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Analyses of Proteinuria, Renal Infiltration of Leukocytes, and Renal Deposition of Proteins in Lupus-prone MRL/lpr Mice
Published on: June 8, 2022
Renal manifestations of paediatric systemic lupus erythematosus
Thomas Dowsett1, Louise Oni2,3
1Department of Paediatric Nephrology, Royal Manchester Children's NHS Trust Hospital, Manchester.
Insights
Paediatric lupus nephritis requires improved management strategies. Current treatments show inadequate response rates, highlighting the need for novel therapies and biomarkers for better patient outcomes.
Area of Science:
- Pediatric Nephrology
- Rheumatology
- Immunology
Background:
- Systemic lupus erythematosus (SLE) is a chronic, multisystemic autoimmune disease.
- Approximately 20% of SLE patients present in childhood, often with more severe disease and higher rates of lupus nephritis.
- Paediatric lupus nephritis presents unique challenges compared to adult-onset disease.
Purpose of the Study:
- To review the current literature on the management of lupus nephritis in children.
- To highlight the challenges and recent advancements in treating this condition.
Main Methods:
- Comprehensive literature search of studies focusing on paediatric lupus nephritis management.
- Analysis of current treatment guidelines, clinical trial data, and emerging therapeutic strategies.
Main Results:
- Standard treatment involves intensive induction followed by long-term maintenance immunosuppression.
- Complete remission rates are suboptimal (40-60%), with frequent disease flares.
- Progression to end-stage renal disease occurs in over 10% within a decade; CKD stages 1-4 progression is less defined.
- Biologic agents are being investigated as adjunctive therapies.
Conclusions:
- There is a critical need for improved therapeutic outcomes in paediatric lupus nephritis.
- Biomarkers for monitoring disease activity and novel treatment approaches are essential.
- Further research into pathogenesis and targeted therapies holds promise for better management.
Purpose Of Review:
Systemic lupus erythematosus is a lifelong, multisystemic disease. Around a fifth of patients present during childhood. Children are recognized to have a more active disease course with more renal involvement (lupus nephritis) when compared with adults. This review article summarizes the current literature surrounding the management of paediatric lupus nephritis.
Recent Findings:
International recommendations agree that active, proliferative forms of lupus nephritis are treated with a period of intense induction therapy aimed at inducing remission followed by maintenance immunosuppressive therapy for at least 3 years. Complete response rates in lupus nephritis remain inadequate, in the region of 40-60%, and disease flares are common. Revised histological classification have been proposed but they are yet to be adopted in clinical practice. Lupus nephritis progresses to chronic kidney disease (CKD) stage 5 (kidney failure) in more than 10% of patients within 10 years however the rates of CKD stages 1-4 remain largely unknown. Current trials are focused on the use of biologic agents as adjuncts to current therapy.
Summary:
There is an urgent need for better outcomes in paediatric lupus nephritis. The use of biomarkers to monitor lupus nephritis and scientific studies to improve our understanding of the pathogenesis offer hope of improved outcomes.
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