Paediatric-onset immune-mediated inflammatory diseases raise mortality risk

People with paediatric-onset immune-mediated inflammatory diseases (pIMID) have a roughly fourfold increased risk of death in adulthood, as suggested in a study.

For the study, researchers used data from the nationwide Danish healthcare registers. They identified 11,581 patients diagnosed with pIMID in Denmark, which accounted for 1,371,994 person-years of follow-up. These patients were matched to 99,665 no-pIMID control individuals from the general population.

PIMID included autoimmune hepatitis, primary sclerosing cholangitis, Crohn's disease, ulcerative colitis, juvenile idiopathic arthritis, lupus erythematosus, or vasculitis diagnosed before 18 years of age. The primary outcome was all-cause mortality, and the secondary outcome was cause-specific mortality. Outcomes were analysed in relation to pIMID using Cox and Aalen survival analyses.

The median age at diagnosis of pIMID in the population was 12.6 years. A total of 152 patients with pIMID and 316 controls died during the follow-up, corresponding to 6.9 (95 percent confidence interval [CI], 5.3–8.5) additional deaths per 10,000 person-years. Mortality was 3.8-fold higher in the pIMID group than in the control group (adjusted HR [aHR], 3.8, 95 percent CI, 3.1–4.7).

The association was especially pronounced for patients with gastrointestinal diseases (aHR, 22.8, 95 percent CI, 9.6–64.1), gastrointestinal cancers (aHR, 19.2, 95 percent CI, 5.0–74.2), and lymphoproliferative disorders (aHR, 6.8, 95 percent CI, 2.8–16.8).

IMID have more aggressive phenotypes when diagnosed in children than in adults, according to the researchers. The findings underscore the severe disease course of pIMID and highlight the need for multidisciplinary care.