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Therapy for children with henoch-schonlein purpura nephritis: a systematic review
Marco Zaffanello1, Milena Brugnara, Massimo Franchini
1Department of Paediatrics, University of Verona, Italy. marco.zaffanello@univr.it
Insights
Kidney complications in Henoch-Schönlein purpura (HSP) require careful monitoring in children. While steroids and cyclophosphamide show promise, further research is needed for other treatments like ACE-I and plasmapheresis in HSP nephritis.
Area of Science:
- Pediatric Nephrology
- Rheumatology
- Immunology
Background:
- Henoch-Schönlein purpura (HSP) can cause significant kidney involvement (nephritis) in children, though less common than in adults.
- Morbidity associated with HSP nephritis in children necessitates vigilant follow-up and evaluation.
- Current therapeutic strategies for HSP nephritis range from corticosteroids to combined immunosuppressive regimens.
Purpose of the Study:
- To review and evaluate current and potential treatment options for Henoch-Schönlein purpura nephritis in pediatric patients.
- To identify established treatments, promising alternatives, and areas requiring further investigation for childhood HSP nephritis.
Main Methods:
- Review of existing literature on therapeutic interventions for HSP nephritis in children.
- Analysis of drug efficacy, including steroids (methylprednisolone, prednisone), cyclophosphamide, and other immunosuppressants.
- Evaluation of non-pharmacological treatments such as plasmapheresis.
Main Results:
- Corticosteroids (methylprednisolone pulse, oral prednisone) are commonly used, often in combination with immunosuppressants.
- Cyclophosphamide has demonstrated efficacy in a recent randomized controlled trial for HSP nephritis.
- Plasmapheresis showed potential in delaying kidney disease progression in a multicenter study, but further evidence is needed for other agents like ACE-I, azathioprine, mycophenolate mofetil, urokinase, IVIG, and nutritional supplements.
Conclusions:
- Treatment for childhood HSP nephritis varies, with steroids and cyclophosphamide being key components.
- Further well-designed studies are essential to establish the efficacy of emerging therapies, including ACE inhibitors and plasmapheresis, for pediatric HSP nephritis.
- Long-term follow-up remains crucial for managing renal complications in children with HSP.
Abstract:
Although severe kidney involvement in children with Henoch-Shonlein purpura (HSP) is rarer than that in adults, morbidity should not be underevaluated and follow-up is mandatory. Some drugs are introduced as well-defined treatment options, others can be promising therapeutic alternatives. Therapy of HSP nephritis in children can range from simply steroids to combined immunosuppressant treatments. The prophylactic treatment for renal complication of patients with HSP has been sometimes suggested, but with conflicting results and ultimately not clearly proven. The treatment of overt HSP nephritis includes steroids and other immunosuppressant drugs. Methylprednisolone pulse therapy and prednisone per os are tested drugs. These steroids could be used in combination with other immunosuppressant drugs, such as cyclosporin A and cyclophosphamide. Unfortunately, of these two drugs, only cyclophosphamide is demonstrated as effective in a recent randomized controlled trial. However, since there are insufficient data and unstructured study designs, ACE-I, azathioprine, mycophenolate mofetil, and urokinase need to be more tested in childhood HSP nephritis. In addition to drugs, other techniques are used to treat the severe form of nephritis. Of these, in a multicenter study, plasmapheresis demonstrated efficacy in delaying the progression of kidney disease. However, no convincing studies have been made to date concerning either intravenous immunoglobulin, factor XIII administration, antioxidant vitamin E, and fish oil to treat HSP nephritis.
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