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RitvaKoskela

Primary Sclerosing Cholangitis

Essentials

  • Sclerosing cholangitis is a chronic, fibrotizing and constrictive inflammation of the biliary ducts that progresses slowly and leads to cholestatic liver damage, to cirrhosis and in some patients to cholangiocarcinoma
  • E.g. in the Nordic countries, the prevalence of sclerosing cholangitis is particularly high and it is the most common indication for liver transplantation.

Epidemiology

Aetiology

  • 60-80% of the cases are associated with inflammatory bowel diseases (IBD), especially with ulcerative colitis. Respectively, 2-8% of patients with ulcerative colitis in turn are diagnosed with sclerosing cholangitis. Ehttp://www.dynamed.com/condition/primary-sclerosing-cholangitis-psc#ASSOCIATED_CONDITIONS
  • HLA associations, concomitant other autoimmune diseases and circulating antibodies suggest an autoimmune aetiology.
    • No specific antibodies (pANCA, antinuclear or anti-smooth muscle antibodies)

Symptoms, signs and diagnosis

  • Most patients are asymptomatic at the time of diagnosis; abnormal liver function tests (alkaline phosphatase) are detected incidentally. Ehttp://www.dynamed.com/condition/primary-sclerosing-cholangitis-psc#GUID-5BEB3886-A934-4685-9A2D-C4695385661B
  • Investigations
  • Cholangitis with fever is possible. Weight loss and jaundice are symptoms of a late phase.
  • Diagnosis is based on abnormal findings on the examination of the biliary tract. Ehttp://www.dynamed.com/condition/primary-sclerosing-cholangitis-psc#GUID-433BE2CD-FCE2-4509-A04C-E33F85DBA35E
    • MR cholangiography is used for screening.
    • Endoscopic retrograde cholangiography (ERCP) is the "golden standard": confirmation of the diagnosis, samples from the biliary tract, concurrent dilatation and stenting of the biliary ducts if needed.
    • Liver biopsy: in case of non-specific histology, in the differential diagnostics of diseases of the small biliary ducts or other chronic hepatitides, gradation of the inflammation and fibrosis
  • Colonoscopy to detect silent colitis is indicated in all patients.
  • Autoimmune cholangitis is a rarer diagnostic alternative.
    • The patient often has other autoimmune diseases; IBD in less than 10%
    • Diagnosis: MRI; serum IgG4 increased; typical histology in the papilla/bile ducts, IgG4-positive plasma cells
    • Good response to glucocorticoid therapy

Complications

  • Strictures of the biliary tract
  • Increased risk for gallstones and cancer of the gallbladder.
  • Cholangiocarcinoma develops in 10-20% of the patients.
  • Cirrhosis develops at the end stage of the disease.

Treatment

Follow-up

Related Keywords

ATC Code:

C10AC01

J04AB02

J01MA01

J01MA02

J01MA06

J01MA12

J01MA14

J01MA23

A05AA02

J01XD01

Primary/Secondary Keywords