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Primary Sclerosing Cholangitis

From Mediwikis

Autoimmune disease attacking larger bile ducts leading to inflammation, scarring, strictures and cirrhosis.

Epidemiology

  • Second most common autoimmune liver disease, and it is on the rise
  • M:F ratio is 3:1
  • Average age of 35
  • >80% of patients also have ulcerative colitis

Presentation[1]

  • Jaundice
  • Fatigue
  • Pruritus
  • Cirrhosis
  • Portal hypertension
  • Steatorrhoea
  • Dark urine

Investigations[2]

Two of:

  • ALP > 1.5 x upper limit of normal - cholestatic jaundice
  • Cholangiography showing biliary strictures (MRCP)
  • Liver histology (biopsy) showing fibrosis

Antibodies

  • ANCA
  • Less specific- ANA, ASMA

Other tests

  • FBC, U&Es, Renal Function, Bilirubin
  • Liver enzymes
  • Screen for bowel cancer- 90% overlap

Management

  • Antipruritics
  • Cholestyramine to remove bile acids
  • Antibodies to treat cholangitis
  • KADE vitamin supplements
  • ERCP to open biliary strictures
  • Liver transplant

References

  1. http://www.britishlivertrust.org.uk/liver-information/liver-conditions/primary-sclerosing-cholangitis/
  2. Charatcharoenwitthaya P, Lindor KD (Feb 2006). "Primary sclerosing cholangitis: diagnosis and management". Current Gastroenterology Reports8 (1): 75–82. doi:10.1007/s11894-006-0067-8.PMID 16510038.