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Published on: August 7, 2017
Paediatric-onset immune-mediated inflammatory disease is associated with an increased mortality risk-A nationwide
Mikkel Malham1,2,3,4,5, Sabine Jansson1,2, Helene Ingels6
1Department of Paediatric and Adolescence Medicine, Copenhagen University Hospital-Amager and Hvidovre, Hvidovre, Denmark.
Insights
Children diagnosed with immune-mediated inflammatory diseases (pIMID) face a fourfold increased mortality risk. This highlights the severe nature of pIMID and the need for specialized, multidisciplinary care from diagnosis through adulthood.
Area of Science:
- Pediatric Rheumatology
- Immunology
- Public Health
Background:
- Paediatric-onset immune-mediated inflammatory diseases (pIMID) often present with more aggressive disease patterns compared to adult-onset cases.
- Existing mortality data for pIMID are frequently based on studies of adult populations, potentially underestimating the true risk in younger individuals.
Purpose of the Study:
- To accurately estimate the impact of paediatric-onset immune-mediated inflammatory diseases (pIMID) on all-cause and cause-specific mortality.
Main Methods:
- A population-based cohort study utilized nationwide Danish healthcare registers from 1980 to 2018.
- Included patients diagnosed with specific pIMID before age 18, comparing mortality outcomes against a reference population.
- Employed Cox and Aalen survival analyses to calculate hazard ratios and rate differences for mortality risk.
Main Results:
- The study included 11,581 patients with pIMID and 99,665 reference individuals, with a median age at diagnosis of 12.6 years.
- Patients with pIMID exhibited a significantly higher risk of all-cause mortality (adjusted HR 3.8), translating to 6.9 additional deaths per 10,000 person-years.
- Elevated mortality risks were particularly noted for gastrointestinal diseases (aHR 22.8), gastrointestinal cancers (aHR 19.2), and lymphoproliferative disorders (aHR 6.8).
Conclusions:
- Individuals diagnosed with pIMID face a substantially increased mortality risk, approximately fourfold higher into early adulthood compared to the general population.
- These findings underscore the severe and prolonged impact of pIMID, emphasizing the critical need for comprehensive, multidisciplinary care pathways.
Background:
Paediatric-onset immune-mediated inflammatory diseases (pIMID) show more aggressive phenotypes than when diagnosed in adults. However, data on mortality are often extrapolated from adult studies.
Aim:
To estimate the effect of pIMID on mortality.
Methods:
In a population-based cohort study using the nationwide Danish healthcare registers, we included all patients diagnosed with pIMID in Denmark from 1980 to 2018. PIMID were defined as ICD codes indicative of autoimmune hepatitis, primary sclerosing cholangitis, Crohn's disease, ulcerative colitis, juvenile idiopathic arthritis, lupus erythematosus, or vasculitis registered before age 18 years. All-cause mortality was the primary outcome; cause-specific mortality was the secondary outcome. We used Cox survival analysis to estimate hazard ratios (HR), and Aalen survival analysis to estimate rate differences.
Results:
We included 11,581 individuals diagnosed with pIMID and 99,665 reference individuals, accounting for 1,371,994 person-years of follow-up. Median and interquartile (IQR) age at diagnosis was 12.6 (7.9-15.9) years. During follow-up, 152 patients with pIMID and 316 reference individuals died; adjusted HR (aHR) was 3.8 (95% confidence interval [CI] 3.1-4.7). This corresponded to 6.9 (95% CI: 5.3-8.5) additional deaths per 10,000 person-years. The strongest associations were found for gastrointestinal diseases (aHR 22.8; 95% CI 9.6-64.1), gastrointestinal cancers (aHR 19.2; 95% CI 5.0-74.2) and lymphoproliferative disorders (aHR 6.8; 95% CI 2.8-16.8).
Conclusion:
Patients diagnosed with pIMID have a fourfold higher risk of mortality when followed into early adulthood compared with reference individuals. This underlines the severe disease course of pIMID and highlights the need for multidisciplinary care.
