The Natural History of Pediatric Primary Sclerosing Cholangitis

Nearly half of children experienced an adverse liver-related outcome in the 10 years following diagnosis.

Much is known about adult-onset primary sclerosing cholangitis (PSC) but not pediatric PSC, which is extremely rare.

To describe the natural history of pediatric PSC and identify prognostic factors for long-term outcomes, researchers retrospectively assessed clinical endpoints in 781 children (median age, 12 years) who received a diagnosis of PSC from multiple centers around the world. Endpoints included portal hypertensive complications, biliary complications, cholangiocarcinoma, liver transplantation, and death.

Thirty-three percent of patients had co-occurring autoimmune hepatitis, 76% had inflammatory bowel disease (IBD), and 13% had small-duct PSC. During 4277 person-years of follow-up, results were as follows:

Event-free survival (i.e., …

Reviewing Author

Atif Zaman, MD, MPH

Disclosures

Nothing to disclose

Atif Zaman, MD, MPH

Disclosures

Nothing to disclose

The Natural History of Pediatric Primary Sclerosing Cholangitis

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Reviewing Author