primary biliary cholangitis

Question 1

define primary biliary cholangitis

Answer 1

• autoimmune destruction of bile ducts within liver
• leads to fibrosis, cirrhosis and live failure

Question 2

what is the pathology behind primary biliary cholangitis?

Answer 2

1. autoantibodies cause interlobular bile duct damage
2. this is chronic autoimmune granulomatous inflammation
3. causes obstruction of the outflow of bile aka cholestasis
4. bile leaks out into blood and other liver cells
5. leads to fibrosis, cirrhosis, portal hypertension and infection

Question 3

what are the risk factors for primary biliary cholangitis?

Answer 3

-female
-40-50
-other autoimmune diseases
-smoking
-rheumatoid conditions
-family history

Question 4

what is the other treatment for primary biliary cholangitis?

Answer 4

• vit ADEK supplements
• immunosupressants
• cholestyramine- for priritus

Question 5

what is the first line of treatment for primary biliary cholangitis?

Answer 5

• ursodeoxycholic acid - reduces cholestasis

Question 6

what are the initial symptoms for primary biliary cholangitis?

Answer 6

• initially asymptomatic- routine test shows increased AMA

Question 7

what are the early symptoms for primary biliary cholangitis?

Answer 7

-pruritus
-fatigue

Question 8

what are the later symptoms for primary biliary cholangitis?

Answer 8

• jaundice
• -hepatomegaly
• Xanthelasma- yellow in inner corner of eyes or near nose

Question 9

what are the other symptoms for primary biliary cholangitis?

Answer 9

• abdominal pain
• pale stools
• signs of cirrhosis and LF

Question 10

how is primary biliary cholangitis diagnosed?

Answer 10

1. LFTs
2. Serology
3. Rule out acute Hep
4. ultrasound
5. Liver biopsy

Question 11

in primary biliary cholangitis what would LFTs look like?

Answer 11

-high ALP
-high conjugated bilirubin
-high GGT
-low albumin

Question 12

in primary biliary cholangitis what would serology look like?

Answer 12

• 90% have AMA antibodies
  -35% have ANA antibodies

Question 13

in primary biliary cholangitis when ruling out acute hep, what are you looking for?

Answer 13

• HepBsAg, HCVab

Question 14

in primary biliary cholangitis why is an ultrasound done?

Answer 14

• to exclude extra hepatic cholestasis

Question 15

in primary biliary cholangitis what are you looking for in a liver biopsy?

Answer 15

• portal tract infiltrate
• 40% granulomatous
• portal tract fibrosis