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

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.