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The morbidity and mortality associated with childhood onset systemic lupus erythematosus
1Department of Pediatrics, Chang Gung Memorial Hospital, Taipei, Taiwan, R.O.C.
Insights
This study analyzed 137 childhood-onset systemic lupus erythematosus cases from 1977-1991. Sepsis and renal failure were the primary causes of death, highlighting the need for early intervention in pediatric lupus patients.
Area of Science:
- Pediatric Rheumatology
- Immunology
- Internal Medicine
Background:
- Systemic lupus erythematosus (SLE) is a chronic autoimmune disease that can affect multiple organ systems.
- Childhood-onset SLE presents unique challenges in diagnosis and management compared to adult-onset disease.
- Understanding the long-term morbidity and mortality is crucial for improving patient outcomes.
Purpose of the Study:
- To analyze the clinical characteristics, complications, and outcomes of childhood-onset systemic lupus erythematosus.
- To identify major causes of morbidity and mortality in pediatric SLE patients.
- To evaluate the survival rates in this cohort.
Main Methods:
- Retrospective review of medical records for 137 pediatric patients diagnosed with SLE between 1977 and 1991.
- Analysis of clinical and laboratory data, focusing on presenting features, complications, and causes of death.
- Calculation of 5-year survival rate using life-table methods.
Main Results:
- Clinical and laboratory findings were consistent with existing literature on pediatric SLE.
- Renal failure was present in 8% at initial diagnosis.
- Common non-infectious complications included hypertension, avascular necrosis, and cataracts; sepsis was a frequent infectious complication.
- Sepsis (42%) and renal failure (30.7%) were the leading causes of death.
- The 5-year survival rate was 76.3%.
Conclusions:
- Childhood-onset SLE exhibits significant morbidity and mortality, with sepsis and renal failure being major concerns.
- Effective management strategies are needed to reduce complications and improve survival rates in pediatric lupus.
- Long-term follow-up is essential for monitoring disease progression and outcomes in this vulnerable population.
Abstract:
From 1977 till 1991, the diagnosis of systemic lupus erythematosus was made on 137 children aged 18 years or under in Chang Gung Memorial Hospital. The medical records were reviewed and the clinical data were analysed with emphasis on the morbidity and mortality of this disease. The clinical and laboratory characteristics were similar to the findings from most other reports. Renal failure accounted for 8% of the initial presentation. The non-infectious complications were, in the order of frequencies, hypertension, avascular necrosis of femoral head, cataract, encephalopathy, and venous thrombosis. Sepsis, cutaneous infection and urinary tract infection were the frequently encountered infectious complications. The major causes of death in childhood onset systemic lupus erythematosus were sepsis (42%) and renal failure (30.7%). Forty patients were lost to follow-up. The 5-year survival rate, calculated by life-table, was 76.3%.