Friday, 19 November 2010

MRCP revision battle 54.1: Primary sclerosing cholangitis

Today is in pairs: a pair of gastro battles, a pair of cardiology battles, a pair of haematology battles then a strange inherted disorder to finish on....

MRCP revision battle 54.1: Primary sclerosing cholangitis
MRCP revision battle 54.2: Jaundice
MRCP revision battle 54.3: Patent ductus arteriosus
MRCP revision battle 54.4: Cardiac axis
MRCP revision battle 54.5: Haemolysis
MRCP revision battle 54.6: Leukoerythroblastic change
MRCP revision battle 54.7: Gaucher's disease

MRCP revision battle 54.1: Primary sclerosing cholangitis

Primary sclerosing cholangitis is a condition of unknown aetiology in which there is inflammation and fibrosis of bile ducts.

  • patients may be asymptomatic with an incidental finding of raised ALP
  • may present as:
    • jaundice
    • pruritus
    • abdo pain
    • fatigue

Diseases associated with primary sclerosing cholangitis include:
  • IBD (UC>crohns; 70% of patients with PSC have UC but only 5% of patients with UC have PSC)
  • HIV
  • HLA A1, B8, DR3

Males are more frequently affected than females.

Diagnosis is by:
  • ERCP - shows strictures of the biliary tree and a 'beaded' appearence
  • liver biopsy - fibrous, obliterative cholangitis
  • ANA, SMA and ANCA may be positive

Treatment is:
  • colestyramine for pruritis
  • ursodeoxycholic acid to improve cholestatis
  • ?stenting of strictures 
  • ?liver transplant

Complications include:
  • decreased absorption of fat-soluble vitamins A,D,E and K
    • clotting abnormalities and easy bruising
    • osteoporosis
  • cholangiocarcinoma (10-30%)
  • bacterial cholangitis
  • liver cirrhosis
    • portal hypertension

Survival tends to be around 10 yrs

As an aside, the other 2 conditions associated with cholangiocarcinoma are:
  • liver flukes
  • Caroli's disease = dilation of hepatic ducts

Into the yellow...