Unique features of primary sclerosing cholangitis in children.

PURPOSE OF REVIEW

To summarize publications on juvenile primary sclerosing cholangitis (PSC) published over the past 5 years. These studies contribute to the understanding of the clinical features, diagnostic pathways, genetic aspects, treatment and outcome of this condition.

RECENT FINDINGS

Sclerosing cholangitis with strong autoimmune features is particularly frequent in paediatric age, where it is more common in girls, responds to immunosuppressive treatment and has a better prognosis than classical PSC. Modern-day magnetic resonance cholangiography allows accurate diagnosis of bile duct disease in most cases. Prolonged oral vancomycin treatment may be beneficial in difficult-to-treat PSC associated with inflammatory bowel disease. Juvenile PSC has a high recurrence rate after liver transplantation. PSC susceptibility and resistance are associated with both human leucocyte antigen-related and unrelated genetic factors.

SUMMARY

Studies on large cohorts of children with PSC are needed for a better understanding of pathogenic mechanisms, response to treatment and outcome of this serious condition.