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1. Evidence from the large VALIGA cohort validates the subclassification of focal segmental glomerulosclerosis in IgA nephropathy

Author

Coppo, R., Feehaly, J., Troyanov, S., Cattran, D.C., Cook, H.T., Roberts, I., Radcliffe, John, Russo, M.L., Tesar, V., Maixnerova, D., Lundberg, S., Gesualdo, L., Emma, F., Fuiano, L., Beltrame, G., Rollino, C., Amore, A., Camilla, R., Peruzzi, L., Praga, M., Feriozzi, S., Polci, R., Segoloni, G., Colla, L., Pani, A., Piras, D., Angioi, A., Cancarini, G., Ravera, S., Durlik, M., Moggia, E., Ballarin, J., Di Giulio, S., Pugliese, F., Serriello, I., Caliskan, Y., Sever, M., Kilicaslan, I., Locatelli, F., Del Vecchio, L., Wetzels, J.F.M., Peters, H., Berg, U., Carvalho, F., da Costa Ferreira, A.C., Maggio, M., Wiecek, A., Ots-Rosenberg, M., Magistroni, R., Topaloglu, R., Bilginer, Y., D’Amico, M., Papagianni, K., Stangou, M., Giacchino, F., Goumenos, D., Papasotirious, M., Kalliakmani, P., Gerolymos, M., Galesic, K., Toric, L., Geddes, C., Siamopoulos, K., Balafa, O., Galliani, M., Stratta, P., Quaglia, M., Bergia, R., Cravero, R., Salvadori, M., Cirami, L., Fellstrom, B., Kloster Smerud, H., Ferrario, F., Stellato, T., Egido, J., Martin, C., Floege, J., Eitner, F., Lupo, A., Bernich, P., Menè, P., Morosetti, M., van Kooten, C., Rabelink, T., Reinders, M.E.J., Boria Grinyo, J.M., Cusinato, S., Benozzi, L., Savoldi, S., Licata, C., Mizerska-Wasiak, M., Roszkowska-Blaim, M., Hryszko, T., Klinger, M., Kamińska, D., Krajewska, M., Martina, G., Messuerotti, A., Dal Canton, A., Esposito, C., Migotto, C., Triolo, G., Mariano, F., Pozzi, C., Boero, R., Cambier, A., Bellur, S., Mazzucco, G., Giannakakis, C., Honsova, E., Sundelin, B., Di Palma, A.M., Diomedi-Casadei, F., Gutiérrez, E., Asunis, A.M., Barratt, J., Tardanico, R., Perkowska-Ptasinska, A., Arce Terroba, J., Fortunato, M., Pantzaki, A., Ozluk, Y., Steenbergen, E., Soderberg, M., Riispere, Z., Furci, L., Orhan, D., Kipgen, D., Casartelli, D., Galesic Ljubanovic, D., Gakiopoulou, H., Bertoni, E., Cannata Ortiz, P., Karkoszka, H., Groene, H.J., Stoppacciaro, A., Bajema, I., Bruijn, J., Fulladosa Oliveras, X., Maldyk, J., Ioachim, E., Royal, V., Bellur, Shubha S., Troyanov, Stéphan, Vorobyeva, Olga, Coppo, Rosanna, and Roberts, Ian S.D.

Published
2024
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2. Renal Recovery for Patients with ANCA-Associated Vasculitis and Low eGFR in the ADVOCATE Trial of Avacopan

Author

Peh, C. Au, Chakera, A., Cooper, B., Kurtkoti, J., Langguth, D., Levidiotis, V., Luxton, G., Mount, P., Mudge, D., Noble, E., Phoon, R., Ranganathan, D., Ritchie, A., Ryan, J., Suranyi, M., Rosenkranz, A., Lhotta, K., Kronbichler, A., Demoulin, N., Bovy, C., Hellemans, R., Hougardy, J., Sprangers, B., Wissing, K., Pagnoux, C., Barbour, S., Brachemi, S., Cournoyer, S., Girard, L., Laurin, L., Liang, P., Philibert, D., Walsh, M., Tesar, V., Becvar, R., Horak, P., Rychlik, I., Szpirt, W., Dieperink, H., Gregersen, J., Ivarsen, P., Krarup, E., Lyngsoe, C., Rigothier, C., Augusto, J., Belot, A., Chauveau, D., Cornec, D., Jourde-Chiche, N., Ficheux, M., Karras, A., Klein, A., Maurier, F., Mesbah, R., Moranne, O., Neel, A., Quemeneur, T., Saadoun, D., Terrier, B., Zaoui, P., Schaier, M., Benck, U., Bergner, R., Busch, M., Floege, J., Grundmann, F., Haller, H., Haubitz, M., Hellmich, B., Henes, J., Hohenstein, B., Hugo, C., Iking-Konert, C., Arndt, F., Kubacki, T., Kotter, I., Lamprecht, P., Lindner, T., Halbritter, J., Mehling, H., Schönermarck, U., Venhoff, N., Vielhauer, V., Witzke, O., Szombati, I., Szucs, G., Garibotto, G., Alberici, F., Brunetta, E., Dagna, L., De Vita, S., Emmi, G., Gabrielli, A., Manenti, L., Pieruzzi, F., Roccatello, D., Salvarani, C., Dobashi, H., Atsumi, T., Fujimoto, S., Hagino, N., Ihata, A., Kaname, S., Kaneko, Y., Katagiri, A., Katayama, M., Kirino, Y., Kitagawa, K., Komatsuda, A., Kono, H., Kurasawa, T., Matsumura, R., Mimura, T., Morinobu, A., Murakawa, Y., Naniwa, T., Nanki, T., Ogawa, N., Oshima, H., Sada, K., Sugiyama, E., Takeuchi, T., Taki, H., Tamura, N., Tsukamoto, T., Yamagata, K., Yamamura, M., van Daele, P., Rutgers, A., Teng, Y., Walker, R., Chua, I., Collins, M., Rabindranath, K., de Zoysa, J., Svensson, M., Grevbo, B., Kalstad, S., Little, M., Clarkson, M., Molloy, E., Pamplona, I. Agraz, Anton, J., Lucia, V. Barrio, Ciggaran, S., Cid, M. Cinta, Encarnacion, M. Diaz, Oliveras, X. Fulladosa, Soler, M. Jose, Rusinol, H. Marco, Praga, M., Porras, L. Quintana, Segarra, A., Bruchfeld, A., Segelmark, M., Soveri, I., Thomaidi, E., Westman, K., Neumann, T., Burnier, M., Daikeler, T., Dudler, J., Hauser, T., Seeger, H., Vogt, B., Jayne, D., Burton, J., Al Jayyousi, R., Amin, T., Andrews, J., Baines, L., Brogan, P., Dasgupta, B., Doulton, T., Flossmann, O., Griffin, S., Harper, J., Harper, L., Kidder, D., Klocke, R., Lanyon, P., Luqmani, R., McLaren, J., Makanjuola, D., McCann, L., Nandagudi, A., Selvan, S., O'Riordan, E., Patel, M., Patel, R., Pusey, C., Rajakariar, R., Robson, J., Robson, M., Salama, A., Smyth, L., Sznajd, J., Taylor, J., Merkel, P., Sreih, A., Belilos, E., Bomback, A., Carlin, J., Chen Lin, Y. Chang, Derebail, V., Dragoi, S., Dua, A., Forbess, L., Geetha, D., Gipson, P., Gohh, R., Greenwood, G.T., Hugenberg, S., Jimenez, R., Kaskas, M., Kermani, T., Kivitz, A., Koening, C., Langford, C., Marder, G., Mohamed, A., Monach, P., Neyra, N., Niemer, G., Niles, J., Obi, R., Owens, C., Parks, D., Podoll, A., Rovin, B., Sam, R., Shergy, W., Silva, A., Specks, U., Spiera, R., Springer, J., Striebich, C., Swarup, A., Thakar, S., Tiliakos, A., Tsai, Y., Waguespack, D., Wasko, M. Chester, Cortazar, Frank B., Niles, John L., Jayne, David R.W., Merkel, Peter A., Bruchfeld, Annette, Yue, Huibin, Schall, Thomas J., and Bekker, Pirow

Published
2023
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3. Application of the International IgA Nephropathy Prediction Tool one or two years post-biopsy

Author

Russo, M.L., Troyanov, S., Cook, H.T., Roberts, I., Tesar, V., Maixnerova, D., Lundberg, S., Gesualdo, L., Emma, F., Fuiano, L., Beltrame, G., Rollino, C., Amore, A., Camilla, R., Peruzzi, L., Praga, M., Feriozzi, S., Polci, R., Segoloni, G., Colla, L., Pani, A., Piras, D., Angioi, A., Cancarini, G., Ravera, S., Durlik, M., Moggia, E., Ballarin, J., Di Giulio, S., Pugliese, F., Serriello, I., Caliskan, Y., Sever, M., Kilicaslan, I., Locatelli, F., Del Vecchio, L., Wetzels, J.F.M., Peters, H., Berg, U., Carvalho, F., da Costa Ferreira, A.C., Maggio, M., Wiecek, A., Ots-Rosenberg, M., Magistroni, R., Topaloglu, R., Bilginer, Y., D’Amico, M., Stangou, M., Giacchino, F., Goumenos, D., Papachristou, E., Galesic, K., Geddes, C., Siamopoulos, K., Balafa, O., Galliani, M., Stratta, P., Quaglia, M., Bergia, R., Cravero, R., Salvadori, M., Cirami, L., Fellstrom, B., Smerud, H. Kloster, Ferrario, F., Stellato, T., Egido, J., Martin, C., Floege, J., Eitner, F., Lupo, A., Bernich, P., Menè, P., Morosetti, M., van Kooten, C., Rabelink, T., Reinders, M.E.J., Boria Grinyo, J.M., Cusinato, S., Benozzi, L., Savoldi, S., Licata, C., Mizerska-Wasiak, M., Martina, G., Messuerotti, A., Dal Canton, A., Esposito, C., Migotto, C., Triolo, G., Mariano, F., Pozzi, C., Boero, R., Bellur, S., Mazzucco, G., Giannakakis, C., Honsova, E., Sundelin, B., Di Palma, A.M., Gutiérrez, E., Asunis, A.M., Barratt, J., Tardanico, R., Perkowska-Ptasinska, A., Terroba, J. Arce, Fortunato, M., Pantzaki, A., Ozluk, Y., Steenbergen, E., Soderberg, M., Riispere, Z., Furci, L., Orhan, D., Kipgen, D., Casartelli, D., Ljubanovic, D. Galesic, Gakiopoulou, H., Bertoni, E., Cannata Ortiz, P., Karkoszka, H., Groene, H.J., Stoppacciaro, A., Bajema, I., Bruijn, J., Fulladosa Oliveras, X., Maldyk, J., Ioachim, E., Bavbek, N., Cook, T., Alpers, C., Berthoux, F., Bonsib, S., D’Agati, V., D’Amico, G., Emancipator, S., Emmal, F., Fervenza, F., Florquin, S., Fogo, A., Groene, H., Haas, M., Hill, P., Hogg, R., Hsu, S., Hunley, T., Hladunewich, M., Jennette, C., Joh, K., Julian, B., Kawamura, T., Lai, F., Leung, C., Li, L., Li, P., Liu, Z., Massat, A., Mackinnon, B., Mezzano, S., Schena, F., Tomino, Y., Walker, P., Wang, H., Weening, J., Yoshikawa, N., Zeng, C.-H., Shi, S., Nogi, C., Suzuki, H., Koike, K., Hirano, K., Yokoo, T., Hanai, M., Fukami, K., Takahashi, K., Yuzawa, Y., Niwa, M., Yasuda, Y., Maruyama, S., Ichikawa, D., Suzuki, T., Shirai, S., Fukuda, A., Fujimoto, S., Trimarchi, H., Barbour, Sean J., Coppo, Rosanna, Zhang, Hong, Liu, Zhi-Hong, Suzuki, Yusuke, Matsuzaki, Keiichi, Er, Lee, Reich, Heather N., Barratt, Jonathan, and Cattran, Daniel C.

Published
2022
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4. Evidence from the large VALIGA cohort validates the subclassification of focal segmental glomerulosclerosis in IgA nephropathy.

Author

Bellur, Shubha S., primary, Troyanov, Stéphan, additional, Vorobyeva, Olga, additional, Coppo, Rosanna, additional, Roberts, Ian SD., additional, Cattran, D., additional, Cook, H.T., additional, Feehally, J., additional, Peruzzi, L., additional, Tesar, V., additional, Lunberg, S., additional, Gesualdo, L., additional, Emma, F., additional, Praga, M., additional, Feriozzi, S., additional, Pani, A., additional, Cancarini, G., additional, Durlik, M., additional, Moggia, E., additional, Giannakakis, C., additional, Honsova, E., additional, Sundelin, B.B., additional, Di Palma, A.M., additional, Ferrario, F., additional, Gutierrez, E., additional, Asunis, A.M., additional, Barratt, J., additional, Tardanico, R., additional, and Perkowska-Ptasinska, A., additional

Published
2024
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5. Development and testing of an artificial intelligence tool for predicting end-stage kidney disease in patients with immunoglobulin A nephropathy

