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Pathology

Increased incidence in patients with PSC 
Usually patients are asymptomatic until they become jaundiced due to obstruction 

Aetiology

Jaundice, pruritus, weight loss, abdominal pain

Signs

Painless jaundice and palpable gall bladder

Symptoms

Jaundice, pruritus, weight loss, abdominal pain

Investigations

Bloods: LFTs show an increased bilirubin and ALP
Tumour Markers: CA 19-9 and CEA may be raised 
Imaging: Ultrasound Abdomen or MRCP shows dilated bile ducts. ERCP allows for 
tissue biopsy

Treatment


Medical: ERCP to insert stent in bile duct and relieve Obstruction 
Surgical: Resection is rarely possible. Radical hepatectomy and bile duct excision is 
curative. Liver transplantation.

Prognosis

Poor, frequently due to late presentation. 
2-8 month survival if treated palliatively.
Infection of biliary tree and obstruction 

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