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Mortality rates in children, young people, and young adults with JIA: an observational study using the Clinical
Lianne Kearsley-Fleet1, Natasha Shaw1, Jasmine Leslie1
1Centre for Epidemiology Versus Arthritis, Centre for Musculoskeletal Research, Division of Musculoskeletal and Dermatological Sciences, University of Manchester, Manchester Academic Health Sciences Centre, Manchester, UK.
Insights
Juvenile idiopathic arthritis (JIA) patients have a 3.3 times higher mortality rate compared to controls. While death is rare in JIA, systemic JIA carries a higher risk, especially before 2012.
Area of Science:
- Rheumatology
- Pediatric Rheumatology
- Epidemiology
Background:
- Rheumatological conditions often correlate with increased mortality.
- Evidence regarding the impact of juvenile idiopathic arthritis (JIA) on mortality remains conflicting.
- This study addresses the need for clear data on JIA's effect on patient lifespan.
Purpose of the Study:
- To determine the all-cause mortality rate in patients diagnosed with juvenile idiopathic arthritis (JIA).
- To compare JIA patient mortality against matched controls and general population estimates.
- To investigate mortality differences between systemic and non-systemic JIA subtypes.
Main Methods:
- Utilized the UK's Clinical Practice Research Datalink (CPRD) for JIA cases diagnosed before age 16.
- Matched JIA patients 4-to-1 with non-JIA controls based on birth year, gender, and practice.
- Employed Cox-proportional hazards models and calculated standardized mortality ratios (SMRs) for comparison.
Main Results:
- JIA patients exhibited a 3.3 times higher mortality rate (6.2/10,000 person-years) compared to matched controls (1.9/10,000 person-years).
- Systemic JIA patients faced a 3.3 times higher mortality risk than non-systemic JIA patients.
- The standardized mortality ratio (SMR) for JIA was 2.9, with 60% of deaths occurring before 2012.
Conclusions:
- Mortality in young people with JIA is rare, but elevated compared to the general population.
- Systemic JIA is associated with a significantly higher mortality risk.
- Improved outcomes, particularly for systemic JIA, may be linked to advancements in biologic treatments post-2012.
Objectives:
Many rheumatological conditions have increased mortality rates; however, there is conflicting evidence regarding the impact of juvenile idiopathic arthritis (JIA) on mortality. This analysis aimed to calculate the all-cause mortality rate of patients with JIA, compared with (a) matched-control patients and (b) the general population mortality estimates.
Methods:
Using the UK General Practice data Clinical Practice Research Datalink, JIA cases starting <16 years old from England were included, matched 4-to-1 with non-JIA controls (birth year, gender, practice). Exposure started on first JIA-code date (matched-date for controls) or January 1, 2000, whichever was latest. Follow-up continued until December 31, 2018, or death, whichever was first. Cox-proportional hazards models compared mortality in JIA versus matched controls. JIA rates were stratified by systemic versus non-systemic JIA. Standardised mortality rates (SMRs) were generated for JIA versus general population estimates (calendar year, age, gender).
Results:
There were 4762 patients with JIA (30 deaths) and 13 957 matched controls (27 deaths); patient demographics were similar between cohorts. Mortality rate for JIA was 6.2/10 000 person years (95% CI: 4.3-8.9), and 1.9/10 000 person years (95% CI: 1.3-2.8) for matched controls; JIA patients had 3.3 times higher mortality (95% CI: 2.0-5.5). Patients with systemic JIA had 3.3 times higher mortality (95% CI: 1.6-6.9) versus those with non-systemic JIA. The SMR for JIA was 2.9 (95% CI: 2.1-4.2). Eighteen (60%) JIA deaths occurred before 2012.
Conclusions:
This analysis calculated mortality rates in young people with JIA in England. Death in young people with JIA is exceedingly rare. Higher rates were observed in patients with systemic JIA. Over half the deaths occurred prior to 2012 when biologic treatment, particularly for systemic JIA, was limited.
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