Small-duct primary sclerosing cholangitis.
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.
Björnsson
Einar
E
Department of Internal Medicine, Section of Gastroenterology and Hepatology, Sahlgrenska University Hospital, Med pol II, SE-413 45 Gothenburg, Sweden. einar.bjornsson@medic.gu.se
D001650:Bile Duct Neoplasms / Q000150:complications / Q000473:pathology; D001653:Bile Ducts, Intrahepatic / Q000473:pathology / Q000601:surgery; D018281:Cholangiocarcinoma / Q000150:complications; D015209:Cholangitis, Sclerosing / Q000150:complications / Q000473:pathology / Q000628:therapy; D018450:Disease Progression; D019693:Hepatitis, Autoimmune / Q000150:complications; D006801:Humans; D015212:Inflammatory Bowel Diseases / Q000150:complications; D008111:Liver Function Tests; D016031:Liver Transplantation; D011379:Prognosis; D012008:Recurrence; D012307:Risk Factors