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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
Pediatric lupus nephritis: more options, more chances?
1Department of Pediatrics, Prince of Songkla University, Songkla 90110,Thailand. vprayong@gmail.com
Insights
Lupus nephritis (LN) is a severe kidney disease in childhood systemic lupus erythematosus (SLE). Current treatments improve survival but long-term remission and safety remain challenges for pediatric patients.
Area of Science:
- Pediatric Nephrology
- Rheumatology
- Immunology
Background:
- Lupus nephritis (LN) presents more severely in childhood-onset systemic lupus erythematosus (SLE) compared to adults.
- LN is a primary cause of acute kidney injury (AKI) and chronic kidney disease (CKD) in children.
- Steroid therapy, introduced in 1970, has significantly improved SLE patient survival from 50% to over 80%.
Purpose of the Study:
- To review the current landscape of lupus nephritis treatment in pediatric SLE.
- To evaluate the effectiveness and safety of various immunosuppressive therapies.
- To highlight the challenges in achieving long-term remission and managing comorbidities.
Main Methods:
- Review of historical and current immunosuppressive treatments for pediatric LN.
- Analysis of survival rates and treatment outcomes from published studies.
- Consideration of drug benefits, adverse events, and patient-specific factors.
Main Results:
- Combination therapies with prednisolone (e.g., cyclophosphamide, mycophenolate mofetil, rituximab, tacrolimus) have increased survival rates to 90%-95%.
- Long-term disease remission remains a challenge, with many treatments showing initial efficacy but lacking sustained results.
- Fatal infections are a significant complication of aggressive chemotherapy regimens.
Conclusions:
- Optimizing treatment for pediatric SLE and LN requires balancing efficacy with safety and managing diverse patient factors.
- Developing safe and effective long-term maintenance therapies is crucial for sustained remission.
- Personalized combination therapies are likely necessary to improve outcomes for children with this debilitating disease.
Abstract:
Lupus nephritis (LN) is more common and severe in childhood-onset systemic lupus erythematosus (SLE) than in adults. It is one of the major causes of acute kidney injury (AKI) and chronic kidney disease (CKD) in children. Steroid therapy has been used as the first-line treatment for SLE since 1970, and has improved the survival of SLE patients from ∼ 50% to >80%. Over the years many immunosuppressive drugs, including pulse methylprednisolone, oral cyclophosphamide, pulse intravenous cyclophosphamide, mycophenolate mofitil, rituximab, and tacrolimus, have been combined with prednisolone, further improving survival rates to 90%-95%. However, the effectiveness of these drugs is still uncertain, as most seem very good in the beginning, but in studies examining longer-term follow-up the remission of disease does not remain. Fatal infection is still a major complication of aggressive chemotherapy, and the potential benefits as well as adverse events from each drug need to be considered. Induction of remission is the major aim of therapy, with safe and effective maintenance therapy for long-term remission. The survival rates of many published studies vary widely because of differences in patients and treatment modalities, severity of disease, renal histopathology, racial factors, and duration of follow-up. Finding the optimal treatment for SLE and related co-morbidities is highly challenging, and will likely involve a complex combination of different drugs for different patients in the search for giving them an opportunity to be free from this debilitating disease.
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