Author

Tesar, V., Maixnerova, D., Lundberg, S., Gesualdo, L., Emma, F., Fuiano, L., Beltrame, G., Rollino, C., Coppo, R., Amore, A., Camilla, R., Peruzzi, L., Praga, M., Feriozzi, S., Polci, R., Segoloni, G., Colla, L., Pani, A., Angioi, A., Piras, L., Feehally, J., Cancarini, G., Ravera, S., Durlik, M., Moggia, E., Ballarin, J., Di Giulio, S., Pugliese, F., Serriello, I., Caliskan, Y., Sever, M., Kilicaslan, I., Locatelli, F., Del Vecchio, L., Wetzels, J.F.M., Peters, H., Berg, U., Carvalho, F., da Costa Ferreira, A.C., Maggio, M., Wiecek, A., Ots-Rosenberg, M., Magistroni, R., Topaloglu, R., Bilginer, Y., D’Amico, M., Stangou, M., Giacchino, F., Goumenos, D., Papasotiriou, M., Galesic, K., Toric, L., Geddes, C., Siamopoulos, K., Balafa, O., Galliani, M., Stratta, P., Quaglia, M., Bergia, R., Cravero, R., Salvadori, M., Cirami, L., Fellstrom, B., Smerud, H. Kloster, Ferrario, F., Stellato, T., Egido, J., Martin, C., Floege, J., Eitner, F., Rauen, T., Lupo, A., Bernich, P., Menè, P., Morosetti, M., van Kooten, C., Rabelink, T., Reinders, M.E.J., Grinyo, J.M. Boria, Cusinato, S., Benozzi, L., Savoldi, S., Licata, C., Mizerska-Wasiak, M., Roszkowska-Blaim, M., Martina, G., Messuerotti, A., Canton, A. Dal, Esposito, C., Migotto, C., Triolo, G., Mariano, F., Pozzi, C., Boero, R., Mazzucco, Giannakakis, C., Honsova, E., Sundelin, B., Di Palma, A.M., Gutiérrez, E., Asunis, A.M., Barratt, J., Tardanico, R., Perkowska-Ptasinska, A., Terroba, J. Arce, Fortunato, M., Pantzaki, A., Ozluk, Y., Steenbergen, E., Soderberg, M., Riispere, Z., Furci, L., Orhan, D., Kipgen, D., Casartelli, D., GalesicLjubanovic, D., Gakiopoulou, H., Bertoni, E., Ortiz, P. Cannata, Karkoszka, H., Groene, H.J., Stoppacciaro, A., Bajema, I., Bruijn, J., FulladosaOliveras, X., Maldyk, J., Ioachim, E., Abbrescia, Daniela, Kouri, Nikoleta, Stangou, Maria, Papagianni, Aikaterini, Scolari, Francesco, Delbarba, Elisa, Bonomini, Mario, Piscitani, Luca, Stallone, Giovanni, Infante, Barbara, Godeas, Giulia, Madio, Desiree, Biancone, Luigi, Campagna, Marco, Zaza, Gianluigi, Squarzoni, Isabella, Cangemi, Concetta, Schena, Francesco Paolo, Anelli, Vito Walter, Trotta, Joseph, Di Noia, Tommaso, Manno, Carlo, Tripepi, Giovanni, D’Arrigo, Graziella, Chesnaye, Nicholas C., Russo, Maria Luisa, Zoccali, Carmine, Tesar, Vladimir, and Coppo, Rosanna

Published
2021
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6. Improving treatment decisions using personalized risk assessment from the International IgA Nephropathy Prediction Tool

Author

Russo, M.L., Troyanov, S., Cook, H.T., Roberts, I., Tesar, V., Maixnerova, D., Lundberg, S., Gesualdo, L., Emma, F., Fuiano, L., Beltrame, G., Rollino, C., Amore, A., Camilla, R., Peruzzi, L., Praga, M., Feriozzi, S., Polci, R., Segoloni, G., Colla, L., Pani, A., Piras, D., Angioi, A., Cancarini, G., Ravera, S., Durlik, M., Moggia, E., Ballarin, J., Di Giulio, S., Pugliese, F., Serriello, I., Caliskan, Y., Sever, M., Kilicaslan, I., Locatelli, F., Del Vecchio, L., Wetzels, J.F.M., Peters, H., Berg, U., Carvalho, F., da Costa Ferreira, A.C., Maggio, M., Wiecek, A., Ots-Rosenberg, M., Magistroni, R., Topaloglu, R., Bilginer, Y., D’Amico, M., Stangou, M., Giacchino, F., Goumenos, D., Kalliakmani, P., Gerolymos, M., Galesic, K., Geddes, C., Siamopoulos, K., Balafa, O., Galliani, M., Stratta, P., Quaglia, M., Bergia, R., Cravero, R., Salvadori, M., Cirami, L., Fellstrom, B., Kloster Smerud, H., Ferrario, F., Stellato, T., Egido, J., Martin, C., Floege, J., Eitner, F., Lupo, A., Bernich, P., Menè, P., Morosetti, M., van Kooten, C., Rabelink, T., Reinders, M.E.J., Boria Grinyo, J.M., Cusinato, S., Benozzi, L., Savoldi, S., Licata, C., Mizerska-Wasiak, M., Martina, G., Messuerotti, A., Dal Canton, A., Esposito, C., Migotto, C., Triolo, G., Mariano, F., Pozzi, C., Boero, R., Bellur, S., Mazzucco, G., Giannakakis, C., Honsova, E., Sundelin, B., Di Palma, A.M., Gutiérrez, E., Asunis, A.M., Barratt, J., Tardanico, R., Perkowska-Ptasinska, A., Arce Terroba, J., Fortunato, M., Pantzaki, A., Ozluk, Y., Steenbergen, E., Soderberg, M., Riispere, Z., Furci, L., Orhan, D., Kipgen, D., Casartelli, D., Galesic Ljubanovic, D., Gakiopoulou, H., Bertoni, E., Cannata Ortiz, P., Karkoszka, H., Groene, H.J., Stoppacciaro, A., Bajema, I., Bruijn, J., Fulladosa Oliveras, X., Maldyk, J., Ioachim, E., Bavbek, N., Cook, T., Alpers, C., Berthoux, F., Bonsib, S., D’Agati, V., D’Amico, G., Emancipator, S., Emmal, F., Fervenza, F., Florquin, S., Fogo, A., Groene, H., Haas, M., Hill, P., Hogg, R., Hsu, S., Hunley, T., Hladunewich, M., Jennette, C., Joh, K., Julian, B., Kawamura, T., Lai, F., Leung, C., Li, L., Li, P., Liu, Z., Massat, A., Mackinnon, B., Mezzano, S., Schena, F., Tomino, Y., Walker, P., Wang, H., Weening, J., Yoshikawa N, N., Zeng, C.-H., Shi, S., Nogi, C., Suzuki, H., Koike, K., Hirano, K., Yokoo, T., Hanai, M., Fukami, K., Takahashi, K., Yuzawa, Y., Niwa, M., Yasuda, Y., Maruyama, S., Ichikawa, D., Suzuki, T., Shirai, S., Fukuda, A., Fujimoto, S., Trimarchi, H., Barbour, Sean J., Canney, Mark, Coppo, Rosanna, Zhang, Hong, Liu, Zhi-Hong, Suzuki, Yusuke, Matsuzaki, Keiichi, Katafuchi, Ritsuko, Induruwage, Dilshani, Er, Lee, Reich, Heather N., Feehally, John, Barratt, Jonathan, and Cattran, Daniel C.

Published
2020
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7. Renal Recovery for Patients with ANCA-Associated Vasculitis and Low eGFR in the ADVOCATE Trial of Avacopan

Author

Cortazar, F. B., Niles, J. L., Jayne, D. R. W., Merkel, P. A., Bruchfeld, A., Yue, H., Schall, T. J., Bekker, P., Peh, C. A., Chakera, A., Cooper, B., Kurtkoti, J., Langguth, D., Levidiotis, V., Luxton, G., Mount, P., Mudge, D., Noble, E., Phoon, R., Ranganathan, D., Ritchie, A., Ryan, J., Suranyi, M., Rosenkranz, A., Lhotta, K., Kronbichler, A., Demoulin, N., Bovy, C., Hellemans, R., Hougardy, J., Sprangers, B., Wissing, K., Pagnoux, C., Barbour, S., Brachemi, S., Cournoyer, S., Girard, L., Laurin, L., Liang, P., Philibert, D., Walsh, M., Tesar, V., Becvar, R., Horak, P., Rychlik, I., Szpirt, W., Dieperink, H., Gregersen, J., Ivarsen, P., Krarup, E., Lyngsoe, C., Rigothier, C., Augusto, J., Belot, A., Chauveau, D., Cornec, D., Jourde-Chiche, N., Ficheux, M., Karras, A., Klein, A., Maurier, F., Mesbah, R., Moranne, O., Neel, A., Quemeneur, T., Saadoun, D., Terrier, B., Zaoui, P., Schaier, M., Benck, U., Bergner, R., Busch, M., Floege, J., Grundmann, F., Haller, H., Haubitz, M., Hellmich, B., Henes, J., Hohenstein, B., Hugo, C., Iking-Konert, C., Arndt, F., Kubacki, T., Kotter, I., Lamprecht, P., Lindner, T., Halbritter, J., Mehling, H., Schonermarck, U., Venhoff, N., Vielhauer, V., Witzke, O., Szombati, I., Szucs, G., Garibotto, G., Alberici, F., Brunetta, E., Dagna, L., De Vita, S., Emmi, G., Gabrielli, A., Manenti, L., Pieruzzi, F., Roccatello, D., Salvarani, C., Dobashi, H., Atsumi, T., Fujimoto, S., Hagino, N., Ihata, A., Kaname, S., Kaneko, Y., Katagiri, A., Katayama, M., Kirino, Y., Kitagawa, K., Komatsuda, A., Kono, H., Kurasawa, T., Matsumura, R., Mimura, T., Morinobu, A., Murakawa, Y., Naniwa, T., Nanki, T., Ogawa, N., Oshima, H., Sada, K., Sugiyama, E., Takeuchi, T., Taki, H., Tamura, N., Tsukamoto, T., Yamagata, K., Yamamura, M., van Daele, P., Rutgers, A., Teng, Y., Walker, R., Chua, I., Collins, M., Rabindranath, K., de Zoysa, J., Svensson, M., Grevbo, B., Kalstad, S., Little, M., Clarkson, M., Molloy, E., Pamplona, I. A., Anton, J., Lucia, V. B., Ciggaran, S., Cid, M. C., Encarnacion, M. D., Oliveras, X. F., Soler, M. J., Rusinol, H. M., Praga, M., Porras, L. Q., Segarra, A., Segelmark, M., Soveri, I., Thomaidi, E., Westman, K., Neumann, T., Burnier, M., Daikeler, T., Dudler, J., Hauser, T., Seeger, H., Vogt, B., Jayne, D., Burton, J., Al Jayyousi, R., Amin, T., Andrews, J., Baines, L., Brogan, P., Dasgupta, B., Doulton, T., Flossmann, O., Griffin, S., Harper, J., Harper, L., Kidder, D., Klocke, R., Lanyon, P., Luqmani, R., Mclaren, J., Makanjuola, D., Mccann, L., Nandagudi, A., Selvan, S., O'Riordan, E., Patel, M., Patel, R., Pusey, C., Rajakariar, R., Robson, J., Robson, M., Salama, A., Smyth, L., Sznajd, J., Taylor, J., Merkel, P., Sreih, A., Belilos, E., Bomback, A., Carlin, J., Chen Lin, Y. C., Derebail, V., Dragoi, S., Dua, A., Forbess, L., Geetha, D., Gipson, P., Gohh, R., Greenwood, G. T., Hugenberg, S., Jimenez, R., Kaskas, M., Kermani, T., Kivitz, A., Koening, C., Langford, C., Marder, G., Mohamed, A., Monach, P., Neyra, N., Niemer, G., Niles, J., Obi, R., Owens, C., Parks, D., Podoll, A., Rovin, B., Sam, R., Shergy, W., Silva, A., Specks, U., Spiera, R., Springer, J., Striebich, C., Swarup, A., Thakar, S., Tiliakos, A., Tsai, Y., Waguespack, D., Wasko, M. C., Cortazar, F, Niles, J, Jayne, D, Merkel, P, Bruchfeld, A, Yue, H, Schall, T, Bekker, P, Peh, C, Chakera, A, Cooper, B, Kurtkoti, J, Langguth, D, Levidiotis, V, Luxton, G, Mount, P, Mudge, D, Noble, E, Phoon, R, Ranganathan, D, Ritchie, A, Ryan, J, Suranyi, M, Rosenkranz, A, Lhotta, K, Kronbichler, A, Demoulin, N, Bovy, C, Hellemans, R, Hougardy, J, Sprangers, B, Wissing, K, Pagnoux, C, Barbour, S, Brachemi, S, Cournoyer, S, Girard, L, Laurin, L, Liang, P, Philibert, D, Walsh, M, Tesar, V, Becvar, R, Horak, P, Rychlik, I, Szpirt, W, Dieperink, H, Gregersen, J, Ivarsen, P, Krarup, E, Lyngsoe, C, Rigothier, C, Augusto, J, Belot, A, Chauveau, D, Cornec, D, Jourde-Chiche, N, Ficheux, M, Karras, A, Klein, A, Maurier, F, Mesbah, R, Moranne, O, Neel, A, Quemeneur, T, Saadoun, D, Terrier, B, Zaoui, P, Schaier, M, Benck, U, Bergner, R, Busch, M, Floege, J, Grundmann, F, Haller, H, Haubitz, M, Hellmich, B, Henes, J, Hohenstein, B, Hugo, C, Iking-Konert, C, Arndt, F, Kubacki, T, Kotter, I, Lamprecht, P, Lindner, T, Halbritter, J, Mehling, H, Schonermarck, U, Venhoff, N, Vielhauer, V, Witzke, O, Szombati, I, Szucs, G, Garibotto, G, Alberici, F, Brunetta, E, Dagna, L, De Vita, S, Emmi, G, Gabrielli, A, Manenti, L, Pieruzzi, F, Roccatello, D, Salvarani, C, Dobashi, H, Atsumi, T, Fujimoto, S, Hagino, N, Ihata, A, Kaname, S, Kaneko, Y, Katagiri, A, Katayama, M, Kirino, Y, Kitagawa, K, Komatsuda, A, Kono, H, Kurasawa, T, Matsumura, R, Mimura, T, Morinobu, A, Murakawa, Y, Naniwa, T, Nanki, T, Ogawa, N, Oshima, H, Sada, K, Sugiyama, E, Takeuchi, T, Taki, H, Tamura, N, Tsukamoto, T, Yamagata, K, Yamamura, M, van Daele, P, Rutgers, A, Teng, Y, Walker, R, Chua, I, Collins, M, Rabindranath, K, de Zoysa, J, Svensson, M, Grevbo, B, Kalstad, S, Little, M, Clarkson, M, Molloy, E, Pamplona, I, Anton, J, Lucia, V, Ciggaran, S, Cid, M, Encarnacion, M, Oliveras, X, Soler, M, Rusinol, H, Praga, M, Porras, L, Segarra, A, Segelmark, M, Soveri, I, Thomaidi, E, Westman, K, Neumann, T, Burnier, M, Daikeler, T, Dudler, J, Hauser, T, Seeger, H, Vogt, B, Burton, J, Al Jayyousi, R, Amin, T, Andrews, J, Baines, L, Brogan, P, Dasgupta, B, Doulton, T, Flossmann, O, Griffin, S, Harper, J, Harper, L, Kidder, D, Klocke, R, Lanyon, P, Luqmani, R, Mclaren, J, Makanjuola, D, Mccann, L, Nandagudi, A, Selvan, S, O'Riordan, E, Patel, M, Patel, R, Pusey, C, Rajakariar, R, Robson, J, Robson, M, Salama, A, Smyth, L, Sznajd, J, Taylor, J, Sreih, A, Belilos, E, Bomback, A, Carlin, J, Chen Lin, Y, Derebail, V, Dragoi, S, Dua, A, Forbess, L, Geetha, D, Gipson, P, Gohh, R, Greenwood, G, Hugenberg, S, Jimenez, R, Kaskas, M, Kermani, T, Kivitz, A, Koening, C, Langford, C, Marder, G, Mohamed, A, Monach, P, Neyra, N, Niemer, G, Obi, R, Owens, C, Parks, D, Podoll, A, Rovin, B, Sam, R, Shergy, W, Silva, A, Specks, U, Spiera, R, Springer, J, Striebich, C, Swarup, A, Thakar, S, Tiliakos, A, Tsai, Y, Waguespack, D, and Wasko, M

