Primary sclerosing cholangitis (PSC): clinical, laboratory and survival analysis in children and adults

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Abstract
Primary sclerosing cholangitis (PSC) is an uncommon disorder, rarely diagnosed in children, moreover, data on its natural history and survival are still lacking. The study was undertaken to compare clinical, laboratory and survival rates in two series of PSC: one in a pediatric group (group A) and the other in an adult population (group B). Group A included 9 patients (5 males, 4 females, mean age 10 yrs, range 7-15); group B included 28 patients (19 males, 9 females, mean age 32 years, range 19-60). The mean follow-up was 5.2 years in group A and 6.9 years in group B (range 1-14 years). ERCP and colonoscopy were performed in each case. Survival was analyzed using the Kaplan-Meier method. At presentation children showed significantly higher levels of IgG and AST compared to adults (p<0.05); moreover, interface hepatitis occurred in 50% of children and in 14.2% in adults (p=ns). During follow-up the following major events occurred: oesophageal bleeding (n=2) in group A; progressive liver failure (n=6), cholangiocarcinoma (n=3), colonic cancer (n=1) in group B. Liver transplantation (OLTx) was performed in 4 adults (one died after a retransplantation). No deaths were observed in children. The Kaplan-Meier curve in adults shows a 65% rate of survival at 10 years. The present findings on PSC suggest a more severe activity of the disease in children than in adults at presentation; nonetheless, the prognosis seems to be better in children than in adults. The Mayo score prognostic index does not predict the development of liver/colonic cancer. A poor outcome (defined as death or being listed for OLTx) only occurred in adults.