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Published on: November 1, 2015
Juvenile systemic lupus erythematosus: onset patterns and short-term outcome in Egyptian children, a single-center
M A Abdel-Hafez1, H Abdel-Nabi2
1Tanta University, Pediatric Nephrology Unit, Tanta University Hospital, Tanta, Egypt maher-ahmed_2005@yahoo.com.
Insights
Egyptian children with systemic lupus erythematosus (SLE) often present with severe symptoms and kidney involvement. However, they show a good response to treatment, indicating a positive short-term outlook.
Area of Science:
- Pediatric Rheumatology
- Systemic Lupus Erythematosus (SLE) Research
- Clinical Immunology
Background:
- Systemic lupus erythematosus (SLE) is a chronic autoimmune disease affecting multiple organ systems.
- Understanding the specific disease patterns and treatment responses in pediatric populations is crucial for effective management.
- Egyptian children represent a unique demographic for studying SLE due to potential genetic and environmental factors.
Purpose of the Study:
- To define the disease onset patterns in Egyptian children diagnosed with SLE.
- To evaluate the response to therapy in this specific patient cohort.
- To correlate disease presentation with treatment outcomes.
Main Methods:
- A prospective cohort study involving 41 Egyptian children with SLE.
- Assessment of disease activity at onset using the SLE Disease Activity Index (SLEDAI) score.
- Evaluation of lupus nephritis through renal biopsy and follow-up assessments over 10-50 months.
Main Results:
- The mean age of diagnosis was 12.12 years, with 87.8% females. Common symptoms included pallor and fever.
- Lupus nephritis was prevalent (65.9%), with ISN classes I and III most common. Neuropsychiatric manifestations occurred in 46.3%.
- High SLEDAI scores (mean 29.95) were recorded at diagnosis. At follow-up, 39.02% achieved complete remission, while 4.87% had active disease.
Conclusions:
- Egyptian pediatric SLE patients often present with severe disease and high rates of lupus nephritis.
- Despite severe onset, these children demonstrate a favorable short-term prognosis with good response to therapy.
- Early diagnosis and management are key to improving outcomes in pediatric SLE.
Objective:
The objective of this article is to define disease onset pattern and understand the response to therapy in children with systemic lupus erythematosus (SLE) in Egypt.
Methods:
A prospective cohort of 41 Egyptian children diagnosed with SLE was analyzed. SLE Disease Activity Index (SLEDAI) score was used to record disease activity at onset, and renal biopsy was performed to define the stage of lupus nephritis. Response to therapy over a follow-up period ranging from 10 to 50 months was evaluated.
Results:
The mean age at diagnosis was 12.12 ± 3.45 years. Thirty-six (87.8%) patients were females. Most patients had multiple manifestations at onset. The most common presenting symptoms were pallor and fever (51.2% and 43.9%, respectively). Lupus nephritis was found in 27 (65.9%) children. International Society of Nephrology (ISN) classes I and III were the most common findings on renal biopsy. Neuropsychiatric manifestations were present at disease onset in 19 patients (46.3%) with a bad prognostic course. At diagnosis, high SLEDAI scores were recorded (mean: 29.95 ± 2.06). The mean renal SLEDAI score was 10.2 ± 4. At follow-up 16 (39.02%) patients were in complete remission, 10 (24.39%) were in partial remission, two (4.87%) had active disease, five (12.9%) had relapsed, four (9.75%) had died and four (9.75%) patients were lost to follow-up.
Conclusions:
Egyptian children with SLE appear to have severe disease on presentation with high SLEDAI scores and high prevalence of lupus nephritis, but respond well to therapy with a favorable short-term prognosis.
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