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Published on: June 8, 2022
Pharmacotherapy of lupus nephritis in children: a recommended treatment approach
Alexa Adams1, Emma Jane MacDermott, Thomas J A Lehman
1Division of Pediatric Rheumatology, Hospital for Special Surgery, New York, NY 10021, USA.
Insights
Systemic lupus erythematosus (SLE) nephritis in children requires effective treatment. Current standards focus on cyclophosphamide, with newer agents like rituximab showing promise for improved outcomes.
Area of Science:
- Pediatric Rheumatology
- Nephrology
- Immunology
Background:
- Systemic lupus erythematosus (SLE) is a multisystem inflammatory disease with recurrent flares.
- Renal involvement (lupus nephritis) is a major cause of morbidity and mortality in pediatric SLE.
- Lupus nephritis presents a spectrum from mild glomerulitis to severe proliferative glomerulonephritis.
Purpose of the Study:
- To review current standards of care for pediatric lupus nephritis.
- To discuss newer and emerging therapies for this condition.
- To provide guidelines for comprehensive care of children with lupus nephritis.
Main Methods:
- Review of current literature on SLE and lupus nephritis treatment in children.
- Analysis of established and investigational therapeutic agents.
- Discussion of clinical guidelines and expert recommendations.
Main Results:
- Corticosteroids are first-line but often supplemented by cytotoxic drugs like cyclophosphamide.
- Mycophenolate mofetil shows less efficacy in children compared to adults.
- Intravenous cyclophosphamide is the standard for highly active pediatric lupus nephritis.
- Rituximab may aid remission induction and reduce cyclophosphamide dosage.
Conclusions:
- Combination therapies and novel agents hold future promise for pediatric lupus nephritis.
- Understanding SLE immune dysregulation is key to developing better treatments.
- Evidence-based guidelines are essential for optimal management and comprehensive care.
Abstract:
Systemic lupus erythematosus (SLE) is a multisystem inflammatory disease of unknown aetiology, which is characterised by recurrent disease flares that may affect any organ system. Renal involvement remains one of the chief causes of morbidity and mortality in children with lupus. Nephritis occurs in approximately two-thirds of patients, ranging from mild glomerulitis to life-threatening occurrences of diffuse proliferative glomerulonephritis. As lupus nephritis is a condition of no single aetiology, there is no single cure. Corticosteroids, although still the first line of treatment, are increasingly being superseded by cytotoxic drugs, in particular cyclophosphamide and corticosteroid-sparing agents. Newer agents such as mycophenolate mofetil, although effective in the treatment of lupus in adults, are less effective in children. Standard of care for highly active lupus nephritis in children remains intravenous cyclophosphamide, although preliminary experience suggests that the addition of rituximab may allow for remission induction with a reduction in cumulative cyclophosphamide dose. Combination therapies and newer agents appear promising for the future as our understanding of the immune system and its dysregulation in SLE improves. In this review, we discuss the current standards of care, newer therapies currently in use, and emerging treatments still undergoing development and investigation. We conclude by discussing our guidelines for treatment at the present time and suggestions for the comprehensive care of children with lupus nephritis.
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