Subjects
avacopan, Clinical Research, renal recovery, Nephrology, low eGFR, complement 5a receptor, complement, ANCA-associated vasculiti
Abstract

INTRODUCTION: In the 330-patient ADVOCATE trial of avacopan for the treatment of antineutrophil cytoplasmic autoantibody (ANCA)-associated vasculitis, in which 81% of patients had renal involvement, estimated glomerular filtration rate (eGFR) increased on average 7.3 ml/min per 1.73 m(2) in the avacopan group and 4.1 ml/min per 1.73 m(2) in the prednisone group (P = 0.029) at week 52. This new analysis examines the results in the patient subgroup with severe renal insufficiency at enrollment into the trial, i.e., eGFR ≤20 ml/min per 1.73 m(2). METHODS: eGFR was determined at baseline and over the course of the trial. Changes in eGFR were compared between the 2 treatment groups. RESULTS: In ADVOCATE, 27 of 166 patients (16%) in the avacopan group and 23 of 164 patients (14%) in the prednisone group had a baseline eGFR ≤20 ml/min per 1.73 m(2). At week 52, eGFR increased on average 16.1 and 7.7 ml/min per 1.73 m(2) in the avacopan and prednisone groups, respectively (P = 0.003). The last eGFR value measured during the 52-week treatment period was ≥2-fold higher than baseline in 41% of patients in the avacopan group compared to 13% in the prednisone group (P = 0.030). More patients in the avacopan group versus prednisone group had increases in eGFR above 20, 30, and 45 ml/min per 1.73 m(2), respectively. Serious adverse events occurred in 13 of 27 patients (48%) in the avacopan group and 16 of 23 patients (70%) in the prednisone group. CONCLUSION: Among patients with baseline eGFR ≤20 ml/min per 1.73 m(2) in the ADVOCATE trial, eGFR improved more in the avacopan group than in the prednisone group.

Published
2023

8. Renal Recovery for Patients with ANCA-Associated Vasculitis and Low eGFR in the ADVOCATE Trial of Avacopan

Author

Cortazar, F, Niles, J, Jayne, D, Merkel, P, Bruchfeld, A, Yue, H, Schall, T, Bekker, P, Peh, C, Chakera, A, Cooper, B, Kurtkoti, J, Langguth, D, Levidiotis, V, Luxton, G, Mount, P, Mudge, D, Noble, E, Phoon, R, Ranganathan, D, Ritchie, A, Ryan, J, Suranyi, M, Rosenkranz, A, Lhotta, K, Kronbichler, A, Demoulin, N, Bovy, C, Hellemans, R, Hougardy, J, Sprangers, B, Wissing, K, Pagnoux, C, Barbour, S, Brachemi, S, Cournoyer, S, Girard, L, Laurin, L, Liang, P, Philibert, D, Walsh, M, Tesar, V, Becvar, R, Horak, P, Rychlik, I, Szpirt, W, Dieperink, H, Gregersen, J, Ivarsen, P, Krarup, E, Lyngsoe, C, Rigothier, C, Augusto, J, Belot, A, Chauveau, D, Cornec, D, Jourde-Chiche, N, Ficheux, M, Karras, A, Klein, A, Maurier, F, Mesbah, R, Moranne, O, Neel, A, Quemeneur, T, Saadoun, D, Terrier, B, Zaoui, P, Schaier, M, Benck, U, Bergner, R, Busch, M, Floege, J, Grundmann, F, Haller, H, Haubitz, M, Hellmich, B, Henes, J, Hohenstein, B, Hugo, C, Iking-Konert, C, Arndt, F, Kubacki, T, Kotter, I, Lamprecht, P, Lindner, T, Halbritter, J, Mehling, H, Schonermarck, U, Venhoff, N, Vielhauer, V, Witzke, O, Szombati, I, Szucs, G, Garibotto, G, Alberici, F, Brunetta, E, Dagna, L, De Vita, S, Emmi, G, Gabrielli, A, Manenti, L, Pieruzzi, F, Roccatello, D, Salvarani, C, Dobashi, H, Atsumi, T, Fujimoto, S, Hagino, N, Ihata, A, Kaname, S, Kaneko, Y, Katagiri, A, Katayama, M, Kirino, Y, Kitagawa, K, Komatsuda, A, Kono, H, Kurasawa, T, Matsumura, R, Mimura, T, Morinobu, A, Murakawa, Y, Naniwa, T, Nanki, T, Ogawa, N, Oshima, H, Sada, K, Sugiyama, E, Takeuchi, T, Taki, H, Tamura, N, Tsukamoto, T, Yamagata, K, Yamamura, M, van Daele, P, Rutgers, A, Teng, Y, Walker, R, Chua, I, Collins, M, Rabindranath, K, de Zoysa, J, Svensson, M, Grevbo, B, Kalstad, S, Little, M, Clarkson, M, Molloy, E, Pamplona, I, Anton, J, Lucia, V, Ciggaran, S, Cid, M, Encarnacion, M, Oliveras, X, Soler, M, Rusinol, H, Praga, M, Porras, L, Segarra, A, Segelmark, M, Soveri, I, Thomaidi, E, Westman, K, Neumann, T, Burnier, M, Daikeler, T, Dudler, J, Hauser, T, Seeger, H, Vogt, B, Burton, J, Al Jayyousi, R, Amin, T, Andrews, J, Baines, L, Brogan, P, Dasgupta, B, Doulton, T, Flossmann, O, Griffin, S, Harper, J, Harper, L, Kidder, D, Klocke, R, Lanyon, P, Luqmani, R, Mclaren, J, Makanjuola, D, Mccann, L, Nandagudi, A, Selvan, S, O'Riordan, E, Patel, M, Patel, R, Pusey, C, Rajakariar, R, Robson, J, Robson, M, Salama, A, Smyth, L, Sznajd, J, Taylor, J, Sreih, A, Belilos, E, Bomback, A, Carlin, J, Chen Lin, Y, Derebail, V, Dragoi, S, Dua, A, Forbess, L, Geetha, D, Gipson, P, Gohh, R, Greenwood, G, Hugenberg, S, Jimenez, R, Kaskas, M, Kermani, T, Kivitz, A, Koening, C, Langford, C, Marder, G, Mohamed, A, Monach, P, Neyra, N, Niemer, G, Obi, R, Owens, C, Parks, D, Podoll, A, Rovin, B, Sam, R, Shergy, W, Silva, A, Specks, U, Spiera, R, Springer, J, Striebich, C, Swarup, A, Thakar, S, Tiliakos, A, Tsai, Y, Waguespack, D, Wasko, M, Cortazar F. B., Niles J. L., Jayne D. R. W., Merkel P. A., Bruchfeld A., Yue H., Schall T. J., Bekker P., Peh C. A., Chakera A., Cooper B., Kurtkoti J., Langguth D., Levidiotis V., Luxton G., Mount P., Mudge D., Noble E., Phoon R., Ranganathan D., Ritchie A., Ryan J., Suranyi M., Rosenkranz A., Lhotta K., Kronbichler A., Demoulin N., Bovy C., Hellemans R., Hougardy J., Sprangers B., Wissing K., Pagnoux C., Barbour S., Brachemi S., Cournoyer S., Girard L., Laurin L., Liang P., Philibert D., Walsh M., Tesar V., Becvar R., Horak P., Rychlik I., Szpirt W., Dieperink H., Gregersen J., Ivarsen P., Krarup E., Lyngsoe C., Rigothier C., Augusto J., Belot A., Chauveau D., Cornec D., Jourde-Chiche N., Ficheux M., Karras A., Klein A., Maurier F., Mesbah R., Moranne O., Neel A., Quemeneur T., Saadoun D., Terrier B., Zaoui P., Schaier M., Benck U., Bergner R., Busch M., Floege J., Grundmann F., Haller H., Haubitz M., Hellmich B., Henes J., Hohenstein B., Hugo C., Iking-Konert C., Arndt F., Kubacki T., Kotter I., Lamprecht P., Lindner T., Halbritter J., Mehling H., Schonermarck U., Venhoff N., Vielhauer V., Witzke O., Szombati I., Szucs G., Garibotto G., Alberici F., Brunetta E., Dagna L., De Vita S., Emmi G., Gabrielli A., Manenti L., Pieruzzi F., Roccatello D., Salvarani C., Dobashi H., Atsumi T., Fujimoto S., Hagino N., Ihata A., Kaname S., Kaneko Y., Katagiri A., Katayama M., Kirino Y., Kitagawa K., Komatsuda A., Kono H., Kurasawa T., Matsumura R., Mimura T., Morinobu A., Murakawa Y., Naniwa T., Nanki T., Ogawa N., Oshima H., Sada K., Sugiyama E., Takeuchi T., Taki H., Tamura N., Tsukamoto T., Yamagata K., Yamamura M., van Daele P., Rutgers A., Teng Y., Walker R., Chua I., Collins M., Rabindranath K., de Zoysa J., Svensson M., Grevbo B., Kalstad S., Little M., Clarkson M., Molloy E., Pamplona I. A., Anton J., Lucia V. B., Ciggaran S., Cid M. C., Encarnacion M. D., Oliveras X. F., Soler M. J., Rusinol H. M., Praga M., Porras L. Q., Segarra A., Segelmark M., Soveri I., Thomaidi E., Westman K., Neumann T., Burnier M., Daikeler T., Dudler J., Hauser T., Seeger H., Vogt B., Jayne D., Burton J., Al Jayyousi R., Amin T., Andrews J., Baines L., Brogan P., Dasgupta B., Doulton T., Flossmann O., Griffin S., Harper J., Harper L., Kidder D., Klocke R., Lanyon P., Luqmani R., McLaren J., Makanjuola D., McCann L., Nandagudi A., Selvan S., O'Riordan E., Patel M., Patel R., Pusey C., Rajakariar R., Robson J., Robson M., Salama A., Smyth L., Sznajd J., Taylor J., Merkel P., Sreih A., Belilos E., Bomback A., Carlin J., Chen Lin Y. C., Derebail V., Dragoi S., Dua A., Forbess L., Geetha D., Gipson P., Gohh R., Greenwood G. T., Hugenberg S., Jimenez R., Kaskas M., Kermani T., Kivitz A., Koening C., Langford C., Marder G., Mohamed A., Monach P., Neyra N., Niemer G., Niles J., Obi R., Owens C., Parks D., Podoll A., Rovin B., Sam R., Shergy W., Silva A., Specks U., Spiera R., Springer J., Striebich C., Swarup A., Thakar S., Tiliakos A., Tsai Y., Waguespack D., Wasko M. C., Cortazar, F, Niles, J, Jayne, D, Merkel, P, Bruchfeld, A, Yue, H, Schall, T, Bekker, P, Peh, C, Chakera, A, Cooper, B, Kurtkoti, J, Langguth, D, Levidiotis, V, Luxton, G, Mount, P, Mudge, D, Noble, E, Phoon, R, Ranganathan, D, Ritchie, A, Ryan, J, Suranyi, M, Rosenkranz, A, Lhotta, K, Kronbichler, A, Demoulin, N, Bovy, C, Hellemans, R, Hougardy, J, Sprangers, B, Wissing, K, Pagnoux, C, Barbour, S, Brachemi, S, Cournoyer, S, Girard, L, Laurin, L, Liang, P, Philibert, D, Walsh, M, Tesar, V, Becvar, R, Horak, P, Rychlik, I, Szpirt, W, Dieperink, H, Gregersen, J, Ivarsen, P, Krarup, E, Lyngsoe, C, Rigothier, C, Augusto, J, Belot, A, Chauveau, D, Cornec, D, Jourde-Chiche, N, Ficheux, M, Karras, A, Klein, A, Maurier, F, Mesbah, R, Moranne, O, Neel, A, Quemeneur, T, Saadoun, D, Terrier, B, Zaoui, P, Schaier, M, Benck, U, Bergner, R, Busch, M, Floege, J, Grundmann, F, Haller, H, Haubitz, M, Hellmich, B, Henes, J, Hohenstein, B, Hugo, C, Iking-Konert, C, Arndt, F, Kubacki, T, Kotter, I, Lamprecht, P, Lindner, T, Halbritter, J, Mehling, H, Schonermarck, U, Venhoff, N, Vielhauer, V, Witzke, O, Szombati, I, Szucs, G, Garibotto, G, Alberici, F, Brunetta, E, Dagna, L, De Vita, S, Emmi, G, Gabrielli, A, Manenti, L, Pieruzzi, F, Roccatello, D, Salvarani, C, Dobashi, H, Atsumi, T, Fujimoto, S, Hagino, N, Ihata, A, Kaname, S, Kaneko, Y, Katagiri, A, Katayama, M, Kirino, Y, Kitagawa, K, Komatsuda, A, Kono, H, Kurasawa, T, Matsumura, R, Mimura, T, Morinobu, A, Murakawa, Y, Naniwa, T, Nanki, T, Ogawa, N, Oshima, H, Sada, K, Sugiyama, E, Takeuchi, T, Taki, H, Tamura, N, Tsukamoto, T, Yamagata, K, Yamamura, M, van Daele, P, Rutgers, A, Teng, Y, Walker, R, Chua, I, Collins, M, Rabindranath, K, de Zoysa, J, Svensson, M, Grevbo, B, Kalstad, S, Little, M, Clarkson, M, Molloy, E, Pamplona, I, Anton, J, Lucia, V, Ciggaran, S, Cid, M, Encarnacion, M, Oliveras, X, Soler, M, Rusinol, H, Praga, M, Porras, L, Segarra, A, Segelmark, M, Soveri, I, Thomaidi, E, Westman, K, Neumann, T, Burnier, M, Daikeler, T, Dudler, J, Hauser, T, Seeger, H, Vogt, B, Burton, J, Al Jayyousi, R, Amin, T, Andrews, J, Baines, L, Brogan, P, Dasgupta, B, Doulton, T, Flossmann, O, Griffin, S, Harper, J, Harper, L, Kidder, D, Klocke, R, Lanyon, P, Luqmani, R, Mclaren, J, Makanjuola, D, Mccann, L, Nandagudi, A, Selvan, S, O'Riordan, E, Patel, M, Patel, R, Pusey, C, Rajakariar, R, Robson, J, Robson, M, Salama, A, Smyth, L, Sznajd, J, Taylor, J, Sreih, A, Belilos, E, Bomback, A, Carlin, J, Chen Lin, Y, Derebail, V, Dragoi, S, Dua, A, Forbess, L, Geetha, D, Gipson, P, Gohh, R, Greenwood, G, Hugenberg, S, Jimenez, R, Kaskas, M, Kermani, T, Kivitz, A, Koening, C, Langford, C, Marder, G, Mohamed, A, Monach, P, Neyra, N, Niemer, G, Obi, R, Owens, C, Parks, D, Podoll, A, Rovin, B, Sam, R, Shergy, W, Silva, A, Specks, U, Spiera, R, Springer, J, Striebich, C, Swarup, A, Thakar, S, Tiliakos, A, Tsai, Y, Waguespack, D, Wasko, M, Cortazar F. B., Niles J. L., Jayne D. R. W., Merkel P. A., Bruchfeld A., Yue H., Schall T. J., Bekker P., Peh C. A., Chakera A., Cooper B., Kurtkoti J., Langguth D., Levidiotis V., Luxton G., Mount P., Mudge D., Noble E., Phoon R., Ranganathan D., Ritchie A., Ryan J., Suranyi M., Rosenkranz A., Lhotta K., Kronbichler A., Demoulin N., Bovy C., Hellemans R., Hougardy J., Sprangers B., Wissing K., Pagnoux C., Barbour S., Brachemi S., Cournoyer S., Girard L., Laurin L., Liang P., Philibert D., Walsh M., Tesar V., Becvar R., Horak P., Rychlik I., Szpirt W., Dieperink H., Gregersen J., Ivarsen P., Krarup E., Lyngsoe C., Rigothier C., Augusto J., Belot A., Chauveau D., Cornec D., Jourde-Chiche N., Ficheux M., Karras A., Klein A., Maurier F., Mesbah R., Moranne O., Neel A., Quemeneur T., Saadoun D., Terrier B., Zaoui P., Schaier M., Benck U., Bergner R., Busch M., Floege J., Grundmann F., Haller H., Haubitz M., Hellmich B., Henes J., Hohenstein B., Hugo C., Iking-Konert C., Arndt F., Kubacki T., Kotter I., Lamprecht P., Lindner T., Halbritter J., Mehling H., Schonermarck U., Venhoff N., Vielhauer V., Witzke O., Szombati I., Szucs G., Garibotto G., Alberici F., Brunetta E., Dagna L., De Vita S., Emmi G., Gabrielli A., Manenti L., Pieruzzi F., Roccatello D., Salvarani C., Dobashi H., Atsumi T., Fujimoto S., Hagino N., Ihata A., Kaname S., Kaneko Y., Katagiri A., Katayama M., Kirino Y., Kitagawa K., Komatsuda A., Kono H., Kurasawa T., Matsumura R., Mimura T., Morinobu A., Murakawa Y., Naniwa T., Nanki T., Ogawa N., Oshima H., Sada K., Sugiyama E., Takeuchi T., Taki H., Tamura N., Tsukamoto T., Yamagata K., Yamamura M., van Daele P., Rutgers A., Teng Y., Walker R., Chua I., Collins M., Rabindranath K., de Zoysa J., Svensson M., Grevbo B., Kalstad S., Little M., Clarkson M., Molloy E., Pamplona I. A., Anton J., Lucia V. B., Ciggaran S., Cid M. C., Encarnacion M. D., Oliveras X. F., Soler M. J., Rusinol H. M., Praga M., Porras L. Q., Segarra A., Segelmark M., Soveri I., Thomaidi E., Westman K., Neumann T., Burnier M., Daikeler T., Dudler J., Hauser T., Seeger H., Vogt B., Jayne D., Burton J., Al Jayyousi R., Amin T., Andrews J., Baines L., Brogan P., Dasgupta B., Doulton T., Flossmann O., Griffin S., Harper J., Harper L., Kidder D., Klocke R., Lanyon P., Luqmani R., McLaren J., Makanjuola D., McCann L., Nandagudi A., Selvan S., O'Riordan E., Patel M., Patel R., Pusey C., Rajakariar R., Robson J., Robson M., Salama A., Smyth L., Sznajd J., Taylor J., Merkel P., Sreih A., Belilos E., Bomback A., Carlin J., Chen Lin Y. C., Derebail V., Dragoi S., Dua A., Forbess L., Geetha D., Gipson P., Gohh R., Greenwood G. T., Hugenberg S., Jimenez R., Kaskas M., Kermani T., Kivitz A., Koening C., Langford C., Marder G., Mohamed A., Monach P., Neyra N., Niemer G., Niles J., Obi R., Owens C., Parks D., Podoll A., Rovin B., Sam R., Shergy W., Silva A., Specks U., Spiera R., Springer J., Striebich C., Swarup A., Thakar S., Tiliakos A., Tsai Y., Waguespack D., and Wasko M. C.

