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The implications of focal segmental glomerulosclerosis in children with IgA nephropathy

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Abstract

Focal segmental glomerular sclerotic lesions in IgA nephropathy (IgAN), considered for years a chronic histologic feature related to proteinuria in remnant nephrons without any active role in the pathogenesis and progression of glomerular damage of IgAN, have been recently reconsidered. The Oxford classification of IgAN reported it as the “S” score and found it to be an independent risk factor for progression of IgAN. Its prognostic value was confirmed also in children. The identification of some histologic subvariants of the S lesion has produced interesting insights into different pathogenetic mechanisms of glomerular damage in IgAN. Tip lesion and podocyte hypertrophy are considered secondary to active podocytopathy and are correlated with higher levels of proteinuria and a faster decline in glomerular filtration rate. Moreover, endocapillary and mesangial hypercellularity might contribute in children with IgAN to formation and progression of S lesions. Considering the pathophysiology of these processes, children with some S features may benefit not only from nephroprotective measures but also from immunosuppression.

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Author notes

  1. Hernán Trimarchi and Rosanna Coppo have contributed equally to the development of the manuscript

Authors and Affiliations

  1. Nephrology Service, Hospital Británico de Buenos Aires, Perdriel 74 (1280), Buenos Aires, Argentina

    Hernán Trimarchi

  2. Fondazione Ricerca Molinette, Regina Margherita Hospital, Turin, Italy

    Rosanna Coppo

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  1. Hernán Trimarchi
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  2. Rosanna Coppo
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Correspondence to Hernán Trimarchi.

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Hernán Trimarchi has received honoraria from Calliditas and Retrophin. Rosanna Coppo has received honoraria from Calliditas, Retrophin, and Omeros.

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Trimarchi, H., Coppo, R. The implications of focal segmental glomerulosclerosis in children with IgA nephropathy. Pediatr Nephrol 35, 2043–2047 (2020). https://doi.org/10.1007/s00467-019-04414-4

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