Many patients suffering from primary sclerosing cholangitis (PSC) have no s
ymptoms - or rather unspecific symptoms. Most patients have a cholestatic b
iochemical profile, but a specific blood test for the diagnosis of PSC is l
acking. The diagnostic test (endoscopic retrograde cholangiography (ERC)) i
s an invasive procedure with potential complications. Also, in some patient
s the diagnosis of PSC is not easy, even when ERC has been performed. There
fore true incidence and prevalence data on PSC are extremely few. Neverthel
ess, it seems well established that the epidemiology of PSC is not the same
all over the world. PSC is most often seen in Northern Europe. In this par
t of the world PSC is also associated with inflammatory bowel disease in mo
st cases - and in the Nordic Countries PSC has become the primary indicatio
n for hepatic transplantation.