The implications of focal segmental glomerulosclerosis in children with IgA nephropathy
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REVIEW
The implications of focal segmental glomerulosclerosis in children with IgA nephropathy Hernán Trimarchi 1
&
Rosanna Coppo 2
Received: 28 September 2019 / Revised: 30 October 2019 / Accepted: 31 October 2019 # IPNA 2019
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. Keywords IgA nephropathy . Proteinuria . Glomerulosclerosis . Hypertension . Chronic kidney disease . Endocapillary hypercellularity . Mesangial hypercellularity
Introduction IgAN is a glomerular disease which usually presents in children with episodes of macroscopic hematuria in coincidence with upper respiratory tract infections or persistent mild microscopic hematuria [1]. The clinical course is mostly benign within the pediatric age; hence, pediatricians have for years given little importance to these clinical features, without performing diagnostic renal biopsies and/or treating these children. Over the last decades, it has become clear that, even in childhood, several cases still progress to end-stage kidney disease (ESKD), while others experience it in young adult age. Hernán Trimarchi and Rosanna Coppo have contributed equally to the development of the manuscript * Hernán Trimarchi [email protected] 1
Nephrology Service, Hospital Británico de Buenos Aires, Perdriel 74 (1280), Buenos Aires, Argentina
2
Fondazione Ricerca Molinette, Regina Margherita Hospital, Turin, Italy
In the long-term follow-up of the European Validation study of IgAN classification (VALIGA), the combined end point of 50% decline in eGFR or ESKD at 10 years was 9% versus 30% in adults [2], while at 20 years, it was estimated by Kaplan-Meier curve to be 18% in children versus 53% in adults (unpublished data from VALIGA follow-up report) [3]. The median follow-up of VALIGA subjects was prolonged from 4.9 (IQR 2.4–7.9) years to 7.0 (IQR 4.1– 10.8) years. According to this criticism, we reported in R1 the estimate calculated by Kaplan-Meier curve at 20 years [3]. A single-center
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