Primary sclerosing cholangitis related to inflammatory bowel disease (PSC-IBD) diagnosed under the age of 6 years (i.e.VEO-IBD) may have unique characteristics and disease course. We aimed to analyze the characteristics and natural history of children with VEO PSC-IBD and compare them to children diagnosed with PSC-IBD at an older age.
This was a multicenter, retrospective, study evaluating patients diagnosed with both IBD and PSC before (VEO-PSC-IBD) or after the age of 6 years (PSC-IBD), followed at 14 centers affiliated with the Porto IBD Interest group of ESPGHAN. Demographic, clinical, laboratory, endoscopic, and imaging data were collected at baseline and every six months thereafter for a minimum follow-up of 12 months. Outcomes were analyzed at 1, 3 and 5 years
A total of 69 children with both IBD and PSC were included: 28 with VEO PSC-IBD [median age 5.2 years, 21 UC (75%)] and 41 with PSC-IBD [median age 15.7 years, 34 UC (83%)]. Most patients with UC presented with extensive disease at diagnosis (89% in VEO PSC-UC vs. 89% in PSC-UC, p=0.72). Both groups presented most often with mild intestinal disease at diagnosis (mean PUCAI of VEO IBD-PSC 34±16, vs 31±19 of IBD-PSC, p=0.11). A higher number of VEO-IBD-PSC patients were diagnosed with autoimmune SC than older children [24 (86%) vs. 27 (66%) PSC-IBD, p=0.04], whereas no other differences were found for PSC-related variables. The risk of developing biliary strictures and starting vancomycin after ursodehoxicolic acid was lower in the VEO-PSC-IBD group (Log-rank p=0.02 and p=0.02), while no difference was found for portal hypertension and liver transplantation at 5-year follow-up. No cases of cholangiocarcinoma or death were found.
IBD-PSC has similar baseline characteristics whether diagnosed as VEOIBD or thereafter. However, a milder disease course in terms of biliary complications and the need for PSC-related therapy escalation characterize the VEOIBD subcohort.