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The bm12 Inducible Model of Systemic Lupus Erythematosus SLE in C57BL/6 Mice
Published on: November 1, 2015
Long-term outcomes of childhood-onset systemic lupus erythematosus
Anne Mirguet1,2, Florence A Aeschlimann3, Irene Lemelle4
1Department of Pediatric Nephrology, Children Hospital, University Hospital of Nancy, Lorraine University, Vandoeuvre-lès-Nancy, France.
Insights
Long-term outcomes for childhood-onset systemic lupus erythematosus (cSLE) show persistent high rates of organ damage and active disease. Sub-Saharan African ethnicity is linked to increased odds of active disease in these patients.
Area of Science:
- Pediatric Rheumatology
- Systemic Lupus Erythematosus (SLE)
- Autoimmune Diseases
Background:
- Long-term outcome data for childhood-onset SLE (cSLE) are limited.
- Understanding long-term sequelae is crucial for managing this chronic condition.
Purpose of the Study:
- To describe the long-term outcomes of patients with cSLE.
- To identify factors associated with disease damage and persistent activity.
Main Methods:
- Retrospective multicentre study using data from the PEDIALUP registry.
- Collected demographic, clinical, laboratory, radiological, histological, and treatment data from medical records.
Main Results:
- 138 cSLE patients followed for a median of 15.4 years.
- 51% had cumulative damage (SLICC-DI ≥1), increasing with follow-up duration.
- 34% had active disease (SLEDAI ≥6); Sub-Saharan African ethnicity associated with 7-fold increased odds.
Conclusions:
- High prevalence of damage persists in cSLE patients, even with recent diagnoses.
- Duration of follow-up and ethnicity are significant factors in long-term outcomes.
Objective:
Data on the long-term outcome of patients with childhood-onset SLE (cSLE) are scarce. Aims of this study were to describe the long-term outcomes of cSLE and to identify factors associated with the development of damage and persistent disease activity.
Methods:
We conducted a retrospective multicentre study using data from the PEDIALUP registry of the Juvenile Inflammatory Rheumatism (JIR) cohort database. Demographic characteristics, clinical manifestations, laboratory, radiological, histological and treatment data were collected from medical records during follow-up.
Results:
A total of 138 patients with cSLE, diagnosed between 1971 and 2015, were included. With a median follow-up of 15.4 [9.6-22.4] years, 51% of patients had a SLICC-damage index (DI) score ≥1 at last follow-up with the musculoskeletal, cutaneous, renal, neurological and cardiovascular damage being the most common manifestations. The proportion of patients with a SLICC-DI score ≥1 increased significantly with the duration of the follow-up (P < 0.001). On multivariate analysis, duration of follow-up was associated with increased risk of cumulative damage (OR 1.08, 95% CI 1.01, 1.15, P = 0.035). At the last visit, 34% of patients still had active disease with a SLEDAI score of ≥6. On multivariate analysis, sub-Saharan African ethnicity was associated with 7-fold increased odds of having active disease at the last visit compared with Caucasians (OR 7.44, 95% CI 2.24, 24.74, P = 0.0002).
Conclusion:
The prevalence of damage remains high in patients with cSLE even when the diagnosis of cSLE has been made in the recent decades.
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