ArticleClinical journal of the American Society of Nephrology : CJASN2020
Response to First Course of Intensified Immunosuppression in Genetically Stratified Steroid Resistant Nephrotic Syndrome.
Article in Clinical journal of the American Society of Nephrology : CJASN, 2020. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 32 papers.
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32 citing papers in PubMed, 49 citations in OpenAlex.
- Monogenic variants drive low recurrence risk in pediatric steroid-resistant nephrotic syndrome after kidney transplantation: a high-consanguinity cohort.Pediatric nephrology (Berlin, Germany) · 2026Article
- Rituximab in Pediatric Steroid-Resistant Nephrotic Syndrome: How Early is Early Enough?Kidney international reports · 2026Article
- Treatment outcomes and ongoing pediatric trials in steroid-resistant nephrotic syndrome.Pediatric nephrology (Berlin, Germany) · 2026Review
- Pursuing the optimal strategy for immunosuppression discontinuation in children treated with rituximab for frequently relapsing, steroid-dependent nephrotic syndrome.Pediatric nephrology (Berlin, Germany) · 2026Article
- Kidney Survival in Children With Steroid-Resistant Nephrotic Syndrome Treated by Rituximab.Kidney international reports · 2026Article
- Genetic mutation patterns in Indonesian children with primary steroid-resistant nephrotic syndrome.Pediatric nephrology (Berlin, Germany) · 2026Article
- KDOQI US Commentary on the KDIGO 2025 Clinical Practice Guideline for the Management of Nephrotic Syndrome in Children.American journal of kidney diseases : the official journal of the National Kidney Foundation · 2026Review
- Efficacy of Calcineurin Inhibition in Children With Steroid-Resistant Nephrotic Syndrome.Kidney international reports · 2025Article
- Childhood idiopathic nephrotic syndrome: recent advancements shaping future guidelines.Pediatric nephrology (Berlin, Germany) · 2025Review
- Outcomes and prognostic factors in childhood-onset steroid-resistant nephrotic syndrome: a retrospective single-center study.Pediatric nephrology (Berlin, Germany) · 2025Article
- Long-Term Outcomes in Nephrotic Syndrome by Kidney Biopsy Diagnosis and Proteinuria.Journal of the American Society of Nephrology : JASN · 2025Article
- Article
- Clinical Decision-Making About Immunosuppressive Treatment in Focal Segmental Glomerulosclerosis.Kidney medicine · 2025Article
- Clinical characteristics and prognosis of steroid-resistant nephrotic syndrome in children: a multi-center retrospective study.Italian journal of pediatrics · 2024Article
- Clinical characteristics and favorable treatment responses of recurrent focal segmental glomerulosclerosis or steroid-resistant nephrotic syndrome in children after kidney transplantation.Pediatric nephrology (Berlin, Germany) · 2024Article
- Rare heterozygous variants in paediatric steroid resistant nephrotic syndrome - a population-based analysis of their significance.Scientific reports · 2024Article
- The Role of Genetic Testing in Adult CKD.Journal of the American Society of Nephrology : JASN · 2024Article
- A genomic association study revealing subphenotypes of childhood steroid-sensitive nephrotic syndrome in a larger genomic sequencing cohort.Genes & diseases · 2024Article
- A Review of Focal Segmental Glomerulosclerosis Classification With a Focus on Genetic Associations.Kidney medicine · 2024Article
- Implementation and Feasibility of Clinical Genome Sequencing Embedded Into the Outpatient Nephrology Care for Patients With Proteinuric Kidney Disease.Kidney international reports · 2023Article
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12 authors at 4 institutions in 2 countries.
Funding
Abstract
BACKGROUND AND
objectivesIntensified immunosuppression in steroid-resistant nephrotic syndrome is broadly applied, with disparate outcomes. This review of patients from the United Kingdom National Study of Nephrotic Syndrome cohort aimed to improve disease stratification by determining, in comprehensively genetically screened patients with steroid-resistant nephrotic syndrome, if there is an association between response to initial intensified immunosuppression and disease progression and/or post-transplant recurrence. DESIGN, SETTING, PARTICIPANTS, & MEASUREMENTS: Pediatric patients with steroid-resistant nephrotic syndrome were recruited
resultsOf 271 patients, 178 (92 males, median onset age 4.7 years) received intensified immunosuppression with response available. A total of 4% of patients with monogenic disease showed complete response, compared with 25% of genetic-testing-negative patients (
conclusionsPatients with monogenic steroid-resistant nephrotic syndrome had a poor therapeutic response and no post-transplant recurrence. In genetic-testing-negative patients, there was an association between response to first intensified immunosuppression and long-term outcome. Patients with complete response rarely progressed to kidney failure, whereas nonresponders had poor kidney survival and a high post-transplant recurrence rate. Patients with secondary steroid resistance were more likely to respond, particularly to rituximab.
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