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Open Access 26-07-2023 | Pioglitazone | Brief Report

Pioglitazone, a PPAR-y agonist, as one of the new therapeutic candidates for C3 glomerulopathy

Authors: Elia Balestra, Egidio Barbi, Viola Ceconi, Vittorio Di Maso, Ester Conversano, Marco Pennesi

Published in: Pediatric Nephrology | Issue 1/2024

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Abstract

Background

C3-glomerulopathy (C3G) is a rare pediatric kidney disease characterised by dysregulation of the alternative complement pathway, with glomerular deposition of C3. C3G may often present as a steroid-resistant nephrotic syndrome (SRNS), and there is no established effective therapy: the usual treatment involves corticosteroids and immunosuppressive drugs. Pioglitazone, a PPAR-γ agonist with a protective action on podocytes, was reported in a few cases as helpful in reducing proteinuria when combined with steroids.

Case-Diagnosis/Treatment

We report the case of a 13-year-old girl with silent past medical history who presented with SRNS. A kidney biopsy showed findings indicative of C3G. A low sodium diet and angiotensin-converting enzyme inhibitor were started; immunosuppressive treatment with mycophenolate mofetil (MMF) was administered due to the cortico-resistance. Because of poor response to the immunosuppressant, a trial with eculizumab was attempted without significant response and persistence of proteinuria in the nephrotic range. A further therapeutic trial was performed with tacrolimus with no disease remission. Due to a severe deterioration in her condition, the girl was hospitalized and treated with high-dose steroid bolus. A daily dose of oral prednisone and MMF were re-started without benefit with persistent levels of nephrotic range proteinuria. The administration of pioglitazone consistently lowered proteinuria levels for the first time since the onset of the disease, with a maintenance of the effect and normalization (< 0.15 g/24 h) at the 10-month follow-up.

Conclusions

In this patient affected by C3G, pioglitazone proved effective in reducing proteinuria levels.
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Metadata
Title
Pioglitazone, a PPAR-y agonist, as one of the new therapeutic candidates for C3 glomerulopathy
Authors
Elia Balestra
Egidio Barbi
Viola Ceconi
Vittorio Di Maso
Ester Conversano
Marco Pennesi
Publication date
26-07-2023
Publisher
Springer Berlin Heidelberg
Published in
Pediatric Nephrology / Issue 1/2024
Print ISSN: 0931-041X
Electronic ISSN: 1432-198X
DOI
https://doi.org/10.1007/s00467-023-06088-5

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