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
Why do DEXA scans every 5 years in PBC
Osteoporosis - vit D malabsorption -
26
PBC on hisotlogy
Bauder lesions Small intrahepatic ducts Classic granulomatous disease
27
Classic presentation of PBC
Itching in middle aged woman
28
Ass diseases with PBC
Sjrogen - 80% patients RA Systemic sclerosis Thyroid disease
29
Early symptoms PBC
Raised ALP Fatigue Pruritis
30
PBC symtpoms mid stage
Cholestatic jaundice Hyperpigmentation esp over pressure points RUQ pain Xanthelasma, xanthomata May progress to liver failure
31
Why are Anti-mitochondrial antibodies (AMA) M2 subtype so good for diagnosis of PBC
Present in 98% of patients - highly specific
32
Antibodies in PBC
AMA subtype M2 Smooth muscle antibodies Raised serum IgM
33
First line for pruritis
cholestyramine
34
INdications for liver transplant
Bilirubin >100
35
What supplement is it imporatnt to give in PBC
Fat soluble vitamins
35
Complications of PBC on bone
Osteomalacia and osteoporosis