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Mortality outcomes in pediatric rheumatology in the US
Philip J Hashkes1, Bridget M Wright, Michael S Lauer
1Cleveland Clinic, Cleveland, Ohio, USA. hashkep@ccf.org
Insights
Overall mortality in pediatric rheumatic diseases is not increased, with rates lower than previously reported. Specific conditions like lupus and dermatomyositis showed higher mortality, but overall risk factors were identified.
Area of Science:
- Rheumatology
- Pediatric Medicine
- Epidemiology
Background:
- Pediatric rheumatic diseases encompass a range of autoimmune and inflammatory conditions affecting children.
- Understanding mortality patterns is crucial for improving patient outcomes and guiding clinical management.
- Previous studies have indicated varying mortality rates, necessitating updated data.
Purpose of the Study:
- To determine mortality rates and causes of death in a large cohort of pediatric rheumatic disease patients in the US.
- To identify risk factors associated with mortality in this population.
- To compare current mortality rates with historical data.
Main Methods:
- Utilized the Indianapolis Pediatric Rheumatology Disease Registry (49,023 newly diagnosed patients, 1992-2001).
- Matched patient identifiers with the Social Security Death Index (censored March 2005).
- Confirmed deaths via certificates, physicians, and medical records; analyzed causes of death and calculated standardized mortality ratios (SMRs).
Main Results:
- Confirmed 110 deaths (0.23%) in 48,885 patients, with a significantly decreased overall SMR (0.65).
- Elevated SMRs were observed for systemic lupus erythematosus (3.06) and dermatomyositis (2.64), while pain syndromes showed a decreased SMR (0.41).
- Key mortality risk factors included rheumatic diagnosis, age at diagnosis, sex, and early use of systemic steroids and methotrexate.
Conclusions:
- The overall mortality rate for pediatric rheumatic diseases in the US is not elevated and is lower than previously reported.
- While certain conditions like systemic lupus erythematosus and dermatomyositis have increased mortality, the rates are decreasing.
- Identifying risk factors provides targets for intervention to further reduce mortality in pediatric rheumatic diseases.
Objective:
To describe mortality rates, causes of death, and potential mortality risk factors in pediatric rheumatic diseases in the US.
Methods:
We used the Indianapolis Pediatric Rheumatology Disease Registry, which includes 49,023 patients from 62 centers who were newly diagnosed between 1992 and 2001. Identifiers were matched with the Social Security Death Index censored for March 2005. Deaths were confirmed by death certificates, referring physicians, and medical records. Causes of death were derived by chart review or from the death certificate. Standardized mortality ratios (SMRs) and 95% confidence intervals (95% CIs) were determined.
Results:
After excluding patients with malignancy, 110 deaths among 48,885 patients (0.23%) were confirmed. Patients had been followed up for a mean +/- SD of 7.9 +/- 2.7 years. The SMR of the entire cohort was significantly decreased (0.65 [95% CI 0.53-0.78]), with differences in patients followed up for > or =9 years. The SMR was significantly greater for systemic lupus erythematosus (3.06 [95% CI 1.78-4.90]) and dermatomyositis (2.64 [95% CI 0.86-6.17]) but not for systemic juvenile rheumatoid arthritis (1.8 [95% CI 0.66-3.92]). The SMR was significantly decreased in pain syndromes (0.41 [95% CI 0.21-0.72]). Causes of death were related to the rheumatic diagnosis (including complications) in 39 patients (35%), treatment complications in 11 (10%), non-natural causes in 25 (23%), background disease in 23 (21%), and were unknown in 12 patients (11%). Rheumatic diagnoses, age at diagnosis, sex, and early use of systemic steroids and methotrexate were significantly associated with the risk of death.
Conclusion:
Our findings indicate that the overall mortality rate for pediatric rheumatic diseases was not increased. Even for the diseases and conditions associated with increased mortality, mortality rates were significantly lower than those reported in previous studies.
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