Outcomes of steroid-resistant nephrotic syndrome in children not treated with intensified immunosuppression
© 2022. The Author(s)..
BACKGROUND: The aim of the current PodoNet registry analysis was to evaluate the outcome of steroid-resistant nephrotic syndrome (SRNS) in children who were not treated with intensified immunosuppression (IIS), focusing on the potential for spontaneous remission and the role of angiotensin blockade on proteinuria reduction.
METHODS: Ninety-five pediatric patients who did not receive any IIS were identified in the PodoNet Registry. Competing risk analyses were performed on 67 patients with nephrotic-range proteinuria at disease onset to explore the cumulative rates of complete or partial remission or progression to kidney failure, stratified by underlying etiology (genetic vs. non-genetic SRNS). In addition, Cox proportional hazard analysis was performed to identify factors predicting proteinuria remission.
RESULTS: Eighteen of 31 (58.1%) patients with non-genetic SRNS achieved complete remission without IIS, with a cumulative likelihood of 46.2% at 1 year and 57.7% at 2 years. Remission was sustained in 11 children, and only two progressed to kidney failure. In the genetic subgroup (n = 27), complete resolution of proteinuria occurred very rarely and was never sustained; 6 (21.7%) children progressed to kidney failure at 3 years. Almost all children (96.8%) received proteinuria-lowering renin-angiotensin-aldosterone system (RAAS) antagonist treatment. On antiproteinuric treatment, partial remission was achieved in 7 of 31 (22.6%) children with non-genetic SRNS and 9 of 27 children (33.3%) with genetic SRNS.
CONCLUSION: Our results demonstrate that spontaneous complete remission can occur in a substantial fraction of children with non-genetic SRNS and milder clinical phenotype. RAAS blockade increases the likelihood of partial remission of proteinuria in all forms of SRNS. A higher resolution version of the Graphical abstract is available as Supplementary information.
| Medienart: |
E-Artikel |
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| Erscheinungsjahr: |
2023 |
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| Erschienen: |
2023 |
| Enthalten in: |
Zur Gesamtaufnahme - volume:38 |
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| Enthalten in: |
Pediatric nephrology (Berlin, Germany) - 38(2023), 5 vom: 31. Mai, Seite 1499-1511 |
| Sprache: |
Englisch |
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| Beteiligte Personen: |
Trautmann, Agnes [VerfasserIn] |
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| Links: |
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| Themen: |
Antiproteinuric treatment |
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| Anmerkungen: |
Date Completed 31.03.2023 Date Revised 14.04.2023 published: Print-Electronic Citation Status MEDLINE |
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| doi: |
10.1007/s00467-022-05762-4 |
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| funding: |
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| Förderinstitution / Projekttitel: |
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NLM348295219 |
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| 100 | 1 | |a Trautmann, Agnes |e verfasserin |4 aut | |
| 245 | 1 | 0 | |a Outcomes of steroid-resistant nephrotic syndrome in children not treated with intensified immunosuppression |
| 264 | 1 | |c 2023 | |
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| 500 | |a Date Completed 31.03.2023 | ||
| 500 | |a Date Revised 14.04.2023 | ||
| 500 | |a published: Print-Electronic | ||
| 500 | |a Citation Status MEDLINE | ||
| 520 | |a © 2022. The Author(s). | ||
