening if cirrhotic

24
Q

HCC screening what do

A

AFP + US 6 monthly

25
Q

Why do DEXA scans every 5 years in PBC

A

Osteoporosis - vit D malabsorption -

26
Q

PBC on hisotlogy

A

Bauder lesions
Small intrahepatic ducts
Classic granulomatous disease

27
Q

Classic presentation of PBC

A

Itching in middle aged woman

28
Q

Ass diseases with PBC

A

Sjrogen - 80% patients
RA
Systemic sclerosis
Thyroid disease

29
Q

Early symptoms PBC

A

Raised ALP
Fatigue
Pruritis

30
Q

PBC symtpoms mid stage

A

Cholestatic jaundice
Hyperpigmentation esp over pressure points
RUQ pain
Xanthelasma, xanthomata
May progress to liver failure

31
Q

Why are Anti-mitochondrial antibodies (AMA) M2 subtype so good for diagnosis of PBC

A

Present in 98% of patients - highly specific

32
Q

Antibodies in PBC

A

AMA subtype M2
Smooth muscle antibodies
Raised serum IgM

33
Q

First line for pruritis

A

cholestyramine

34
Q

INdications for liver transplant

A

Bilirubin >100

35
Q

What supplement is it imporatnt to give in PBC

A

Fat soluble vitamins

35
Q

Complications of PBC on bone

A

Osteomalacia and osteoporosis

gi, liver, kidney (50 decks)

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