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Pediatric PSC-IBD confers specific disease phenotype
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Children with inflammatory bowel disease and primary sclerosing cholangitis may have milder disease activity, but the lack of symptoms does not always correlate with an absence of mucosal inflammation, according to published findings.
Amanda Ricciuto, MD, FRCPC, PhD, of the division of gastroenterology, hepatology and nutrition at the Hospital for Sick Children in Toronto, and colleagues wrote that a specific phenotype of IBD that coexists with primary sclerosing cholangitis (PSC) has been confirmed in adult studies but has not been fully established in children.
“Accurate phenotype characterization is a fundamental steppingstone toward elucidation of disease pathogenesis and therapy development,” they wrote. “Furthermore, it may enable early disease recognition. Given the paucity and limitations of existing pediatric data, we aimed to delineate the IBD phenotype occurring in children with PSC and to establish whether or not it is congruent with the adult phenotype.”
Researchers performed a retrospective study comprising 74 children with PSC-IBD who were diagnosed between 2000 and 2018 and matched them with two control children with ulcerative colitis or unclassified IBD based on sex, date of birth and IBD type. They compared clinical activity including remission, medication use, hospitalization and colectomy between the groups.
Ricciuto and colleagues found that the PSC-IBD group has higher proportions of children who had backwash ileitis, pancolitis and rectal sparing, as well as more severe right-sided disease compared with controls (P < .05). Additionally, patients with PSC-IBD were more likely to be treated with 5-aminosalicylate therapy (OR = 3.04; 95% CI, 1.44-6.41) and to be in clinical remission (OR = 2.94; 95% CI, 1.78-4.87) compared with controls. Patients with PSC-IBD were also at lower risk for colectomy (HR = 0.24; 95% CI, 0.12-0.52).
However, investigators found that lack of clinical symptoms in children with PSC-IBD was not always associated with endoscopic activity. Among patients in clinical remission, patients with PSC were less likely to be in endoscopic remission (OR = 0.44; 95% CI, 0.2-0.96).
Ricciuto and colleagues wrote that similar to what is found in adults, pediatric PSC-IBD has a distinct phenotype that includes pancolitis, backwash ileitis and subclinical inflammation.
“The finding of subclinical inflammation highlights the need for disease monitoring strategies that extend beyond symptom assessment,” they wrote. “In addition, recognition of the unique IBD phenotype may facilitate earlier recognition of PSC, at a stage when the mechanisms that underlie progressive biliary inflammation and fibrosis are best elucidated and much needed therapeutic interventions developed.” – by Alex Young
Disclosures: Ricciuto reports she received education research fellowship funding from Janssen. Please see the study for all other authors’ relevant financial disclosures.
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