FN: Primary Sclerosing cholangitis

Question 1

Pathophysiology

Answer 1

􏰀 Inflammation, fibrosis and strictures and intra- and
extra-hepatic ducts.
􏰀 Chronic biliary obstruction → 2O biliary cirrhosis → liver
failure

Question 2

Epi

Answer 2

􏰀 Age: 30-50yrs

􏰀 Sex: M>F = 2:1

Question 3

Presentation

Answer 3

􏰀 May be asypmto and Dx incidentally (↑ALP)

Question 4

Symptoms

Answer 4

􏰀 Jaundice
􏰀 Pruritus and fatigue
􏰀 Abdo pain

Question 5

Signs

Answer 5

􏰀 Jaundice: dark urine, pale stools

􏰀 HSM

Question 6

Complications

Answer 6

􏰀 Bacterial cholangitis

􏰀 ↑ Cholangiocarcinoma 􏰀 ↑ CRC

Question 7

Associated Diseases

Answer 7

􏰀 UC
􏰁 3 % o f t h o s e ̄c U C h a v e P S C
􏰁 80-100% of those ̄c PSC have UC/Crohn’s 􏰀 Crohn’s (much rarer)
􏰀 AIH 􏰀 HIV

Question 8

Ix

Answer 8

􏰀 LFTs: ↑ALP initially, then ↑BR
􏰀 Abs: pANCA (80%), ANA and SMA may be +ve 􏰀 ERCP/MRCP: “beaded” appearance of ducts
􏰀 Biopsy: fibrous, obliterative cholangitis

Question 9

Rx overall

Answer 9

No curative medical therapy: transplant needed

Question 10

Symptomatic Rx

Answer 10

􏰁 Pruritus: colestyramine, naltrexone

􏰁 Diarrhoea: codeine phosphate

Question 11

Specific treatment

Answer 11

􏰁 ADEK vitamins
􏰁 Ursodeoxycholic acid improves cholestasis only 􏰁 Abx for cholangitis
􏰁 Endoscopic stenting for dominant strictures

Question 12

Screening

Answer 12

􏰁 Cholangiocarcinoma: US + Ca19-9 􏰁 CRC: colonoscopy

Question 13

Transplant recurrence

Answer 13

􏰁 Recurrence occurs in 30%