Outcomes of steroid-resistant nephrotic syndrome in children not treated with intensified immunosuppression.

Abstract

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.

• Authors: Agnes Trautmann, Svenja Seide, Beata S Lipska-Ziętkiewicz, Fatih Ozaltin, Maria Szczepanska, Marta Azocar, Augustina Jankauskiene, Alexandra Zurowska, Salim Caliskan, Bassam Saeed, William Morello, Francesco Emma, Mieczyslaw Litwin, Alexey Tsygin, Svitlana Fomina, Anna Wasilewska, Anette Melk, Elisa Benetti, Jutta Gellermann, Natasa Stajic, Marcin Tkaczyk, Sergey Baiko, Larisa Prikhodina, Dagmar Csaicsich, Anna Medynska, Regina Krisam, Heike Breitschwerdt, Franz Schaefer, PodoNet Consortium
• Journal: Pediatric nephrology (Berlin, Germany) (Pediatr Nephrol) Vol. 38 Issue 5 Pg. 1499-1511 (05 2023) ISSN: 1432-198X [Electronic] Germany
• PMID: 36315273 (Publication Type: Journal Article, Research Support, Non-U.S. Gov't)
• Copyright: © 2022. The Author(s).

Chemical References

• Immunosuppressive Agents

Topics

• Child
• Humans
• Nephrotic Syndrome (drug therapy, genetics)
• Immunosuppressive Agents (therapeutic use)
• Proteinuria (drug therapy, etiology)
• Immunosuppression Therapy
• Renal Insufficiency (drug therapy)
• Drug Resistance