Autoimmune Hepatobiliary Disease Flashcards
Autoimmune hepatitis (AH):
What is it?
2 ways it presents?
Autoimmune hepatocyte damage
- Signs of ALF and systemic autoimmune symptoms - fever, polyarthritis, malaise, urticaria
- Also present as CLD
Autoimmune hepatitis (AH) - Inv:
What autoantibodies can be done to look for autoimmune disease?
What immunoglobulin is also found in AH?
What is always needed to diagnose?
ANA - anti-nuclear Ab - raised
ASMA - Anti-smooth muscle Ab - raised
Raised IgG
Biopsy
Autoimmune hepatitis (AH):
How is this managed?
Immunosupression - Prednisolone +/- azathioprine
PBC:
What does it stand for?
What is it?
What does it lead to?
Primary Biliary Cholangitis
(Small) INTRAhepatic bile duct destruction
Results in cholestasis causing hepatocyte damage, fibrosis and eventually cirrhosis
PBC - Risk factors:
What sex is it more common in?
Other factors you may ask about in the history?
Women - 10x more common > Mainly middle aged women
PMH
FH of autoimmune disease
PBC - S+S:
Asymptomatic (incidental finding):
What bone disease do they get and why?
What S+S will they get with a build up of bile acids? - 3
They can also get Sjögren’s syndrome. What symptoms will it cause?
PBC prevents the liver from controlling lipid levels. What signs on examination may you see?
Why is cholesterol control no longer the same? - 2
What may happen to their skin?
What will happen to their stool?
There is also hepatomegaly!
Aetiology of bone loss is multifactorial and not entirely understood. Patients with cholestatic liver disease are particularly susceptible to osteoporosis due to the interference of cholestasis with vit D metabolism - can’t absorb FATS
- Itch
- Lethargy
- Eyes yellow - jaundice
- # Eyes dry - sicca
- Xanthelasma
Cholesterol - raised - due to increased synthesis and reduced biliary clearance
- Skin pigmentation due to increased melanin deposition
- Steatorrhoea due to fat malabsorption
PBC - Investigations:
Obviously LFTs
What autoantibody is highly specific for the disease?
What immunoglobulin is also found in PBC?
Why is an abdo USS done?
What is done to confirm PBC?.
Anti-mitochondrial Ab (AMA)
IgM
Both M’s
To exclude focal lesion in obstructing duct
Liver biopsy
PBC - Management:
Lifestyle:
- What should be avoided?
- What should be supplemented?
- What should be monitored for liver function? - 3
- Why is a DEXA scan done?
Reduce alc intake
Supplementation of fat-soluble vitamins (ADEK)
LFTs, PT and albumin
Osteoporosis - due to lack of vit D absorption
PBC - Management - Disease Progression:
UDCA is used to reduce cholesterol absorption from the bowel, therefore, fewer bile acids, less itch. What does it stand for?
What is used to help the itch? - starts with c
Surgical Rx?
If cirrhotic, what 2 pathologies need to be screened?
Ursodeoxycholic acid
Bile acid sequestrats - Cholestyramine - helps body remove bile acids
Transplantation
HCC and varices
PSC:
What does it stand for?
What is it?
What does it lead to?
What is another disease that tends to happen in conjunction with PSC?
Primary Sclerosing Cholangitis
Inflammation of the INTRA + EXTRA hepatic (medium and large) bile ducts.
Blockage and fibrosis which in turn leads to hepatocyte damage and fibrosis
IBD - UC
PSC - S+S:
What sex is it more common in?
Hepatic symptoms - 4
Systemic symptoms - 3
How is it usually found?
Men
RUQ pain
Jaundice
Itch
Hepato/splenomegaly
Fatigue
Weight loss
Fevers
Incidental
PSC - Investigations:
What autoantibody is found in PSC?
What will an abdo USS do?
What is found on MRCP?
ANCA
Show duct changes
Rule out other causes
Multiple strictures with normal or dilated segments (Beading)
(ERCP has a higher risk of introducing infection)
PSC - Management:
Lifestyle:
- What should be avoided to reduce cholangiocarcinoma risk?
- What should be done to screen for cholangiocarcinoma? - 2
- What should be supplemented which may not be absorbed by the GI tract?
- What should be monitored for liver function? - 3
- Why is a DEXA scan done?
Alcohol
CA19-9 and abdo USS
Supplementation of fat-soluble vitamins (ADEK)
LFTs, PT and albumin
Osteoporosis - due to lack of Vit D absorption - also add bisphosphonates and calcium if needed
PSC - Management:
What is used to help the itch? - starts with c
What ERCP can be done for biliary strictures. What can be done in the procedure?
Cholestyramine - helps the body remove bile acids
UDCA not used in PSC
Balloon dilation or stenting
PSC - Complications:
Cholangiocarcinoma
Bacterial cholangitis
