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Si us plau utilitzeu sempre aquest identificador per citar o enllaçar aquest document: https://hdl.handle.net/2445/220069
Anti-slit diaphragm antibodies on kidney biopsy identify pediatric patients with steroid-resistant nephrotic syndrome responsive to second-line immunosuppressants
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Podocytopathies represent a group of glomerular disorders associated with minimal changes (MC) or focal segmental glomerulosclerosis (FSGS) lesion patterns at biopsy and heterogeneous responses to steroids. Anti-nephrin antibodies were previously found in such patients, suggesting an autoimmune form of podocytopathy. High resolution confocal microscopy on kidney biopsies of a cohort of 128 pediatric patients revealed localization of IgG along the slit diaphragm in 30% of patients with MC and 25% of those with FSGS, but not in other lesion patterns. Anti-nephrin IgG ELISA assay in the serum and stimulated emission depletion microscopy of kidney biopsies showed IgG-nephrin co-localization only in 77.8% of cases. Similar observations were obtained in a cohort of 48 adult patients with MC or FSGS at kidney biopsy, where IgG-nephrin colocalization was only 44.4%, suggesting the existence of autoantibodies binding to other slit proteins. Patients with anti-slit antibodies showed nephrotic syndrome at onset in 94.4% of cases. Patients with primary steroid-resistance had anti-slit antibodies in 27%, while those with secondary steroid-resistance in 87.5% of cases, irrespective of the histopathological lesion pattern. Steroid-resistant patients with anti-slit antibodies responded to second-line immunosuppressants in 92.3% vs. only 20% of patients that were anti-slit negative. No patient with anti-slit antibodies developed kidney failure vs. 51.7% of those negative for antibodies (66.7% with a genetic cause and 41.2% with a non-genetic cause). Thus, the detection of anti-slit antibodies can identify patients with an autoimmune podocytopathy responsive to treatment with second-line immunosuppressants, irrespective of the histopathological lesion pattern at biopsy.
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RAGLIANTI, Valentina, ANGELOTTI, Maria lucia, CIRILLO, Luigi, RAVAGLIA, Fiammetta, LANDINI, Samuela, PALAZZO, Viviana, MELICA, Maria elena, ANTONELLI, Giulia, CONTE, Carolina, BUTI, Elisa, ERRICHIELLO, Carmela, DE CHIARA, Letizia, PEIRED, Anna julie, LASAGNI, Laura, BUCCOLIERO, Anna maria, ALLINOVI, Marco, MANONELLES MONTERO, Anna, CRUZADO, Josep ma., BRUSCHI, Maurizio, GHIGGERI, Gian marco, ANGELETTI, Andrea, ANDERS, Hans-joachim, LAZZERI, Elena, MAZZINGHI, Benedetta, BECHERUCCI, Francesca, ROMAGNANI, Paola. Anti-slit diaphragm antibodies on kidney biopsy identify pediatric patients with steroid-resistant nephrotic syndrome responsive to second-line immunosuppressants. _Kidney International_. 2024. Vol. 106, núm. 6, pàgs. 1124-1134. [consulta: 23 de gener de 2026]. ISSN: 0085-2538. [Disponible a: https://hdl.handle.net/2445/220069]