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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.
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. http://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. http://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. http://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.