Abstract

Introduction: In the 330-patient ADVOCATE trial of avacopan for the treatment of antineutrophil cytoplasmic autoantibody (ANCA)-associated vasculitis, in which 81% of patients had renal involvement, estimated glomerular filtration rate (eGFR) increased on average 7.3 ml/min per 1.73 m2 in the avacopan group and 4.1 ml/min per 1.73 m2 in the prednisone group (P = 0.029) at week 52. This new analysis examines the results in the patient subgroup with severe renal insufficiency at enrollment into the trial, i.e., eGFR ≤20 ml/min per 1.73 m2. Methods: eGFR was determined at baseline and over the course of the trial. Changes in eGFR were compared between the 2 treatment groups. Results: In ADVOCATE, 27 of 166 patients (16%) in the avacopan group and 23 of 164 patients (14%) in the prednisone group had a baseline eGFR ≤20 ml/min per 1.73 m2. At week 52, eGFR increased on average 16.1 and 7.7 ml/min per 1.73 m2 in the avacopan and prednisone groups, respectively (P = 0.003). The last eGFR value measured during the 52-week treatment period was ≥2-fold higher than baseline in 41% of patients in the avacopan group compared to 13% in the prednisone group (P = 0.030). More patients in the avacopan group versus prednisone group had increases in eGFR above 20, 30, and 45 ml/min per 1.73 m2, respectively. Serious adverse events occurred in 13 of 27 patients (48%) in the avacopan group and 16 of 23 patients (70%) in the prednisone group. Conclusion: Among patients with baseline eGFR ≤20 ml/min per 1.73 m2 in the ADVOCATE trial, eGFR improved more in the avacopan group than in the prednisone group.

Published
2023

9. Determinants of renal and patient outcomes in a Spanish cohort of patients with ANCA-associated vasculitis and renal involvement

Author

Marco, H., Draibe, J., Villacorta, J., Quintana, L. F., Martin, N., Garcia-Osuna, R., Cabre, C., Martín-Gómez, MA., Balius, A., Saurina, A., Picazo, M., Gich-Saladich, I., Navarro-Díaz, M., Praga, M., Cavero, T., Ballarin, J., Díaz-Encarnación, M M, and for the Spanish Group for the Study of Glomerular Disease (GLOSEN)

Published
2018
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10. WCN23-0403 AN OPEN-LABEL, NON-RANDOMIZED EXTENSION STUDY TO EVALUATE LONG-TERM EFFICACY, SAFETY, AND TOLERABILITY OF LNP023 IN SUBJECTS WITH C3 GLOMERULOPATHY: INTERIM ANALYSIS OF PHASE 2 STUDY

Author

Nester, C.M., primary, Eisenberger, U., additional, Karras, A., additional, le Quintrec-Donnette, M., additional, Lightstone, L., additional, Praga, M., additional, Remuzzi, G., additional, Jose Soler, M., additional, Liu, J., additional, Meier, M., additional, Tawfik, R., additional, Junge, G., additional, Biondani, A., additional, J Trapani, A., additional, Webb, N., additional, and K Wong, E., additional

Published
2023
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11. Glomerular diseases in pregnancy: pragmatic recommendations for clinical management.

Author

Fakhouri, F., Schwotzer, N., Cabiddu, G., Barratt, J., Legardeur, H., Garovic, V., Orozco-Guillen, A., Wetzels, J., Daugas, E., Moroni, G., Noris, M., Audard, V., Praga, M., Llurba, E., Wuerzner, G., Attini, R., Desseauve, D., Zakharova, E., Luders, C., Wiles, K., Leone, F., Jesudason, S., Costedoat-Chalumeau, N., Kattah, A., Soto-Abraham, V., Karras, A., Prakash, J., Lightstone, L., Ronco, P., Ponticelli, C., Appel, G., Remuzzi, G., Tsatsaris, V., Piccoli, G.B., Fakhouri, F., Schwotzer, N., Cabiddu, G., Barratt, J., Legardeur, H., Garovic, V., Orozco-Guillen, A., Wetzels, J., Daugas, E., Moroni, G., Noris, M., Audard, V., Praga, M., Llurba, E., Wuerzner, G., Attini, R., Desseauve, D., Zakharova, E., Luders, C., Wiles, K., Leone, F., Jesudason, S., Costedoat-Chalumeau, N., Kattah, A., Soto-Abraham, V., Karras, A., Prakash, J., Lightstone, L., Ronco, P., Ponticelli, C., Appel, G., Remuzzi, G., Tsatsaris, V., and Piccoli, G.B.

Abstract

01 februari 2023, Item does not contain fulltext, Our understanding of the various aspects of pregnancy in women with kidney diseases has significantly improved in the last decades. Nevertheless, little is known about specific kidney diseases. Glomerular diseases are not only a frequent cause of chronic kidney disease in young women, but combine many challenges in pregnancy: immunologic diseases, hypertension, proteinuria, and kidney tissue damage. An international working group undertook the review of available current literature and elicited expert opinions on glomerular diseases in pregnancy with the aim to provide pragmatic information for nephrologists according to the present state-of-the-art knowledge. This work also highlights areas of clinical uncertainty and emphasizes the need for further collaborative studies to improve maternal and fetal health.

Published
2023

12. Mayo Clinic consensus report on membranous nephropathy: proposal for a novel classification.

Author

Sethi, S., Beck LH, J.r., Glassock, R.J., Haas, Mark, Vriese, A.S. de, Caza, T.N., Hoxha, E., Lambeau, G., Tomas, N.M., Madden, B., Debiec, H., D'Agati, V.D., Alexander, M.P., Amer, H., Appel, G.B., Barbour, S.J., Caravaca-Fontan, F., Cattran, D.C., Casal Moura, M., D'Avila, D.O., Eick, R.G., Garovic, V.D., Greene, E.L., Herrera Hernandez, L.P., Jennette, J.C., Lieske, J.C., Markowitz, G.S., Nath, K.A., Nasr, S.H., Nast, C.C., Pani, A., Praga, M., Remuzzi, G., Rennke, H.G., Ruggenenti, P., Roccatello, D., Soler, M.J., Specks, U., Stahl, R.A.K., Singh, R.D., Theis, J.D., Velosa, J.A., Wetzels, J.F.M., Winearls, C.G., Yandian, F., Zand, L., Ronco, P., Fervenza, F.C., Sethi, S., Beck LH, J.r., Glassock, R.J., Haas, Mark, Vriese, A.S. de, Caza, T.N., Hoxha, E., Lambeau, G., Tomas, N.M., Madden, B., Debiec, H., D'Agati, V.D., Alexander, M.P., Amer, H., Appel, G.B., Barbour, S.J., Caravaca-Fontan, F., Cattran, D.C., Casal Moura, M., D'Avila, D.O., Eick, R.G., Garovic, V.D., Greene, E.L., Herrera Hernandez, L.P., Jennette, J.C., Lieske, J.C., Markowitz, G.S., Nath, K.A., Nasr, S.H., Nast, C.C., Pani, A., Praga, M., Remuzzi, G., Rennke, H.G., Ruggenenti, P., Roccatello, D., Soler, M.J., Specks, U., Stahl, R.A.K., Singh, R.D., Theis, J.D., Velosa, J.A., Wetzels, J.F.M., Winearls, C.G., Yandian, F., Zand, L., Ronco, P., and Fervenza, F.C.

