Immune Mediated Liver Disease Flashcards
Autoimmune diseases of the bile duct
Primary Biliary Cholangitis
Primary Sclerosing Cholangitis
Autoimmune diseases of the liver
Autoimmune Hepatitis
What is the main disease affecting the small microscopic bile ducts?
PBC
Features of PBC
More females 9:1 >40 years Small bile duct destruction Autoimmune Raised ALP and GGT Detect anti-mitochondrial antibody in blood (M2 subunit)
Symptoms of PBC
Jaundice - late
Pruritis
Fatigue
What are the main liver enzymes involved with biliary disease?
ALP
Gamma - GGT
How is PBC diagnosed?
AMA + persistent chronic cholestasis and other causes excluded
Biopsy - destruction of small bile ductules + inflammation + sometimes granulomas
Treatment of PBC
Ursodeoxycholic acid( UDCA) - good outcome for 2/3 So.. Obeticholic Acid 2nd line Bezafibrate 3rd line Liver Transplantation
MOA OF UDCA
Ursodeoxycholic acid
Derivate of naturally occurring bile acid
Makes bile flow more freely
Symptom treatment for PBC
Itch = cholestyramine, rifampicin
Fatigue
Bones - osteoporosis in chronic post menopausal women
Features of PSC
M>F
>20s
65% have IBD
Large bile duct sclerosis and loss
Symptoms of PSC
Jaundice - earlier
Pruritus
Cholangitis- infections
Cancer & PSC
Increased risk
- cholangiocarcinoma
- colorectal cancer
Treatment for PSC
- none effective
- UDCA sometimes used
- ERCP for strictures
- AB for infections
- liver transplant for progressive disease is often only option
ERCP
Endoscopic retrograde cholangiopancreatography
- for dominant strictures
- to diagnose cholangiocarcinoma
- balloon dilatation vs. stenting vs. both
- risks = infection, pancreatitis