Primary biliary cholangitis Flashcards
What is PBC
Rare, treatable, chronic inflammatory condition that effect young people
What biochem suggests PBC
AMA + raised ALP
Who is more susceptible to PBC
Female, middle aged
Genetic, triggers from environement, hormones
Hyperferritenemia
Pathology of PBC
Immune insult
Bile duct damage
Inflammation
Fibrosis/cirrhosis
Symptoms of PBC
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
Diagnostic criteria for PBC
Abnormal LFTs - cholestatic, raised ALP
+AMA subtype M2 antibodies
Compatible histology - liver biopsy
2 = probable 3 = definitie
Investigations for PBC
Exclude drug, viral and metabolic causes
US - biliary obstruction
Liver biopsy
Signs of PBC on liver biopsy
Florid duct lesions - granulomas + duct obliteration (ductopenia_
What does ductopenia on biopsy mean for patinet
a high risk marker on biopsy for agressive disease - need trnasplant
First line treatment PBC
Ursodeoxycholic acid - UDCA
What is UDCA and how used
Hydrophilic bile acid
13-15mg/kg/day at night
Response defined by ALP
What ALP means need to up UDCA dose
> 200
Side effects of UDCA
Weight gain, hair thinning, GI side effects
Add ins with UDCA
Fibrates
Obeticholic acid
Liver transplantation
Fibrates what are and do
Anti cholesterol drug
improve LFTs in PBC
Why need to monitor fibrates and how
Risk of liver injury - raise ALT -> hepatits
Fortnightly LFTs and U+Es
Do antihistamines work in cholestasis
Often completely ineffecive
What check when itch
Ferritin - low can cause an itch
Itch control ladder management
1- Bile salt sequestrants - cholestyreine or cholecefalam
2 - rifampacin
3- gabapentin
4-naltrexone
5- sertraline
6 - PUVA
7 - liver transplant
What need to do when taking UDCA and bile sequestrants eg cholecysterine
Separate by 4 hours as drug interactions cancels both effects
What need to monitor with rifampacin
2 weekly blood tests - acute liver injury monitor
Orange secretion warn
What to investigate to potentially treat with fatigue
Anaemia
Thyroid disease
Autonomic dysfunction
OSA
itch
Monitoring in PBC
Review symptoms
DEXA every 5 years
Cirrhosis evidence - US, fibroscan, bloods (low albumin, platelets, jaundice)
HCC + varices screening if cirrhotic
HCC screening what do
AFP + US 6 monthly
Why do DEXA scans every 5 years in PBC
Osteoporosis - vit D malabsorption -
PBC on hisotlogy
Bauder lesions
Small intrahepatic ducts
Classic granulomatous disease
Classic presentation of PBC
Itching in middle aged woman
Ass diseases with PBC
Sjrogen - 80% patients
RA
Systemic sclerosis
Thyroid disease
Early symptoms PBC
Raised ALP
Fatigue
Pruritis
PBC symtpoms mid stage
Cholestatic jaundice
Hyperpigmentation esp over pressure points
RUQ pain
Xanthelasma, xanthomata
May progress to liver failure
Why are Anti-mitochondrial antibodies (AMA) M2 subtype so good for diagnosis of PBC
Present in 98% of patients - highly specific
Antibodies in PBC
AMA subtype M2
Smooth muscle antibodies
Raised serum IgM
First line for pruritis
cholestyramine
INdications for liver transplant
Bilirubin >100
What supplement is it imporatnt to give in PBC
Fat soluble vitamins
Complications of PBC on bone
Osteomalacia and osteoporosis