Abstract

Contains fulltext : 300117.pdf (Publisher’s version ) (Open Access), Membranous nephropathy (MN) is a pattern of injury caused by autoantibodies binding to specific target antigens, with accumulation of immune complexes along the subepithelial region of glomerular basement membranes. The past 20 years have brought revolutionary advances in the understanding of MN, particularly via the discovery of novel target antigens and their respective autoantibodies. These discoveries have challenged the traditional classification of MN into primary and secondary forms. At least 14 target antigens have been identified, accounting for 80%-90% of cases of MN. Many of the forms of MN associated with these novel MN target antigens have distinctive clinical and pathologic phenotypes. The Mayo Clinic consensus report on MN proposes a 2-step classification of MN. The first step, when possible, is identification of the target antigen, based on a multistep algorithm and using a combination of serology, staining of the kidney biopsy tissue by immunofluorescence or immunohistochemistry, and/or mass spectrometry methodology. The second step is the search for a potential underlying disease or associated condition, which is particularly relevant when knowledge of the target antigen is available to direct it. The meeting acknowledges that the resources and equipment required to perform the proposed testing may not be generally available. However, the meeting consensus was that the time has come to adopt an antigen-based classification of MN because this approach will allow for accurate and specific MN diagnosis, with significant implications for patient management and targeted treatment., 01 december 2023

Published
2023

15. Application of the International IgA Nephropathy Prediction Tool one or two years post-biopsy

Author

Barbour, Sean J., primary, Coppo, Rosanna, additional, Zhang, Hong, additional, Liu, Zhi-Hong, additional, Suzuki, Yusuke, additional, Matsuzaki, Keiichi, additional, Er, Lee, additional, Reich, Heather N., additional, Barratt, Jonathan, additional, Cattran, Daniel C., additional, Russo, M.L., additional, Troyanov, S., additional, Cook, H.T., additional, Roberts, I., additional, Tesar, V., additional, Maixnerova, D., additional, Lundberg, S., additional, Gesualdo, L., additional, Emma, F., additional, Fuiano, L., additional, Beltrame, G., additional, Rollino, C., additional, Amore, A., additional, Camilla, R., additional, Peruzzi, L., additional, Praga, M., additional, Feriozzi, S., additional, Polci, R., additional, Segoloni, G., additional, Colla, L., additional, Pani, A., additional, Piras, D., additional, Angioi, A., additional, Cancarini, G., additional, Ravera, S., additional, Durlik, M., additional, Moggia, E., additional, Ballarin, J., additional, Di Giulio, S., additional, Pugliese, F., additional, Serriello, I., additional, Caliskan, Y., additional, Sever, M., additional, Kilicaslan, I., additional, Locatelli, F., additional, Del Vecchio, L., additional, Wetzels, J.F.M., additional, Peters, H., additional, Berg, U., additional, Carvalho, F., additional, da Costa Ferreira, A.C., additional, Maggio, M., additional, Wiecek, A., additional, Ots-Rosenberg, M., additional, Magistroni, R., additional, Topaloglu, R., additional, Bilginer, Y., additional, D’Amico, M., additional, Stangou, M., additional, Giacchino, F., additional, Goumenos, D., additional, Papachristou, E., additional, Galesic, K., additional, Geddes, C., additional, Siamopoulos, K., additional, Balafa, O., additional, Galliani, M., additional, Stratta, P., additional, Quaglia, M., additional, Bergia, R., additional, Cravero, R., additional, Salvadori, M., additional, Cirami, L., additional, Fellstrom, B., additional, Smerud, H. Kloster, additional, Ferrario, F., additional, Stellato, T., additional, Egido, J., additional, Martin, C., additional, Floege, J., additional, Eitner, F., additional, Lupo, A., additional, Bernich, P., additional, Menè, P., additional, Morosetti, M., additional, van Kooten, C., additional, Rabelink, T., additional, Reinders, M.E.J., additional, Boria Grinyo, J.M., additional, Cusinato, S., additional, Benozzi, L., additional, Savoldi, S., additional, Licata, C., additional, Mizerska-Wasiak, M., additional, Martina, G., additional, Messuerotti, A., additional, Dal Canton, A., additional, Esposito, C., additional, Migotto, C., additional, Triolo, G., additional, Mariano, F., additional, Pozzi, C., additional, Boero, R., additional, Bellur, S., additional, Mazzucco, G., additional, Giannakakis, C., additional, Honsova, E., additional, Sundelin, B., additional, Di Palma, A.M., additional, Gutiérrez, E., additional, Asunis, A.M., additional, Barratt, J., additional, Tardanico, R., additional, Perkowska-Ptasinska, A., additional, Terroba, J. Arce, additional, Fortunato, M., additional, Pantzaki, A., additional, Ozluk, Y., additional, Steenbergen, E., additional, Soderberg, M., additional, Riispere, Z., additional, Furci, L., additional, Orhan, D., additional, Kipgen, D., additional, Casartelli, D., additional, Ljubanovic, D. Galesic, additional, Gakiopoulou, H., additional, Bertoni, E., additional, Cannata Ortiz, P., additional, Karkoszka, H., additional, Groene, H.J., additional, Stoppacciaro, A., additional, Bajema, I., additional, Bruijn, J., additional, Fulladosa Oliveras, X., additional, Maldyk, J., additional, Ioachim, E., additional, Bavbek, N., additional, Cook, T., additional, Alpers, C., additional, Berthoux, F., additional, Bonsib, S., additional, D’Agati, V., additional, D’Amico, G., additional, Emancipator, S., additional, Emmal, F., additional, Fervenza, F., additional, Florquin, S., additional, Fogo, A., additional, Groene, H., additional, Haas, M., additional, Hill, P., additional, Hogg, R., additional, Hsu, S., additional, Hunley, T., additional, Hladunewich, M., additional, Jennette, C., additional, Joh, K., additional, Julian, B., additional, Kawamura, T., additional, Lai, F., additional, Leung, C., additional, Li, L., additional, Li, P., additional, Liu, Z., additional, Massat, A., additional, Mackinnon, B., additional, Mezzano, S., additional, Schena, F., additional, Tomino, Y., additional, Walker, P., additional, Wang, H., additional, Weening, J., additional, Yoshikawa, N., additional, Zeng, C.-H., additional, Shi, S., additional, Nogi, C., additional, Suzuki, H., additional, Koike, K., additional, Hirano, K., additional, Yokoo, T., additional, Hanai, M., additional, Fukami, K., additional, Takahashi, K., additional, Yuzawa, Y., additional, Niwa, M., additional, Yasuda, Y., additional, Maruyama, S., additional, Ichikawa, D., additional, Suzuki, T., additional, Shirai, S., additional, Fukuda, A., additional, Fujimoto, S., additional, and Trimarchi, H., additional

Published
2022
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16. Acute Treatment Effects on GFR in Randomized Clinical Trials of Kidney Disease Progression

Author

Neuen, B.L., Tighiouart, H., Heerspink, H.J., Vonesh, E.F., Chaudhari, J., Miao, S., Chan, T.M., Fervenza, F.C., Floege, J., Goicoechea, M., Herrington, W.G., Imai, E., Jafar, T.H., Lewis, J.B., Li, P.K., Locatelli, F., Maes, B.D., Perrone, R.D., Praga, M., Perna, A., Schena, F.P., Wanner, C., Wetzels, J.F.M., Woodward, M., Xie, D., Greene, T., Inker, L.A., Neuen, B.L., Tighiouart, H., Heerspink, H.J., Vonesh, E.F., Chaudhari, J., Miao, S., Chan, T.M., Fervenza, F.C., Floege, J., Goicoechea, M., Herrington, W.G., Imai, E., Jafar, T.H., Lewis, J.B., Li, P.K., Locatelli, F., Maes, B.D., Perrone, R.D., Praga, M., Perna, A., Schena, F.P., Wanner, C., Wetzels, J.F.M., Woodward, M., Xie, D., Greene, T., and Inker, L.A.

Abstract

Item does not contain fulltext, BACKGROUND: Acute changes in GFR can occur after initiation of interventions targeting progression of CKD. These acute changes complicate the interpretation of long-term treatment effects. METHODS: To assess the magnitude and consistency of acute effects in randomized clinical trials and explore factors that might affect them, we performed a meta-analysis of 53 randomized clinical trials for CKD progression, enrolling 56,413 participants with at least one estimated GFR measurement by 6 months after randomization. We defined acute treatment effects as the mean difference in GFR slope from baseline to 3 months between randomized groups. We performed univariable and multivariable metaregression to assess the effect of intervention type, disease state, baseline GFR, and albuminuria on the magnitude of acute effects. RESULTS: The mean acute effect across all studies was -0.21 ml/min per 1.73 m(2) (95% confidence interval, -0.63 to 0.22) over 3 months, with substantial heterogeneity across interventions (95% coverage interval across studies, -2.50 to +2.08 ml/min per 1.73 m(2)). We observed negative average acute effects in renin angiotensin system blockade, BP lowering, and sodium-glucose cotransporter 2 inhibitor trials, and positive acute effects in trials of immunosuppressive agents. Larger negative acute effects were observed in trials with a higher mean baseline GFR. CONCLUSION: The magnitude and consistency of acute GFR effects vary across different interventions, and are larger at higher baseline GFR. Understanding the nature and magnitude of acute effects can help inform the optimal design of randomized clinical trials evaluating disease progression in CKD.

Published
2022

17. Diagnosis and Treatment of Patients With Focal Segmental Glomerulosclerosis/Steroid-Resistant Nephrotic Syndrome: A Delphi Survey

Author

Floege, J., Gibson, K.L., Praga, M., Radhakrishnan, J., Reich, H.N., Schreuder, M.F., Wetzels, J.F.M., Tesař, V., Vivarelli, M., Biechele, S., Tonelli, M., Floege, J., Gibson, K.L., Praga, M., Radhakrishnan, J., Reich, H.N., Schreuder, M.F., Wetzels, J.F.M., Tesař, V., Vivarelli, M., Biechele, S., and Tonelli, M.

Abstract

Contains fulltext : 283145.pdf (Publisher’s version ) (Open Access)

Published
2022

18. Is there long-term value of pathology scoring in immunoglobulin A nephropathy? A validation study of the Oxford Classification for IgA Nephropathy (VALIGA) update

Author

Coppo, R., D'Arrigo, G., Tripepi, G., Russo, M. L., Roberts, I. S. D., Bellur, S., Cattran, D., Cook, T. H., Feehally, J., Tesar, V., Maixnerova, D., Peruzzi, L., Amore, A., Lundberg, S., Di Palma, A. M., Gesualdo, L., Emma, F., Rollino, C., Praga, M., Biancone, L., Pani, A., Feriozzi, S., Polci, R., Barratt, J., Del Vecchio, L., Locatelli, F., Pierucci, A., Caliskan, Y., Perkowska-Ptasinska, A., Durlik, M., Moggia, E., Ballarin, J. C., Wetzels, J. F. M., Goumenos, D., Papasotiriou, M., Galesic, K., Toric, L., Papagianni, A., Stangou, M., Benozzi, L., Cusinato, S., Berg, U., Topaloglu, R., Maggio, M., Ots-Rosenberg, M., D'Amico, M., Geddes, C., Balafa, O., Quaglia, M., Cravero, R., Cirami, C. L., Fellstrom, B., Floege, J., Egido, J., Mallamaci, F., Zoccali, C., Fuiano, L., Beltrame, G., Camilla, R., Segoloni, G., Colla, L., Angioi, A., Piras, L., Cancarini, G., Ravera, S., Ballarin, J., Di Giulio, S., Pugliese, F., Serriello, I., Sever, M., Kilicaslan, I., Peters, H., Carvalho, F., Da Costa Ferreira, A. C., Wiecek, A., Magistroni, R., Bilginer, Y., Giacchino, F., Papastirou, M., Siamopoulos, K., Galliani, M., Stratta, P., Bergia, R., Salvadori, M., Cirami, L., Kloster Smerud, H., Ferrario, F., Stellato, T., Martin, C., Eitner, F., Rauen, T., Lupo, A., Bernich, P., Mene, P., Morosetti, M., Van Kooten, C., Rabelink, T., Reinders, M. E. J., Boria Grinyo, J. M., Savoldi, S., Licata, C., Mizerska-Wasiak, M., Roszkowska-Blaim, M., Martina, G., Messuerotti, A., Dal Canton, A., Esposito, C., Migotto, C., Triolo, G., Mariano, F., Pozzi, C., Boero, R., Mazzucco, G., Giannakakis, C., Honsova, E., Sundelin, B., Gutierrez, E., Asunis, A. M., Tardanico, R., Arce Terroba, J., Fortunato, M., Pantzaki, A., Ozluk, Y., Steenbergen, E., Soderberg, M., Riispere, Z., Furci, L., Orhan, D., Kipgen, D., Casartelli, D., Galesic Ljubanovic, D., Akiopoulou, H., Bertoni, E., Cannata Ortiz, P., Karkoszka, H., Groene, H. J., Stoppacciaro, A., Bajema, I., Bruijn, J., Fulladosa Oliveras, X., Maldyk, J., Ioachim, E., and Internal Medicine

Subjects
Adult, Male, Pathology, medicine.medical_specialty, Adolescent, 030232 urology & nephrology, Renal function, 030204 cardiovascular system & hematology, Kidney, Nephropathy, Cohort Studies, 03 medical and health sciences, Glomerulonephritis, 0302 clinical medicine, renal biopsy, Interquartile range, IgA nephropathy, progression, risk factors, Child, Disease Progression, Female, Follow-Up Studies, Glomerular Filtration Rate, Glomerulonephritis, IGA, Humans, Prognosis, Medicine, Endocapillary hypercellularity, IGA, Transplantation, medicine.diagnostic_test, business.industry, medicine.disease, medicine.anatomical_structure, Renal pathology, Nephrology, Cohort, Renal biopsy, Renal disorders Radboud Institute for Health Sciences [Radboudumc 11], business
Abstract

Background It is unknown whether renal pathology lesions in immunoglobulin A nephropathy (IgAN) correlate with renal outcomes over decades of follow-up. Methods In 1130 patients of the original Validation Study of the Oxford Classification for IgA Nephropathy (VALIGA) cohort, we studied the relationship between the MEST score (mesangial hypercellularity, M; endocapillary hypercellularity, E; segmental glomerulosclerosis, S; tubular atrophy/interstitial fibrosis, T), crescents (C) and other histological lesions with both a combined renal endpoint [50% estimated glomerular filtration rate (eGFR) loss or kidney failure] and the rate of eGFR decline over a follow-up period extending to 35 years [median 7 years (interquartile range 4.1–10.8)]. Results In this extended analysis, M1, S1 and T1–T2 lesions as well as the whole MEST score were independently related with the combined endpoint (P Conclusion Long-term follow-up analyses of the VALIGA cohort showed that the independent relationship between kidney biopsy findings and the risk of progression towards kidney failure in IgAN remains unchanged across all age groups and decades after the renal biopsy.

