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Primary Biliary Cirrhosis (Treatment (Diarrhoea: oral codeine phosphate,…
Primary Biliary Cirrhosis
Definition
Cirrhosis that occurs from cholestasis due to damaged interlobular bile ducts from chronic autoimmune granulomatous inflammation
Aetiology
Genetic susceptibility
Antimitochondrial antibodies are the hallmark
Unknown environmental triggers
Risk Factors
Smoking
Other autoimmune disease
Recurrent UTIs
Family history
Epidemiology
F:M = 9:1
Typical age at presentation = 50 years
Prevalence: 4/100,000
Signs
Xanthomata
Pruritus + jaundice
Hepatosplenomegaly
Complications
Malabsorption of fat soluble vitamins (A, D, E, K) due to cholestasis and decreased bilirubin in gut lumen
Osteomalacia
Osteoporosis
Hepatocellular carcinoma
Investigations
In late disease: raised bilirubin, low albumin, raised prothrombin time
AMA M2 subtype +ve
Bloods: high alk phos, high AST + ALT
Raised Igs
Ultrasound to exclude extrahepatic cholestasis
Treatment
Diarrhoea: oral codeine phosphate
Pruritus: oral rifampicin
Osteoporosis prevention
Vitamin A, D, K
Ursodeoxycholic acid
Liver transplant for end stage disease
Prognosis: once jaundice develops, <2 years without transplant