The Natural History of Primary Sclerosing Cholangitis in Children

Abstract

In this largest reported pediatric PSC cohort, liver transplantation rate at 10 years was lower than that reported in adults. ASC and PSC had similar biochemical abnormalities and degree of fibrosis at diagnosis. PSC that developed after IBD diagnosis had a milder course, possibly reflecting earlier disease detection or milder phenotype.

The Riddle of Juvenile Sclerosing Cholangitis

“…All retrospective studies recognize a form of SC with strong autoimmune features, but while in some this condition is reported to respond favourably to immunosuppression, having a better prognosis than PSC, in others the prognosis of AIH/PSC overlap is reported to be severe and not ameliorated by immunosuppressive treatment or similar to that of PSC irrespective of treatment . In agreement with the current study by Deneau et al, small‐duct SC is reported to have a more favorable prognosis …”

The natural history of primary sclerosing cholangitis in 781 children: A multicenter, international collaboration

“…PSC–IBD carried a more favorable prognosis in our cohort as well. PSC patients with IBD had a lower rate of cirrhosis and higher survival with native liver in children . Adult PSC–IBD patients with a Crohn phenotype did markedly better in an adult series, though patients with ulcerative colitis did marginally worse .…”

“…Primary sclerosing cholangitis (PSC) is a rare disease in pediatric patients, with an incidence of 0.2 cases per 100,000 children . Existing descriptions of the natural history and characteristics of PSC are largely from small, single‐center case series with limited long‐term follow‐up . Objective predictors of patient outcome are lacking.…”

Usefulness of serum Wisteria floribunda agglutinin‐positive Mac‐2 binding protein in children with primary sclerosing cholangitis

“…Primary sclerosing cholangitis (PSC) is a chronic cholestatic liver disease characterized by inflammation and fibrosis of the intra‐ and/or extrahepatic ducts . Although the disease progression of PSC is various, PSC could lead to end‐stage liver disease and cirrhosis, which require liver transplantation . Because PSC is frequently clinically asymptomatic, the delay of diagnosis of PSC is not uncommon in children .…”

“…Because PSC is frequently clinically asymptomatic, the delay of diagnosis of PSC is not uncommon in children . Therefore, advanced liver fibrosis (septal/bridging fibrosis and cirrhosis) is developed in 10–65% of children with PSC at the time of diagnosis . Because a cholangiographic assessment is the gold standard to establish a diagnosis of large duct PSC, liver biopsy is not essential to establish a diagnosis, except for small duct PSC .…”