FN: Primary Sclerosing cholangitis Flashcards Preview

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Flashcards in FN: Primary Sclerosing cholangitis Deck (13):
1

Pathophysiology

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

2

Epi

􏰀 Age: 30-50yrs
􏰀 Sex: M>F = 2:1

3

Presentation

􏰀 May be asypmto and Dx incidentally (↑ALP)

4

Symptoms

􏰀 Jaundice
􏰀 Pruritus and fatigue
􏰀 Abdo pain

5

Signs

􏰀 Jaundice: dark urine, pale stools
􏰀 HSM

6

Complications

􏰀 Bacterial cholangitis
􏰀 ↑ Cholangiocarcinoma 􏰀 ↑ CRC

7

Associated Diseases

􏰀 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

8

Ix

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

9

Rx overall

No curative medical therapy: transplant needed

10

Symptomatic Rx

􏰁 Pruritus: colestyramine, naltrexone
􏰁 Diarrhoea: codeine phosphate

11

Specific treatment

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

12

Screening

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

13

Transplant recurrence

􏰁 Recurrence occurs in 30%

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