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Small-duct PSC

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Curr Gastroenterol Rep. 2009 Feb;11(1):37-41.

Small-duct primary sclerosing cholangitis.

Björnsson E

Department of Internal Medicine, Section of Gastroenterology and

Hepatology, Sahlgrenska University Hospital, Med pol II, SE-413 45

Gothenburg, Sweden. einar.bjornsson@....

Patients with cholestatic liver tests and typical histologic features

of primary sclerosing cholangitis (PSC) but a normal cholangiogram

have been identified as having small-duct PSC. This subgroup of PSC

has been less well characterized than the classic large-duct form.

Some patients characterized as having small-duct PSC develop

cholangiographic features of PSC during follow-up. Three papers

published in 2002 on small-duct PSC patients suggested a better

prognosis in patients with small-duct PSC than in those with large-

duct PSC. However, these studies included a limited number of

patients and had a short follow-up. A combined cohort of these

patients with a prolonged follow-up recently confirmed these previous

observations. However, some patients will suffer from liver-related

mortality or will need to undergo liver transplantation. Recurrence

of small-duct PSC after liver transplantation has been reported.

Cholangiocarcinoma, the most feared complication of PSC, has not been

described in a patient with small-duct PSC without progression first

to large-duct PSC. PMID: 19166657.

Dave

(father of (23); PSC 07/03; UC 08/03)

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