Primary sclerosing cholangitis
- Primary sclerosing cholangitis (PSC) is a chronic cholestatic liver
disease of unknown causes characterised by inflammation and fibrosis
of the biliary tree.
- The mean age at diagnosis is 40 years and men are
affected twice as often as women. There is a reported annual incidence
of PSC of 0.9 to 1.31 in 100,000 and point prevalence of 8.5 to 13.6 in
100,000.
- The onset of PSC is usually insidious and many patients are
asymptomatic at diagnosis or have mild symptoms only such as fatigue,
abdominal discomfort and pruritus (itching). In late stages, splenomegaly (enlargment of the spleen) and
jaundice may be a feature.
- In most, the disease progresses to cirrhosis
and liver failure. Cholangiocarcinoma (cancer of the bile ducts) develops in 8 to 30% of patients.
- PSC is thought to be immune mediated and is often associated with
inflammatory bowel disease, especially ulcerative colitis.
- The disease
is diagnosed on typical cholangiographic and histological findings (X-ray examination and biopsy by endoscopic way) and
after exclusion of secondary sclerosing cholangitis.
- Median survival has
been estimated to be 12 years from diagnosis in symptomatic patients.
Patients who are asymptomatic at diagnosis, the majority of whom will
develop progressive disease, have a survival rate greater than 70% at 16
years after diagnosis.
- Liver transplantation remains the only effective
therapeutic option for patients with end-stage liver disease from PSC,
although high dose ursodeoxycholic acid (Ursodiol, Actigall, Urso Forte, Urso 250) may have a beneficial effect.
- Source: Dr Roger CHAPMAN MD; Dr Joy WORTHINGTON MD. Orphanet