Published
2020

19. Development and validation of a nomogram to predict kidney survival at baseline in patients with C3 glomerulopathy

Author

Caravaca-Fontan, Fernando, Rivero, M., Cavero, T., Díaz Encarnación, Montserrat Mercedes, Cabello, V., Ariceta, G., Quintana, Luis F, Marco, H., Barros, X., Ramos, N., Rodríguez-Mendiola, N., Cruz, S., Fernández-Juárez, G., Rodríguez, A., De José, A.P., Rabasco, C., Rodado, R., Fernández, L., Pérez-Gómez, Maria Vanessa, Ávila, A., Bravo, L., Espinosa, N., Allende, N., De La Nieta, M.D.S., Rodríguez, E., Olea, T., Melgosa, M., Huerta, A., Miquel, R., Mon, C., Fraga, Gloria, De Lorenzo, A., Draibe, J., González, F., Shabaka, A., López-Rubio, M.E., Fenollosa, M.Á., Martín-Penagos, L., Da Silva, I., Alonso Titos, J., De Córdoba, S.R., De Jorge, E.G., Praga, M., Universitat Autònoma de Barcelona, Instituto de Salud Carlos III, Red Española de Investigación Renal, Comunidad de Madrid, Ministerio de Economía y Competitividad (España), Caravaca-Fontán, Fernando, Cavero, Teresa, Díaz-Encarnación, Montserrat M., Ariceta, Gema, Quintana, Luis F., Barros, Xoana, Ramos, Natalia, Rodríguez-Mendiola, Nuria, Fernández-Juárez, Gema, Pérez de José, Ana, Pérez Gómez, Vanessa, Allende, Natalia, Sánchez de la Nieta, María Dolores, Olea, Teresa, Melgosa, Marta, Huerta, Ana, Mon, Carmen, de Lorenzo, Alberto, Draibe, Juliana, González, Fayna, Shabaka, Amir, Martín-Penagos, L., Rodríguez de Córdoba, Santiago, Goicoechea de Jorge, Elena, Praga, Manuel, and Universidad de Cantabria

Subjects
nomogram, Transplantation, Nephrology, Calibration, Discrimination, Glomerulopathy, Kidney failure, C3 glomerulopathy, calibration, Nomogram, discrimination, kidney failure
Abstract

10 p.-4 fig.-2 tab. 1 graph. abst., Background: C3 glomerulopathy is a rare and heterogeneous complement-driven disease. It is often challenging to accurately predict in clinical practice the individual kidney prognosis at baseline. We herein sought to develop and validate a prognostic nomogram to predict long-term kidney survival., Methods: We conducted a retrospective, multicenter observational cohort study in 35 nephrology departments belonging to the Spanish Group for the Study of Glomerular Diseases. The dataset was randomly divided into a training group (n = 87) and a validation group (n = 28). The least absolute shrinkage and selection operator (LASSO) regression was used to screen the main predictors of kidney outcome and to build the nomogram. The accuracy of the nomogram was assessed by discrimination and risk calibration in the training and validation sets., Results: The study group comprised 115 patients, of whom 46 (40%) reached kidney failure in a median follow-up of 49 months (range 24–112). No significant differences were observed in baseline estimated glomerular filtration rate (eGFR), proteinuria or total chronicity score of kidney biopsies, between patients in the training versus those in the validation set. The selected variables by LASSO were eGFR, proteinuria and total chronicity score. Based on a Cox model, a nomogram was developed for the prediction of kidney survival at 1, 2, 5 and 10 years from diagnosis. The C-index of the nomogram was 0.860 (95% confidence interval 0.834–0.887) and calibration plots showed optimal agreement between predicted and observed outcomes., Conclusions: We constructed and validated a practical nomogram with good discrimination and calibration to predict the risk of kidney failure in C3 glomerulopathy patients at 1, 2, 5 and 10 years., Work on this study was supported by the Instituto de Salud Carlos III / Fondo Europeo de Desarrollo Regional (ISCIII/FEDER; grants PI16/01685 and PI19/1624) and Red de Investigación Renal (RD12/0021/0029; to M.P.) and the Autonomous Region of Madrid (S2017/BMD-3673; to M.P.). S.R.d.C. is supported by the Ministerio de Economia y Competitividad (grant PID2019-104912RB-I00) and the Autonomous Region of Madrid (grant S2017/BMD-3673).

Published
2022

20. Correction to: Efficacy and safety of the long-acting C5 inhibitor ravulizumab in patients with atypical hemolytic uremic syndrome triggered by pregnancy: a subgroup analysis (BMC Nephrology, (2021), 22, 1, (5), 10.1186/s12882-020-02190-0)

Author

Gackler A., Schonermarck U., Dobronravov V., La Manna G., Denker A., Liu P., Vinogradova M., Yoon S. -S., Praga M., Gackler A., Schonermarck U., Dobronravov V., La Manna G., Denker A., Liu P., Vinogradova M., Yoon S.-S., and Praga M.

Subjects
Ravulizumab, Thrombotic microangiopathy, Pregnancy microangiopathies, Atypical hemolytic uremic syndrome
Abstract

An amendment to this paper has been published and can be accessed via the original article.

Published
2021

28. Avacopan for the Treatment of ANCA-Associated Vasculitis

Author

Jayne, David R W, Merkel, Peter A, Schall, Thomas J, Bekker, Pirow, ADVOCATE Study Group:, C Au Peh, Chakera, A, Cooper, B, Kurtkoti, J, Langguth, D, Levidiotis, V, Luxton, G, Mount, P, Mudge, D, Noble, E, Phoon, R, Ranganathan, D, Ritchie, A, Ryan, J, Suranyi, M, Rosenkranz, A, Lhotta, K, Kronbichler, A, Demoulin, N, Bovy, C, Hellemans, R, Hougardy, J, Sprangers, B, Wissing, K, Pagnoux, C, Barbour, S, Brachemi, S, Cournoyer, S, Girard, L, Laurin, L, Liang, P, Philibert, D, Walsh, M, Tesar, V, Becvar, R, Horak, P, Rychlik, I, Szpirt, W, Dieperink, H, Gregersen, J, Ivarsen, P, Krarup, E, Lyngsoe, C, Rigothier, C, Augusto, J, Belot, A, Chauveau, D, Cornec, D, Jourde-Chiche, N, Ficheux, M, Karras, A, Klein, A, Maurier, F, Mesbah, R, Moranne, O, Neel, A, Quemeneur, T, Saadoun, D, Terrier, B, Zaoui, P, Schaier, M, Benck, U, Bergner, R, Busch, M, Floege, J, Grundmann, F, Haller, H, Haubitz, M, Hellmich, B, Henes, J, Hohenstein, B, Hugo, C, Iking-Konert, C, Arndt, F, Kubacki, T, Kotter, I, Lamprecht, P, Lindner, T, Halbritter, J, Mehling, H, Schönermarck, U, Venhoff, N, Vielhauer, V, Witzke, O, Szombati, I, Szucs, G, Garibotto, G, Alberici, F, Brunetta, E, Dagna, L, S De Vita, Emmi, G, Gabrielli, A, Manenti, L, Pieruzzi, F, Roccatello, D, Salvarani, C, Dobashi, H, Atsumi, T, Fujimoto, S, Hagino, N, Ihata, A, Kaname, S, Kaneko, Y, Katagiri, A, Katayama, M, Kirino, Y, Kitagawa, K, Komatsuda, A, Kono, H, Kurasawa, T, Matsumura, R, Mimura, T, Morinobu, A, Murakawa, Y, Naniwa, T, Nanki, T, Ogawa, N, Oshima, H, Sada, K, Sugiyama, E, Takeuchi, T, Taki, H, Tamura, N, Tsukamoto, T, Yamagata, K, Yamamura, M, P van Daele, Rutgers, A, Teng, Y, Walker, R, Chua, I, Collins, M, Rabindranath, K, J de Zoysa, Svensson, M, Grevbo, B, Kalstad, S, Little, M, Clarkson, M, Molloy, E, I Agraz Pamplona, Anton, J, V Barrio Lucia, Ciggaran, S, M Cinta Cid, M Diaz Encarnacion, X Fulladosa Oliveras, M Soler, J, H Rusinol, M, Praga, M, L Quintana Porras, Segarra, A, Bruchfeld, A, Segelmark, M, Soveri, I, Thomaidi, E, Westman, K, Neumann, T, Burnier, M, Daikeler, T, Dudler, J, Hauser, T, Seeger, H, Vogt, B, Jayne, D, Burton, J, R Al Jayyousi, Amin, T, Andrews, J, Baines, L, Brogan, P, Dasgupta, B, Doulton, T, Flossmann, O, Griffin, S, Harper, J, Harper, L, Kidder, D, Klocke, R, Lanyon, P, Luqmani, R, Mclaren, J, Makanjuola, D, Mccann, L, Nandagudi, A, Selvan, S, O'Riordan, E, Patel, M, Patel, R, Pusey, C, Rajakariar, R, Robson, J, Robson, M, Salama, A, Smyth, L, Sznajd, J, Taylor, J, Merkel, P, Sreih, A, Belilos, E, Bomback, A, Carlin, J, Y Chang Chen Lin, Derebail, V, Dragoi, S, Dua, A, Forbess, L, Geetha, D, Gipson, P, Gohh, R, T Greenwood, G, Hugenberg, S, Jimenez, R, Kaskas, M, Kermani, T, Kivitz, A, Koening, C, Langford, C, Marder, G, Mohamed, A, Monach, P, Neyra, N, Niemer, G, Niles, J, Obi, R, Owens, C, Parks, D, Podoll, A, Rovin, B, Sam, R, Shergy, W, Silva, A, Specks, U, Spiera, R, Springer, J, Striebich, C, Swarup, A, Thakar, S, Tiliakos, A, Tsai, Y, Waguespack, D, M Chester Wasko, Internal Medicine, Immunology, Jayne, D, Merkel, P, Schall, T, Bekker, P, Pieruzzi, F, Jayne, David RW [0000-0002-1712-0637], Apollo - University of Cambridge Repository, and Translational Immunology Groningen (TRIGR)

Subjects
Male, Receptor, Anaphylatoxin C5a/antagonists & inhibitors, Anaphylatoxin C5a, Anaphylatoxin C5a/antagonists & inhibitors, Nipecotic Acids, Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculiti, Administration, Oral, Azathioprine, Kaplan-Meier Estimate, 030204 cardiovascular system & hematology, urologic and male genital diseases, Gastroenterology, C5a receptor, Cyclophosphamide/administration & dosage, Rituximab/administration & dosage, Immunosuppressive Agent, renal vasculitis, Prednisone/administration & dosage, 0302 clinical medicine, Glucocorticoid, immune system diseases, Prednisone, Recurrence, Medicine, 030212 general & internal medicine, skin and connective tissue diseases, Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis/drug therapy, Aniline Compounds, Remission Induction, General Medicine, Aniline Compound, Middle Aged, Administration, Combination, Rituximab, Drug Therapy, Combination, Female, Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis, Cyclophosphamide, Double-Blind Method, Glucocorticoids, Humans, Immunosuppressive Agents, Receptor, Anaphylatoxin C5a, Vasculitis, Receptor, medicine.drug, Human, Azathioprine/administration & dosage, Oral, medicine.medical_specialty, Nipecotic Acid, ANCA-Associated Vasculitis, 03 medical and health sciences, Drug Therapy, Internal medicine, Aniline Compounds/adverse effects, cardiovascular diseases, Immunosuppressive Agents/administration & dosage, Anti-neutrophil cytoplasmic antibody, business.industry, Glucocorticoids/administration & dosage, medicine.disease, Nipecotic Acids/adverse effects, respiratory tract diseases, business
Abstract

BACKGROUND: The C5a receptor inhibitor avacopan is being studied for the treatment of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis.METHODS: In this randomized, controlled trial, we assigned patients with ANCA-associated vasculitis in a 1:1 ratio to receive oral avacopan at a dose of 30 mg twice daily or oral prednisone on a tapering schedule. All the patients received either cyclophosphamide (followed by azathioprine) or rituximab. The first primary end point was remission, defined as a Birmingham Vasculitis Activity Score (BVAS) of 0 (on a scale from 0 to 63, with higher scores indicating greater disease activity) at week 26 and no glucocorticoid use in the previous 4 weeks. The second primary end point was sustained remission, defined as remission at both weeks 26 and 52. Both end points were tested for noninferiority (by a margin of 20 percentage points) and for superiority.RESULTS: A total of 331 patients underwent randomization; 166 were assigned to receive avacopan, and 165 were assigned to receive prednisone. The mean BVAS at baseline was 16 in both groups. Remission at week 26 (the first primary end point) was observed in 120 of 166 patients (72.3%) receiving avacopan and in 115 of 164 patients (70.1%) receiving prednisone (estimated common difference, 3.4 percentage points; 95% confidence interval [CI], -6.0 to 12.8; PCONCLUSIONS: In this trial involving patients with ANCA-associated vasculitis, avacopan was noninferior but not superior to prednisone taper with respect to remission at week 26 and was superior to prednisone taper with respect to sustained remission at week 52. All the patients received cyclophosphamide or rituximab. The safety and clinical effects of avacopan beyond 52 weeks were not addressed in the trial. (Funded by ChemoCentryx; ADVOCATE ClinicalTrials.gov number, NCT02994927.).

Published
2021

29. Quantifying duration of proteinuria remission and association with clinical outcome in IgA nephropathy

Author

Canney, M., Barbour, S. J., Zheng, Y., Coppo, R., Zhang, H., Liu, Z. -H., Matsuzaki, K., Suzuki, Y., Katafuchi, R., Reich, H. N., Cattran, D., Russo, M. L., Troyanov, S., Cook, H. T., Roberts, I., Tesar, V., Maixnerova, D., Lundberg, S., Gesualdo, L., Emma, F., Fuiano, L., Beltrame, G., Rollino, C., Amore, A., Camilla, R., Peruzzi, L., Praga, M., Feriozzi, S., Polci, R., Segoloni, G., Colla, L., Pani, A., Piras, D., Angioi, A., Cancarini, G., Ravera, S., Durlik, M., Moggia, E., Ballarin, J., Di Giulio, S., Pugliese, F., Serriello, I., Caliskan, Y., Sever, M., Kilicaslan, I., Locatelli, F., Del Vecchio, L., Wetzels, J. F. M., Peters, H., Berg, U., Carvalho, F., da Costa Ferreira, A. C., Maggio, M., Wiecek, A., Ots-Rosenberg, M., Magistroni, R., Topaloglu, R., Bilginer, Y., D'Amico, M., Stangou, M., Giacchino, F., Goumenos, D., Kalliakmani, P., Gerolymos, M., Galesic, K., Geddes, C., Siamopoulos, K., Balafa, O., Galliani, M., Stratta, P., Quaglia, M., Bergia, R., Cravero, R., Salvadori, M., Cirami, L., Fellstrom, B., Kloster Smerud, H., Ferrario, F., Stellato, T., Egido, J., Martin, C., Floege, J., Eitner, F., Lupo, A., Bernich, P., Mene, P., Morosetti, M., van Kooten, C., Rabelink, T., Reinders, M. E. J., Boria Grinyo, J. M., Cusinato, S., Benozzi, L., Savoldi, S., Licata, C., Mizerska-Wasiak, M., Martina, G., Messuerotti, A., Dal Canton, A., Esposito, C., Migotto, C., Triolo, G., Mariano, F., Pozzi, C., Boero, R., Bellur, S., Mazzucco, G., Giannakakis, C., Honsova, E., Sundelin, B., Di Palma, A. M., Gutierrez, E., Asunis, A. M., Barratt, J., Tardanico, R., Perkowska-Ptasinska, A., Arce Terroba, J., Fortunato, M., Pantzaki, A., Ozluk, Y., Steenbergen, E., Soderberg, M., Riispere, Z., Furci, L., Orhan, D., Kipgen, D., Casartelli, D., Galesic Ljubanovic, D., Gakiopoulou, H., Bertoni, E., Cannata Ortiz, P., Karkoszka, H., Groene, H. J., Stoppacciaro, A., Bajema, I., Bruijn, J., Fulladosa Oliveras, X., Maldyk, J., Ioachim, E., Bavbek, N., Cook, T., Alpers, C., Feehally, J., Berthoux, F., Bonsib, S., D'Agati, V., D'Amico, G., Emancipator, S., Emmal, F., Fervenza, F., Florquin, S., Fogo, A., Groene, H., Haas, M., Hill, P., Hogg, R., Hsu, S., Hunley, T., Hladunewich, Jennette, C., Joh, K., Julian, B., Kawamura, T., Lai, F., Leung, C., Li, L., Li, P., Liu, Z., Massat, A., Mackinnon, B., Mezzano, S., Schena, F., Tomino, Y., Walker, P., Wang, H., Weening, J., Yoshikawa, N., Zeng, C. -H., Shi, S., Nogi, C., Suzuki, H., Koike, K., Hirano, K., Yokoo, T., Hanai, M., Fukami, K., Takahashi, K., Yuzawa, Y., Niwa, M., Yasuda, Y., Maruyama, S., Ichikawa, D., Suzuki, T., Shirai, S., Fukuda, A., Fujimoto, S., Trimarchi, H., Anesthesiology, Pathology, Graduate School, ACS - Heart failure & arrhythmias, and ACS - Atherosclerosis & ischemic syndromes

