Development of Primary Sclerosing Cholangitis in Pediatric Patients with Inflammatory Bowel Disease

A recent study (A Chandrakumar et al. J Pediatr 2019; 215: 144-51) followed 190 children with inflammatory bowel disease from 2011 to 2018 in a longitudinal population-based cohort in Manitoba and examined the development of primary sclerosing cholangitis (PSC).  The diagnosis of PSC was made on discretion of the treating physician; thus, only a subset of patients underwent extensive evaluations for PSC.

Key findings:

  • 9 developed PSC-UC (9/95) and overall 11 developed PSC-IBD (11/190)
  • Among children with PSC-UC, 8 had high GGT (>50) at baseline and only 1 had a normal GGT at baseline.
  • All UC patients who developed PSC were diagnosed withing 6 months of their UC diagnosis.
  • At baseline, 22 patients with UC had an elevated GGT and 73 had a normal GGT.  Thus, about one-third of patients with an elevated GGT developed PSC (possibly more as all patients were not subjected to extensive testing)

My view: This study reinforces two concepts: 1) GGT is valuable as a screening test 2) PSC (often asymptomatic) is fairly common in UC and needs to be considered especially in the first year of diagnosis.  What this study does not do is help us figure out what should be done about children with asymptomatic PSC as there are no proven therapies.

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