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Pathology
Autoimmune disorder characterised by inflammation and stricture formation of the intra and extra hepatic bile ducts
Aetiology
Autoimmune, higher incidence in men, associated with IBD. Ulcerative colitis has a higher prevalence of disease than Crohn’s.
Signs
Excoriations from pruritus, jaundice if late
Symptoms
Pruritus, lethargy, symptoms of IBD
Investigations
Bloods: LFTs show raised bilirubin and ALP
Auto-Antibody Screen: ANCA positive
Imaging: Ultrasound Abdomen is usually normal, can show bile duct dilatation
Treatment
Medical: ERCP to dilate ducts
Surgical: Liver transplantation
Prognosis
Median survival 12 years from diagnosis
Increased risk of cholangiocarcinoma, colonic carcinoma and cholangitis
Key Facts
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Key Images
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Key References
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