摘要BACKGROUND: Sclerosing cholangitis (SC) is a chronic cho-lestatic hepatobiliary disease with uncertain long-term prog-nosis in pediatric patients. This study aimed to evaluate long-term results in children with SC according to the types of SC. <br> METHODS: We retrospectively followed up 25 children with SC over a period of 4-17 years (median 12). The diagnosis of SC was based on biochemical, histological and cholangio-graphic ifndings. Patients fuliflling diagnostic criteria for probable or deifnite autoimmune hepatitis at the time of diag-nosis were deifned as having autoimmune sclerosing cholangi-tis (ASC); other patients were included in a group of primary sclerosing cholangitis (PSC). The incidence of the following complications was studied: obstructive cholangitis, portal hy-pertension, advanced liver disease and death associated with the primary disease. <br> RESULTS: Fourteen (56%) patients had PSC and 11 (44%) had ASC. Patients with ASC were signiifcantly younger at the time of diagnosis (12.3 vs 15.4 years,P=0.032) and had higher IgG levels (22.7 vs 17.2 g/L,P=0.003). The mentioned compli-cations occurred in 4 (16%) patients with SC, exclusively in the PSC group: one patient died from colorectal cancer, one patient underwent liver transplantation and two patients, in whom severe bile duct stenosis was present at diagnosis, were endoscopically treated for acute cholangitis. Furthermore, two&nbsp;other children with ASC and 2 children with PSC had elevated aminotransferase levels. The 10-year overall survival was 95.8% in all patients, 100% in patients without complicated liver disease, and 75.0% in patients with complications. <br> CONCLUSION: In children, ASC is a frequent type of SC, whose prognosis may be better than that in patients with PSC.