Journal of the American Society of Nephrology : JASN · 2026
Anti-podocin and Anti-KIRREL1 Antibodies and Steroid Resistance, FSGS, and Disease Recurrence after Transplantation in Autoimmune Podocytopathies.
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Raglianti Valentina, Angelotti Maria Lucia, Cirillo Luigi, Becherucci Francesca, De Chiara Letizia, Mazzinghi Benedetta, Di Lorenzo Antea, Salvatore Giulia, Antonelli Giulia, Conte Carolina, Melica Maria Elena, Palazzo Viviana, Peired Anna Julie, Dafichi Tommaso, Diaz-Bulnes Paula, Gnagni Aurora, Malatesta Luca, Allinovi Marco, Caroti Leonardo, Manonelles Anna, Cruzado Josep Maria, Giannese Domenico, Somma Chiara, Mazzierli Tommaso, Lange-Sperandio Baerbel, Baccini Federica, Lasagni Laura, Lazzeri Elena, Anders Hans-Joachim, Romagnani Paola
Аннотация
KEY POINTS: Anti-slit autoantibody profiles identified different podocytopathy phenotypes, with anti-podocin/KIRREL1 associated with FSGS and steroid resistance. Anti-nephrin, anti-podocin, and anti-KIRREL1 testing improved risk stratification, identifying patients at a risk of CKD and post-transplant recurrence.
BACKGROUND: Anti-nephrin, anti-podocin, and anti-KIRREL1 antibodies are increasingly recognized as causes of autoimmune podocytopathies, yet autoantibody-specific clinical phenotypes remain poorly defined.
METHODS: We conducted a multicenter study integrating serology and super-resolution microscopy in 471 samples, including 213 cases with 146 kidney biopsies (minimal change n =63, FSGS n =83), and sera from 128 patients (minimal changes n =30, FSGS n =42, idiopathic nephrotic syndrome or isolated proteinuria n =56), including 61 paired specimens plus 258 controls (75 kidney biopsies, 200 sera, including 17 paired specimens). Paired specimens were used to assess ELISA performance by comparing serum results with IgG colocalization of target proteins within the filtration slit using receiver operating characteristic curves. All assays showed high specificity but lower sensitivity than super-resolution microscopy. Clinical features and long-term outcomes associated with individual autoantibodies and their combinations were also evaluated.
RESULTS: Anti-slit diaphragm antibodies were detected in 67/213 patients: 63% had anti-nephrin, 9% anti-podocin, or 3% anti-KIRREL1 antibodies only, while 25% had multiple autoantibodies. Isolated anti-nephrin antibodies were associated with minimal changes (91%), steroid sensitivity (88%), and absence of CKD over 120 months. In contrast, anti-podocin and/or anti-KIRREL1 antibodies correlated with FSGS (84%), steroid resistance (58%), CKD progression (36%), and kidney failure (24%). Among 26 transplant recipients with FSGS, early post-transplant recurrence occurred in 83% of autoantibodies carriers and in 5% of the others. Recurrent patients showed anti-podocin/KIRREL1 antibodies, with or without anti-nephrin.
CONCLUSIONS: These findings suggest that anti-podocin and anti-KIRREL1 antibodies associate with steroid resistance, FSGS, and disease recurrence after transplantation in autoimmune podocytopathies.
BACKGROUND: Anti-nephrin, anti-podocin, and anti-KIRREL1 antibodies are increasingly recognized as causes of autoimmune podocytopathies, yet autoantibody-specific clinical phenotypes remain poorly defined.
METHODS: We conducted a multicenter study integrating serology and super-resolution microscopy in 471 samples, including 213 cases with 146 kidney biopsies (minimal change n =63, FSGS n =83), and sera from 128 patients (minimal changes n =30, FSGS n =42, idiopathic nephrotic syndrome or isolated proteinuria n =56), including 61 paired specimens plus 258 controls (75 kidney biopsies, 200 sera, including 17 paired specimens). Paired specimens were used to assess ELISA performance by comparing serum results with IgG colocalization of target proteins within the filtration slit using receiver operating characteristic curves. All assays showed high specificity but lower sensitivity than super-resolution microscopy. Clinical features and long-term outcomes associated with individual autoantibodies and their combinations were also evaluated.
RESULTS: Anti-slit diaphragm antibodies were detected in 67/213 patients: 63% had anti-nephrin, 9% anti-podocin, or 3% anti-KIRREL1 antibodies only, while 25% had multiple autoantibodies. Isolated anti-nephrin antibodies were associated with minimal changes (91%), steroid sensitivity (88%), and absence of CKD over 120 months. In contrast, anti-podocin and/or anti-KIRREL1 antibodies correlated with FSGS (84%), steroid resistance (58%), CKD progression (36%), and kidney failure (24%). Among 26 transplant recipients with FSGS, early post-transplant recurrence occurred in 83% of autoantibodies carriers and in 5% of the others. Recurrent patients showed anti-podocin/KIRREL1 antibodies, with or without anti-nephrin.
CONCLUSIONS: These findings suggest that anti-podocin and anti-KIRREL1 antibodies associate with steroid resistance, FSGS, and disease recurrence after transplantation in autoimmune podocytopathies.