Indolent disease. Ivor

[Guest guest]

> But in the UK 80% of us will not need a Ltx.

Hi Ivor;

If this is the case, then it is not well articulated in the UK

publications on PSC. If I am reading the UK PSC literature

correctly, they are saying that the vast majority of asymptomatic

patients will develop progressive disease, and in most the disease

progresses to cirrhosis and liver failure. This does not seem to fit

with your statistic of 80% never needing a transplant?

Orphanet J Rare Dis. 2006 Oct 24;1:41.

Primary sclerosing cholangitis.

Worthington J, Chapman R.

Department of Gastroenterology, Radcliffe Hospital, Headington,

Oxford, OX3 9DU, UK. joyworthington@....

ABSTRACT: Primary sclerosing cholangitis (PSC) is a chronic

cholestatic liver disease of unknown aetiology 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 0.9-1.31/100,000 and

point prevalence of 8.5-13.6/100,000. 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

In late stages, splenomegaly and jaundice may be a feature. In most,

the disease progresses to cirrhosis and liver failure.

Cholangiocarcinoma develops in 8-30% 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 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 may have a

beneficial effect. PMID: 17062136.

Best regards,

Dave

(father of (21); PSC 07/03; UC 08/03)