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.
Presentation:
- 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...
