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Small-duct primary sclerosing cholangitis

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Abstract

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 largeduct 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.

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Authors and Affiliations

  1. Department of Internal Medicine, Section of Gastroenterology and Hepatology, Sahlgrenska University Hospital, Med pol II, SE-413 45, Gothenburg, Sweden

    Einar Björnsson

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  1. Einar Björnsson
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Correspondence to Einar Björnsson.

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Björnsson, E. Small-duct primary sclerosing cholangitis. Curr Gastroenterol Rep 11, 37–41 (2009). https://doi.org/10.1007/s11894-009-0006-6

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