| 520 | |a BACKGROUND: The aim of the current PodoNet registry analysis was to evaluate the outcome of steroid-resistant nephrotic syndrome (SRNS) in children who were not treated with intensified immunosuppression (IIS), focusing on the potential for spontaneous remission and the role of angiotensin blockade on proteinuria reduction | ||
| 520 | |a METHODS: Ninety-five pediatric patients who did not receive any IIS were identified in the PodoNet Registry. Competing risk analyses were performed on 67 patients with nephrotic-range proteinuria at disease onset to explore the cumulative rates of complete or partial remission or progression to kidney failure, stratified by underlying etiology (genetic vs. non-genetic SRNS). In addition, Cox proportional hazard analysis was performed to identify factors predicting proteinuria remission | ||
| 520 | |a RESULTS: Eighteen of 31 (58.1%) patients with non-genetic SRNS achieved complete remission without IIS, with a cumulative likelihood of 46.2% at 1 year and 57.7% at 2 years. Remission was sustained in 11 children, and only two progressed to kidney failure. In the genetic subgroup (n = 27), complete resolution of proteinuria occurred very rarely and was never sustained; 6 (21.7%) children progressed to kidney failure at 3 years. Almost all children (96.8%) received proteinuria-lowering renin-angiotensin-aldosterone system (RAAS) antagonist treatment. On antiproteinuric treatment, partial remission was achieved in 7 of 31 (22.6%) children with non-genetic SRNS and 9 of 27 children (33.3%) with genetic SRNS | ||
| 520 | |a CONCLUSION: Our results demonstrate that spontaneous complete remission can occur in a substantial fraction of children with non-genetic SRNS and milder clinical phenotype. RAAS blockade increases the likelihood of partial remission of proteinuria in all forms of SRNS. A higher resolution version of the Graphical abstract is available as Supplementary information | ||
| 650 | 4 | |a Journal Article | |
| 650 | 4 | |a Research Support, Non-U.S. Gov't | |
| 650 | 4 | |a Antiproteinuric treatment | |
| 650 | 4 | |a Children | |
| 650 | 4 | |a Nephrotic syndrome | |
| 650 | 4 | |a SRNS | |
| 650 | 4 | |a Steroid resistance | |
| 650 | 7 | |a Immunosuppressive Agents |2 NLM | |
| 700 | 1 | |a Seide, Svenja |e verfasserin |4 aut | |
| 700 | 1 | |a Lipska-Ziętkiewicz, Beata S |e verfasserin |4 aut | |
| 700 | 1 | |a Ozaltin, Fatih |e verfasserin |4 aut | |
| 700 | 1 | |a Szczepanska, Maria |e verfasserin |4 aut | |
| 700 | 1 | |a Azocar, Marta |e verfasserin |4 aut | |
| 700 | 1 | |a Jankauskiene, Augustina |e verfasserin |4 aut | |
| 700 | 1 | |a Zurowska, Alexandra |e verfasserin |4 aut | |
| 700 | 1 | |a Caliskan, Salim |e verfasserin |4 aut | |
| 700 | 1 | |a Saeed, Bassam |e verfasserin |4 aut | |
| 700 | 1 | |a Morello, William |e verfasserin |4 aut | |
| 700 | 1 | |a Emma, Francesco |e verfasserin |4 aut | |
| 700 | 1 | |a Litwin, Mieczyslaw |e verfasserin |4 aut | |
| 700 | 1 | |a Tsygin, Alexey |e verfasserin |4 aut | |
| 700 | 1 | |a Fomina, Svitlana |e verfasserin |4 aut | |
| 700 | 1 | |a Wasilewska, Anna |e verfasserin |4 aut | |
| 700 | 1 | |a Melk, Anette |e verfasserin |4 aut | |
| 700 | 1 | |a Benetti, Elisa |e verfasserin |4 aut | |
| 700 | 1 | |a Gellermann, Jutta |e verfasserin |4 aut | |
| 700 | 1 | |a Stajic, Natasa |e verfasserin |4 aut | |
| 700 | 1 | |a Tkaczyk, Marcin |e verfasserin |4 aut | |
| 700 | 1 | |a Baiko, Sergey |e verfasserin |4 aut | |
| 700 | 1 | |a Prikhodina, Larisa |e verfasserin |4 aut | |
| 700 | 1 | |a Csaicsich, Dagmar |e verfasserin |4 aut | |
| 700 | 1 | |a Medynska, Anna |e verfasserin |4 aut | |
| 700 | 1 | |a Krisam, Regina |e verfasserin |4 aut | |
| 700 | 1 | |a Breitschwerdt, Heike |e verfasserin |4 aut | |
| 700 | 1 | |a Schaefer, Franz |e verfasserin |4 aut | |
| 700 | 0 | |a PodoNet Consortium |e verfasserin |4 aut | |
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