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. 2002 May;36(5):586-9.
doi: 10.1016/s0168-8278(02)00036-3.

Natural history and outcome in 32 Swedish patients with small duct primary sclerosing cholangitis (PSC)

Ulrika Broomé  1 , Hans Glaumann, Eva Lindstöm, Lars Lööf, Sven Almer, Hanne Prytz, Hanna Sandberg-Gertzén, Stefan Lindgren, Frans-Thomas Fork, Gunnar Järnerot, Rolf Olsson
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Natural history and outcome in 32 Swedish patients with small duct primary sclerosing cholangitis (PSC)

Ulrika Broomé et al. J Hepatol. 2002 May.

Abstract

Background/aims: This study aims at describing the natural history and outcome of small duct primary sclerosing cholangitis (PSC).

Methods: Thirty-two patients with small duct PSC were studied. The average time taken for diagnosis was 69 (1-168) months. The median follow-up time was 63 (1-194) months.

Results: All patients including one who underwent liver transplantation because of end-stage liver disease and hepatocellular carcinoma were alive at follow-up. None developed cholangiocarcinoma. In 27 patients repeated cholangiographic examinations were done after a median time of 72 (12-192) months from first ERCP. Four developed features of large duct PSC.

Conclusions: Small duct PSC rarely progresses to large bile duct PSC and it seems to have a benign course in most patients and no development of cholangiocarcinoma was found.

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