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Case Reports
. 2025 Oct;40(10):3081-3084.
doi: 10.1007/s00467-025-06799-x. Epub 2025 May 9.

Idiopathic fibrillary glomerulonephritis in pediatric patients: addressing treatment challenges in a 12-year-old girl

Ester Cholbi Vives  1 , Javier Martín Benlloch  2 , Josselyn Hernández Chinchilla  3 , Vicent Martínez Cózar  3 , Judith Pérez Rojas  3 , Pedro Ortega López  2
Affiliations
Case Reports

Idiopathic fibrillary glomerulonephritis in pediatric patients: addressing treatment challenges in a 12-year-old girl

Ester Cholbi Vives et al. Pediatr Nephrol. 2025 Oct.

Abstract

A 12-year-old girl presented with proteinuria and peripheral edema. Initial evaluation did not identify a clear underlying etiology. Upon the diagnosis of nephrotic syndrome, corticosteroid therapy was initiated. However, the therapeutic response was suboptimal, with persistent proteinuria, the onset of de novo microhematuria, and progressive kidney dysfunction. A kidney biopsy was performed, which led to the diagnosis of fibrillary glomerulopathy, a rare pediatric condition characterized histologically by the presence of fibrillary deposits and positive immunostaining for DNAJB9. There is no established effective treatment for this condition. In our case, the patient was treated with antiproteinuric and calcineurin inhibitors, resulting in complete resolution of microhematuria, normalization of kidney function, and substantial improvement in proteinuria.

Keywords: Fibrillary glomerulopathy; Immunosuppressive therapy; Nephrotic syndrome; Pediatric patients.

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Conflict of interest statement

Declarations. Competing interests: The authors declare no competing interests.

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References

    1. Rosenstock JL, Markowitz GS, Valeri AM, Sacchi G, Appel GB, D’Agati VD (2003) Fibrillary and immunotactoid glomerulonephritis: distinct entities with different clinical and pathologic features. Kidney Int 63:1450–1461. https://doi.org/10.1046/j.1523-1755.2003.00853.x - DOI - PubMed
    1. Rosenstock JL, Markowitz GS (2019) Fibrillary glomerulonephritis: an update. Kidney Int Rep 4:917–922. https://doi.org/10.1016/j.ekir.2019.04.013 - DOI - PubMed - PMC
    1. Payan Schober F, Jobson MA, Poulton CJ, Singh HK, Nickeleit V, Falk RJ, Jennette JC, Nachman PH, Pendergraft WF 3rd (2017) Clinical features and outcomes of a racially diverse population with fibrillary glomerulonephritis. Am J Nephrol 45:248–256. https://doi.org/10.1159/000455390 - DOI - PubMed
    1. Javaugue V, Karras A, Glowacki F, McGregor B, Lacombe C, Goujon JM, Ragot S, Aucouturier P, Touchard G, Bridoux F (2013) Long-term kidney disease outcomes in fibrillary glomerulonephritis: a case series of 27 patients. Am J Kidney Dis 62:679–690. https://doi.org/10.1053/j.ajkd.2013.03.031 - DOI - PubMed
    1. El Ters M, Bobart SA, Cornell LD, Leung N, Bentall A, Sethi S, Fidler M, Grande J, Hernandez LH, Cosio FG, Zand L, Amer H, Fervenza FC, Nasr SH, Alexander MP (2020) Recurrence of DNAJB9-positive fibrillary glomerulonephritis after kidney transplantation: a case series. Am J Kidney Dis 76:500–510. https://doi.org/10.1053/j.ajkd.2020.01.018 - DOI - PubMed

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