primary biliary cholangitis

Question 1

definition

Answer 1

chronic
progressive
autoimmune condition
leading to destruction of small intrahepatic bile ducts

Question 2

epidemiology

Answer 2

rare
>50yrs
women

Question 3

pathology

Answer 3

antimitochondrial antibodies attack small bile ducts
- loss of bile ducts - bile acid retained in liver damaging hepatocytes - fibrosis
- fewer ducts means cholestasis and cirrhosis

Question 4

presentation

Answer 4

jaundice
fatigue
pruritis - itchy skin

Question 5

signs

Answer 5

• xanthelsma - choelsterol deposits around the eyes
• hepatomegaly
• hypercholesterolaemia
• stearrhea
• malabsorption of fat soluble lipids

Question 6

investigations

Answer 6

1st line - raised ALP + GGT

gold standard - positive anti-mitochondrial antibodies PDC-E2-ELISA and cholestatic liver enzymes

Question 7

management

Answer 7

1st line - urodeoxycholic acid - lifelong + steroids
2nd line - obeticholic acid - bile acid analogue

in those w chronic liver disease + >50 bilirubin - liver transplant

Question 8

complications

Answer 8

chronic liver disease
cirrhosis

Question 9

associated with

Answer 9

sjogrens
rheumatoid arthiritis
hepatocellular carcinoma
IBS