Primary Sclerosing Cholangitis

Question 1

Define primary sclerosing cholangitis

Answer 1

Chronic progressive cholestatic liver disease characterised by inflammation and fibrosis of the intrahepatic and/or extrahepatic bile ducts that results in diffuse multi-focal stricture formation

Question 2

Aetiology and risk factors of primary sclerosing chlolangitis

Answer 2

Inflammation of medium and large-sized bile ducts → Fibrosis → multi-focal stricturing of ducts → stenosis → obstruction → Progressive fibrosis → Smaller ducts (= Ductopenia) and Bile stasis (= Cholestasis)

Associated with:
Autoimmune disease
Inflammatory bowel disease (80% PSC have UC)
HLA haplotypes

RF:
Male
Ulcerative colitis
FHx

Question 3

Symptoms and signs of primary sclerosing cholangitis

Answer 3

Abdominal Pain (RUQ or epigastric)
Pruritus: generalised, intermittent
Jaundice
Fatigue
Weight loss (due to fat malabsorption)
Fever
Steatorrhoea

portal HTN → splenomegaly, aascites
Encephalopathy signs

Question 4

Investigations for primary sclerosing cholangitis

Answer 4

Urine copper concentration: ?Wilson’s

LFTs: raised GGT/ALP (+ ALT/AST), raised bilirubin
Clotting: PT normal or prolonged
Albumin: normal or low
FBC
Autoimmune serology: P-ANCA positive (AMA, ANA, ASMA -)
If: IgG/IgM elevated
IgG4: normal/elevated
hepatic opper concentration: elevated
Caeruloplasmin: elevated

Abdo US: Abnormal bile duct ± cirrhotic liver, ascites, splenomegaly
MRCP: multi-focal strictures and dilatations (“Beaded” appearance)
ERCP: “Beaded” appearance
CT abdomen: bile duct thickening
Liver biopsy (rare): periductal fibrosis → onion skin appearance

Question 5

Management for primary sclerosing cholangitis

Answer 5

Conservative
- Lifestyle changes such as alcohol avoidance, heat avoidance, emollients
- DEXA at diagnosis and 2-4 year intervals (osteoporosis)
- Ileocolonoscopy at diagnosis + repeat every 5 years

Medical
- Symptom management e.g. Pruritus → Antihistamines → cholestyramine
- Supplementation of fat soluble vitamins (A,D,E,K)
- Consider Ursodeoxycholic acid

Surgical
- Strictures may be dilated via ERCP
- Liver transplantation may be indicated in cases complicated by chronic liver disease and/or hepatobiliary malignancies.

Question 6

Complications of primary sclerosing cholangitis

Answer 6

cholangiocarcinoma (in 10%)
increased risk of colorectal cancer