Subjects
Male, Time Factors, glomerular disease, 030232 urology & nephrology, 030204 cardiovascular system & hematology, Gastroenterology, 0302 clinical medicine, Outcome Assessment, Health Care, Clinical endpoint, Medicine, Clinical Epidemiology, Proteinuria, medicine.diagnostic_test, Remission Induction, Hazard ratio, IgA nephropathy, General Medicine, Middle Aged, end stage kidney disease, epidemiology and outcomes, proteinuria, renal function decline, renal pathology, 3. Good health, Renal pathology, Nephrology, Disease Progression, Female, medicine.symptom, Glomerular Filtration Rate, Adult, medicine.medical_specialty, Nephropathy, 03 medical and health sciences, Internal medicine, Biopsy, Humans, Proportional Hazards Models, Retrospective Studies, business.industry, Surrogate endpoint, Glomerulonephritis, IGA, medicine.disease, Confidence interval, Renal disorders Radboud Institute for Molecular Life Sciences [Radboudumc 11], Kidney Failure, Chronic, Renal disorders Radboud Institute for Health Sciences [Radboudumc 11], business
Abstract

Contains fulltext : 232930.pdf (Publisher’s version ) (Closed access) BACKGROUND: On the basis of findings of observational studies and a meta-analysis, proteinuria reduction has been proposed as a surrogate outcome in IgA nephropathy. How long a reduction in proteinuria needs to be maintained to mitigate the long-term risk of disease progression is unknown. METHODS: In this retrospective multiethnic cohort of adult patients with IgA nephropathy, we defined proteinuria remission as a ≥25% reduction in proteinuria from the peak value after biopsy, and an absolute reduction in proteinuria to

Published
2021

30. Pre-emptive rituximab in focal and segmental glomerulosclerosis patients at risk of recurrence after kidney transplantation

Author

Aunon P, Polanco N, Perez-Saez M, Rodrigo E, Sancho A, Pascual J, Andres A, and Praga M

Subjects
focal segmental glomerulosclerosis, recurrence, rituximab, kidney transplantation
Abstract

Background. The recurrence of proteinuria after kidney transplantation (KT) is a characteristic complication of focal segmental glomerulosclerosis (FSGS). It has been suggested that pre-emptive rituximab might prevent recurrences in patients at risk, but there is no agreement about which factors might help to identify such patients. Methods. We studied 93 kidney transplants with biopsy-proven idiopathic FSGS in order to analyse if preventive rituximab treatment could decrease recurrences in patients at risk. Results. Fifteen patients (16.1%) presented a recurrence after KT, but when we restricted the analysis to the 34 patients presenting nephrotic syndrome at primary disease onset, the recurrence diagnosis rate increased to 44.1%. All patients with recurrence had complete nephrotic syndrome at the time of diagnosis. After multivariate adjustment, the only significant risk factor for recurrence was the presence of complete nephrotic syndrome at diagnosis. Twelve of the 34 patients at risk for recurrence received rituximab at the time of transplantation. Clinical and analytical characteristics were similar in all patients at risk. The number of recurrences was similar among treated (50%) and non-treated patients (40.9%). Conclusions. Nephrotic syndrome with hypoalbuminaemia at diagnosis is the most important feature to identify patients at risk of recurrence. Our data suggest that pre-emptive rituximab is not effective to prevent FSGS recurrences.

Published
2021

31. Quantifying duration of proteinuria remission and association with clinical outcome in IgA nephropathy

Author

Canney, M. Barbour, S.J. Zheng, Y. Coppo, R. Zhang, H. Liu, Z.-H. Matsuzaki, K. Suzuki, Y. Katafuchi, R. Reich, H.N. Cattran, D. Russo, M.L. Troyanov, S. Cook, H.T. Roberts, I. Tesar, V. Maixnerova, D. Lundberg, S. Gesualdo, L. Emma, F. Fuiano, L. Beltrame, G. Rollino, C. Amore, A. Camilla, R. Peruzzi, L. Praga, M. Feriozzi, S. Polci, R. Segoloni, G. Colla, L. Pani, A. Piras, D. Angioi, A. Cancarini, G. Ravera, S. Durlik, M. Moggia, E. Ballarin, J. Di Giulio, S. Pugliese, F. Serriello, I. Caliskan, Y. Sever, M. Kilicaslan, I. Locatelli, F. Del Vecchio, L. Wetzels, J.F.M. Peters, H. Berg, U. Carvalho, F. da Costa Ferreira, A.C. Maggio, M. Wiecek, A. Ots-Rosenberg, M. Magistroni, R. Topaloglu, R. Bilginer, Y. D'Amico, M. Stangou, M. Giacchino, F. Goumenos, D. Kalliakmani, P. Gerolymos, M. Galesic, K. Geddes, C. Siamopoulos, K. Balafa, O. Galliani, M. Stratta, P. Quaglia, M. Bergia, R. Cravero, R. Salvadori, M. Cirami, L. Fellstrom, B. Kloster Smerud, H. Ferrario, F. Stellato, T. Egido, J. Martin, C. Floege, J. Eitner, F. Lupo, A. Bernich, P. Menè, P. Morosetti, M. van Kooten, C. Rabelink, T. Reinders, M.E.J. Boria Grinyo, J.M. Cusinato, S. Benozzi, L. Savoldi, S. Licata, C. Mizerska-Wasiak, M. Martina, G. Messuerotti, A. Dal Canton, A. Esposito, C. Migotto, C. Triolo, G. Mariano, F. Pozzi, C. Boero, R. Bellur, S. Mazzucco, G. Giannakakis, C. Honsova, E. Sundelin, B. Di Palma, A.M. Gutiérrez, E. Asunis, A.M. Barratt, J. Tardanico, R. Perkowska-Ptasinska, A. Arce Terroba, J. Fortunato, M. Pantzaki, A. Ozluk, Y. Steenbergen, E. Soderberg, M. Riispere, Z. Furci, L. Orhan, D. Kipgen, D. Casartelli, D. Galesic Ljubanovic, D. Gakiopoulou, H. Bertoni, E. Cannata Ortiz, P. Karkoszka, H. Groene, H.J. Stoppacciaro, A. Bajema, I. Bruijn, J. Fulladosa Oliveras, X. Maldyk, J. Ioachim, E. Bavbek, N. Cook, T. Alpers, C. Feehally, J. Berthoux, F. Bonsib, S. D'Agati, V. D'Amico, G. Emancipator, S. Emmal, F. Fervenza, F. Florquin, S. Fogo, A. Groene, H. Haas, M. Hill, P. Hogg, R. Hsu, S. Hunley, T. Hladunewich Jennette, C. Joh, K. Julian, B. Kawamura, T. Lai, F. Leung, C. Li, L. Li, P. Liu, Z. Massat, A. Mackinnon, B. Mezzano, S. Schena, F. Tomino, Y. Walker, P. Wang, H. Weening, J. Yoshikawa, N. Zeng, C.-H. Shi, S. Nogi, C. Suzuki, H. Koike, K. Hirano, K. Yokoo, T. Hanai, M. Fukami, K. Takahashi, K. Yuzawa, Y. Niwa, M. Yasuda, Y. Maruyama, S. Ichikawa, D. Suzuki, T. Shirai, S. Fukuda, A. Fujimoto, S. Trimarchi, H. International IgA Nephropathy Network

Abstract

Background On the basis of findings of observational studies and a meta-analysis, proteinuria reduction has been proposed as a surrogate outcome in IgA nephropathy. How long a reduction in proteinuria needs to be maintained to mitigate the long-term risk of disease progression is unknown. Methods In this retrospective multiethnic cohort of adult patients with IgA nephropathy, we defined proteinuria remission as a $25% reduction in proteinuria from the peak value after biopsy, and an absolute reduction in proteinuria to,1 g/d. The exposure of interest was the total duration of first remission, treated as a time-varying covariate using longitudinal proteinuria measurements. We used time-dependent Cox proportional hazards regression models to quantify the association between the duration of remission and the primary outcome (ESKD or a 50% reduction in eGFR). Results During a median follow-up of 3.9 years, 274 of 1864 patients (14.7%) experienced the primary outcome. The relationship between duration of proteinuria remission and outcome was nonlinear. Each 3 months in sustained remission up to approximately 4 years was associated with an additional 9% reduction in the risk of disease progression (hazard ratio [HR], 0.91; 95% confidence interval [95% CI], 0.89 to 0.93). Thereafter, each additional 3 months in remission was associated with a smaller, nonsignificant risk reduction (HR, 0.99; 95% CI, 0.96 to 1.03). These findings were robust to multivariable adjustment and consistent across clinical and histologic subgroups. Conclusions Our findings support the use of proteinuria as a surrogate outcome in IgA nephropathy, but additionally demonstrate the value of quantifying the duration of proteinuria remission when estimating the risk of hard clinical endpoints. Copyright © 2021 by the American Society of Nephrology.

Published
2021

32. Recurrent acute interstitial nephritis: what lies beneath

Author

Caravaca-Fontan F, Shabaka A, Sanchez-Alamo B, de Lorenzo A, Diaz M, Blasco M, Rodriguez E, Sierra-Carpio M, Marin T, Urrestarazu A, Cases C, Praga M, Fernandez-Juarez G, and Spanish Grp Study Glomerular Dis G

Subjects
acute interstitial nephritis, recurrent acute interstitial nephritis, chronic kidney disease
Abstract

Background. Acute interstitial nephritis (AIN) is an emerging cause of acute kidney disease. While this disease usually follows an acute course, it may occasionally recur, representing a major challenge for the clinician. Methods. We performed a retrospective, observational cohort study in 13 nephrology departments belonging to the Spanish Group for the Study of Glomerular Diseases. Patients with biopsy-proven AIN between 1996 and 2018 were included. Results. The study group consisted of 205 patients with AIN, 22 of which developed recurrent AIN (RAIN) after a median of 111 days from diagnosis. RAIN was due to a surreptitious reintroduction of a previously known implicated drug or toxic in six patients (27%), sarcoidosis in two (9%), Sjogren's syndrome in three (14%), light-chain-mediated AIN in two (9%) and tubulointerstitial nephritis and uveitis syndrome in two (9%), while in the rest of cases (32%), no precise cause could be identified. Microscopic haematuria was more frequent in patients with underlying systemic diseases. The first RAIN episode was treated with a repeated course of corticosteroids in 21 patients (95%). In six cases (27%), azathioprine and mycophenolate mofetil were added as corticosteroid-sparing agents. During a median follow-up of 30 months, 50 patients (27%) with no recurrences and 12 patients (55%) with RAIN reached Stages 4 and 5 chronic kidney disease (CKD). By multivariable logistic regression analysis, RAIN was independently associated with the risk of reaching Stages 4 and 5 CKD, even after adjusting for potential covariables. Conclusions. RAIN is infrequent but is associated with poor kidney survival. RAIN should prompt clinicians to search for an underlying aetiology other than drug induced. However, in a large percentage of cases, no precise cause can be identified.

Published
2021

33. The STARMEN trial indicates that alternating treatment with corticosteroids and cyclophosphamide is superior to sequential treatment with tacrolimus and rituximab in primary membranous nephropathy

Author

Fernández-Juárez, G., Rojas-Rivera, J., Logt, A. van de, Justino, J., Sevillano, A., Caravaca-Fontán, F., Ávila, A., Rabasco, C., Cabello, V., Varela, A., Díez, M., Martín-Reyes, G., Diezhandino, M.G., Quintana, L.F., Agraz, I., Gómez-Martino, J.R., Cao, M., Rodríguez-Moreno, A., Rivas, B., Galeano, C., Bonet, J., Romera, A., Shabaka, A., Plaisier, E., Espinosa, M., Egido, J., Segarra, A., Lambeau, G., Ronco, P., Wetzels, J., Praga, M., Fernández-Juárez, G., Rojas-Rivera, J., Logt, A. van de, Justino, J., Sevillano, A., Caravaca-Fontán, F., Ávila, A., Rabasco, C., Cabello, V., Varela, A., Díez, M., Martín-Reyes, G., Diezhandino, M.G., Quintana, L.F., Agraz, I., Gómez-Martino, J.R., Cao, M., Rodríguez-Moreno, A., Rivas, B., Galeano, C., Bonet, J., Romera, A., Shabaka, A., Plaisier, E., Espinosa, M., Egido, J., Segarra, A., Lambeau, G., Ronco, P., Wetzels, J., and Praga, M.

