Primary Biliary vs Primary Sclerosing Cholangitis Flashcards
What is Primary Biliary Cholangitis
Autoimmune disorder against hepatocytes
Causes biliary duct degeneration and granuloma.
Intrahepatic small bile duct destruction
What is Primary Sclerosing Cholangitis
Intra and extrahepatic bile duct inflammation and sclerosis
Cholestatic liver disease characterised by fibrosing inflammatory destruction of both intra and extrahepatic bile ducts
Epidemiology of Primary Biliary Cholangitis
40-60 mainly
Women X9 risk
Epidemiology of Primary Sclerosing Cholangitis
40
Men X2 risk
Clinical signs and symptoms of Primary Biliary Cholangitis
Fatigue Pruritus Xanthelasma Discomfort over liver Blotchiness of hands Pale stools Nausea Go off alcohol/cigarettes Hepatomegaly
What is Pruritus
Severe itching of the skin
What is Xanthelasma
Yellow eye patches
Clinical signs and symptoms of Primary Sclerosing Cholangitis
Fatigue
Pruritus
Jaundice
Investigations of Primary Biliary Cholangitis
+AMA (Anti-mitochondrial antibody - in blood)
Raised IgM
Abnormal LFTs, USS (ultrasound scan), MRI, Biopsy
Investigations of Primary Sclerosing Cholangitis
MRCP or endoscopic retrograde cholangiography
ANCA (Anti-neutrophil cytoplasmic antibody)
Abnormal LFTs, USS, MRI, liver biopsy
Treatment of Primary Biliary Cholangitis
- Lower alcohol
- Itch: Cholestyramine OR antihistamines, opiate antagonists, rifampicin, UV, plasmapheresis
- Fatigue: Modafinil, Sertraline
- Disease modifier: Ursodeoxycholic acid, Methotrexate
- Transplant
Treatment of Primary Sclerosing Cholangitis
- Avoid alcohol
- Itch: Cholestyramine and Vitamin supplement
- Transplant
Associations and Complications of Primary Biliary Cholangitis
Associations: Other autoimmune e.g. RA, Sjorgen’s
Complications: Cirrhosis, Liver failure, Osteoporosis, Increased risk of HCC (Hepatocellular carcinoma)
Associations and Complications of Primary Sclerosing Cholangitis
Associations: IBD, especially Ulcerative Colitis (80%)
Complications: Liver failure, cirrhosis, Vit ADEK deficiency, increased risk of cholangiocarcinoma
Raised globulins of PBC and PSC and AIH (hepatitis)
PBC - IgM
PSC - Variable
AIH - IgG