Abstract

Item does not contain fulltext, A cyclical corticosteroid-cyclophosphamide regimen is recommended for patients with primary membranous nephropathy at high risk of progression. We hypothesized that sequential therapy with tacrolimus and rituximab is superior to cyclical alternating treatment with corticosteroids and cyclophosphamide in inducing persistent remission in these patients. This was tested in a randomized, open-label controlled trial of 86 patients with primary membranous nephropathy and persistent nephrotic syndrome after six-months observation and assigned 43 each to receive six-month cyclical treatment with corticosteroid and cyclophosphamide or sequential treatment with tacrolimus (full-dose for six months and tapering for another three months) and rituximab (one gram at month six). The primary outcome was complete or partial remission of nephrotic syndrome at 24 months. This composite outcome occurred in 36 patients (83.7%) in the corticosteroid-cyclophosphamide group and in 25 patients (58.1%) in the tacrolimus-rituximab group (relative risk 1.44; 95% confidence interval 1.08 to 1.92). Complete remission at 24 months occurred in 26 patients (60%) in the corticosteroid-cyclophosphamide group and in 11 patients (26%) in the tacrolimus-rituximab group (2.36; 1.34 to 4.16). Anti-PLA2R titers showed a significant decrease in both groups but the proportion of anti-PLA2R-positive patients who achieved immunological response (depletion of anti-PLA2R antibodies) was significantly higher at three and six months in the corticosteroid-cyclophosphamide group (77% and 92%, respectively), as compared to the tacrolimus-rituximab group (45% and 70%, respectively). Relapses occurred in one patient in the corticosteroid-cyclophosphamide group, and three patients in the tacrolimus-rituximab group. Serious adverse events were similar in both groups. Thus, treatment with corticosteroid-cyclophosphamide induced remission in a significantly greater number of patients with primary membranous nephropathy tha

Published
2021

40. 2019 Update of the Joint European League against Rheumatism and European Renal Association-European Dialysis and Transplant Association (EULAR/ERA-EDTA) recommendations for the management of lupus nephritis

Author

Fanouriakis, A. Kostopoulou, M. Cheema, K. Anders, H. J. and Aringer, M. Bajema, I. Boletis, J. N. Frangou, E. and Houssiau, F. Hollis, J. Karras, A. Marchiori, F. Marks, S. Moroni, G. Mosca, M. Parodis, I. Praga, M. and Schneider, M. Smolen, J. S. Tesar, V. Trachana, M. and Vollenhoven, R. V. Voskuyl, A. Teng, Y. K. O. Van Leeuw, B. and Bertsias, G. Jayne, D. Boumpas, D.

Abstract

Objective To update the 2012 EULAR/ERA-EDTA recommendations for the management of lupus nephritis (LN). Methods Following the EULAR standardised operating procedures, a systematic literature review was performed. Members of a multidisciplinary Task Force voted independently on their level of agreeement with the formed statements. Results The changes include recommendations for treatment targets, use of glucocorticoids and calcineurin inhibitors (CNIs) and management of end-stage kidney disease (ESKD). The target of therapy is complete response (proteinuria 1 g/24 hours despite renin-Angiotensin-Aldosterone blockade, MMF in combination with glucocorticoids is preferred. Assessment for kidney and extra-renal disease activity, and management of comorbidities is lifelong with repeat kidney biopsy in cases of incomplete response or nephritic flares. In ESKD, transplantation is the preferred kidney replacement option with immunosuppression guided by transplant protocols and/or extra-renal manifestations. Treatment of LN in children follows the same principles as adult disease. Conclusions We have updated the EULAR recommendations for the management of LN to facilitate homogenization of patient care. © Author(s) (or their employer(s)) 2020. No commercial re-use. See rights and permissions. Published by BMJ.

Published
2020

41. Mycophenolate Mofetil in C3 Glomerulopathy and Pathogenic Drivers of the Disease

Author

Caravaca-Fontán F, Díaz-Encarnación MM, Lucientes L, Cavero T, Cabello V, Ariceta G, Quintana LF, Marco H, Barros X, Ramos N, Rodríguez-Mendiola N, Cruz S, Fernández-Juárez G, Rodríguez A, Pérez de José A, Rabasco C, Rodado R, Fernández L, Pérez Gómez V, Ávila AI, Bravo L, Lumbreras J, Allende N, Sanchez de la Nieta MD, Rodríguez E, Olea T, Melgosa M, Huerta A, Miquel R, Mon C, Fraga G, de Lorenzo A, Draibe J, Cano-Megías M, González F, Shabaka A, López-Rubio ME, Fenollosa MÁ, Martín-Penagos L, Da Silva I, Alonso Titos J, Rodríguez de Córdoba S, Goicoechea de Jorge E, Praga M, Spanish Group for the Study of Glomerular Diseases GLOSEN, Instituto de Salud Carlos III, Red Española de Investigación Renal, Comunidad de Madrid, Ministerio de Ciencia, Innovación y Universidades (España), Ministerio de Economía y Competitividad (España), Caravaca-Fontán, Fernando [0000-0002-5830-9663], Díaz-Encarnación, Montserrat M. [0000-0001-5172-3370], Lucientes, Laura [0000-0001-5596-370X], Cavero, Teresa [0000-0001-5187-9906], Ariceta, Gema [0000-0003-1763-1098], Quintana, Luis F. [0000-0001-7582-8476], Barros, Xoana [0000-0001-9690-9769], Ramos, Natalia [0000-0001-9832-326X], Rodríguez-Mendiola, Nuria [0000-0001-6994-7161], Fernández-Juárez, Gema [0000-0001-6641-7763], Pérez de José, Ana [0000-0002-6952-1459], Pérez Gómez, Vanessa [0000-0003-4558-5236], Lumbreras, Javier [0000-0003-1855-0724], Sánchez de la Nieta, María Dolores [0000-0001-8574-0013], Olea, Teresa [0000-0003-2370-1048], Melgosa, Marta [0000-0001-6236-414X], Huerta, Ana [0000-0003-3342-7628], de Lorenzo, Alberto [0000-0001-8847-083X], Draibe, Juliana [0000-0002-2819-8560], González, Fayna [0000-0002-2313-2511], Shabaka, Amir [0000-0001-7039-4701], Martín-Penagos, L. [0000-0003-0159-7358], Rodríguez de Córdoba, Santiago [0000-0001-6401-1874], Praga, Manuel [0000-0001-9270-1071], Goicoechea de Jorge, Elena [0000-0002-4978-2483], Caravaca-Fontán, Fernando, Díaz-Encarnación, Montserrat M., Lucientes, Laura, Cavero, Teresa, Ariceta, Gema, Quintana, Luis F., Barros, Xoana, Ramos, Natalia, Rodríguez-Mendiola, Nuria, Fernández-Juárez, Gema, Pérez de José, Ana, Pérez Gómez, Vanessa, Lumbreras, Javier, Sánchez de la Nieta, María Dolores, Olea, Teresa, Melgosa, Marta, Huerta, Ana, de Lorenzo, Alberto, Draibe, Juliana, González, Fayna, Shabaka, Amir, Martín-Penagos, L., Rodríguez de Córdoba, Santiago, Praga, Manuel, and Goicoechea de Jorge, Elena

Subjects
Nephrology, Male, Time Factors, Epidemiology, 030232 urology & nephrology, Disease, Critical Care and Intensive Care Medicine, Gastroenterology, 0302 clinical medicine, Glomerulonephritis, Adrenal Cortex Hormones, Recurrence, Risk Factors, Medicine, C3 glomerulopathy, Child, 0303 health sciences, Proteinuria, Mycophenolate mofetil, Remission Induction, Complement C3, Middle Aged, Treatment Outcome, Alternative complement pathway, Disease Progression, Drug Therapy, Combination, Female, medicine.symptom, Immunosuppressive Agents, Cohort study, Adult, medicine.medical_specialty, Adolescent, Lower risk, 03 medical and health sciences, Young Adult, Glomerulopathy, Internal medicine, Humans, mycophenolate mofetil, 030304 developmental biology, Retrospective Studies, Transplantation, business.industry, Mycophenolic Acid, medicine.disease, Discontinuation, Spain, Propensity score matching, business
Abstract

12 p.-4 fig.-4 tab., BACKGROUND AND OBJECTIVES: C3 glomerulopathy is a complement-mediated disease arising from abnormalities in complement genes and/or antibodies against complement components. Previous studies showed that treatment with corticosteroids plus mycophenolate mofetil (MMF) was associated with improved outcomes, although the genetic profile of these patients was not systematically analyzed. This study aims to analyze the main determinants of disease progression and response to this therapeutic regimen., DESIGN, SETTING, PARTICIPANTS, & MEASUREMENTS: We conducted a retrospective, multicenter, observational cohort study in 35 nephrology departments belonging to the Spanish Group for the Study of Glomerular Diseases. Patients diagnosed with C3 glomerulopathy (n=81) or dense deposit disease (n=16) between January 1995 and March 2018 were enrolled. Multivariable and propensity score matching analyses were used to evaluate the association of clinical and genetic factors with response to treatment with corticosteroids and MMF as measured by proportion of patients with disease remission and kidney survival (status free of kidney failure)., RESULTS: The study group comprised 97 patients (84% C3 glomerulopathy, 16% dense deposit disease). Forty-two patients were treated with corticosteroids plus MMF, and this treatment was associated with a higher rate of remission and lower probability of kidney failure (79% and 14%, respectively) compared with patients treated with other immunosuppressives (24% and 59%, respectively), or ecluzimab (33% and 67%, respectively), or conservative management (18% and 65%, respectively). The therapeutic superiority of corticosteroids plus MMF was observed both in patients with complement abnormalities and with autoantibodies. However, patients with pathogenic variants in complement genes only achieved partial remission, whereas complete remissions were common among patients with autoantibody-mediated forms. The main determinant of no remission was baseline proteinuria. Relapses occurred after treatment discontinuation in 33% of the patients who had achieved remission with corticosteroids plus MMF, and a longer treatment length of MMF was associated with a lower risk of relapse., CONCLUSIONS: The beneficial response to corticosteroids plus MMF treatment in C3 glomerulopathy appears independent of the pathogenic drivers analyzed in this study., Work in this study was supported by the Instituto de Salud CarlosIII/ Fondo Europeo de Desarrollo Regional (ISCIII/FEDER) grant PI16/01685 and Red de Investigación Renal (RedInRen) (RD12/0021/0029) (to M. Praga), and the Autonomous Region of Madrid (S2017/BMD-3673) (to S. Rodríguez de Córdoba and M. Praga).E. Goicoechea de Jorge is supported by the Spanish “Ministerio de Ciencia, Innovación y Universidades” (RYC-2013-13395 and RTI2018-095955-B-100). S. Rodríguez de Córdoba is supported by Ministerio de Economía y Competitividad/FEDER grant SAF2015-66287R and Autonomous Region of Madrid grant S2017/BMD3673.

Published
2020

42. Spanish Group for the Study of Glomerular Diseases GLOSEN: Mycophenolate Mofetil in C3 Glomerulopathy and Pathogenic Drivers of the Disease (vol 15, pg 1287, 2020)

Author

Caravaca-Fontan F, Diaz-Encarnacion M, Lucientes L, Cavero T, Cabello V, Ariceta G, Quintana L, Marco H, Barros X, Ramos N, Rodriguez-Mendiola N, Cruz S, Fernandez-Juarez G, Rodriguez A, de Jose A, Rabasco C, Rodado R, Fernandez L, Gomez V, Avila A, Bravo L, Lumbreras J, Allende N, de la Nieta M, Rodriguez E, Olea T, Melgosa M, Huerta A, Miquel R, Mon C, Fraga G, de Lorenzo A, Draibe J, Cano-Megias M, Gonzalez F, Shabaka A, Lopez-Rubio M, Fenollosa M, Martin-Penagos L, Da Silva I, Titos J, de Cordoba S, de Jorge E, and Praga M

Subjects
erratum, Correction
Published
2020

43. Efectos adversos de la acumulación renal de hemoproteínas: Nuevas herramientas terapéuticas

Author

Guerrero-Hue, M, Rubio-Navarro, A, Sevillano, A, Yuste, C, Gutierrez, E, Palomino-Antolin, A, Roman, E, Praga, M, Egido, J, Moreno, JA, and Instituto de Investigación Sanitaria Fundación Jiménez Díaz (IIS-FJD)

Subjects
Hemeproteins, Medicina, Hemoglobinuria, Iron Chelating Agents, Kidney, lcsh:RC870-923, Hemolysis, Rhabdomyolysis, Acute kidney failure, Rabdomiólisis, Chronic kidney disease, Humans, Renal Insufficiency, Chronic, Enfermedad renal crónica, Hematuria, Clinical Trials as Topic, Hemoglobina, Mioglobina, Myoglobin, Therapies, Investigational, Haemoglobinuria, Blood Proteins, Drugs, Investigational, Acute Kidney Injury, lcsh:Diseases of the genitourinary system. Urology, Fracaso renal agudo, Oxidative Stress, Kidney Tubules, Sodium Bicarbonate, Nephrology, Haemoglobin, Haematuria
Abstract

Haemoglobin and myoglobin are haem proteins that play a key role as they help transport oxygen around the body. However, because of their chemical structure, these molecules can exert harmful effects when they are released massively into the bloodstream, as reported in certain pathological conditions associated with rhabdomyolysis or intravascular haemolysis. Once in the plasma, these haem proteins can be filtered and can accumulate in the kidney, where they become cytotoxic, particularly for the tubular epithelium, inducing acute kidney failure and chronic kidney disease. In this review, we will analyse the different pathological contexts that lead to the renal accumulation of these haem proteins, their relation to both acute and chronic loss of renal function, the pathophysiological mechanisms that cause adverse effects and the defence systems that counteract such actions. Finally, we will describe the different treatments currently used and present new therapeutic options based on the identification of new cellular and molecular targets, with particular emphasis on the numerous clinical trials that are currently ongoing., La hemoglobina y la mioglobina son hemoproteínas que juegan un papel fundamental en el organismo ya que participan en el transporte de oxígeno. Sin embargo, debido a su estructura química, estas moléculas pueden ejercer efectos deletéreos cuando se liberan al torrente sanguíneo de forma masiva, como sucede en determinadas condiciones patológicas asociadas a rabdomiólisis o hemólisis intravascular. Una vez en el plasma, estas hemoproteínas se pueden filtrar y acumular en el riñón, donde resultan citotóxicas, principalmente para el epitelio tubular, e inducen fracaso renal agudo y enfermedad renal crónica. En la presente revisión analizaremos los distintos contextos patológicos que provocan la acumulación renal de estas hemoproteínas, su relación con la pérdida de función renal a corto y largo plazo, los mecanismos fisiopatólogicos responsables de sus efectos adversos y los sistemas de defensa que contrarrestan tales acciones. Por último, describiremos los distintos tratamientos utilizados actualmente y mostraremos nuevas opciones terapéuticas basadas en la identificación de nuevas dianas celulares y moleculares, prestando especial atención a los diversos ensayos clínicos que se encuentran en marcha en la actualidad, Este trabajo ha sido financiado por una ayuda de la Sociedad Española de Nefrología a Investigación en Nefrología, Fondo de Investigaciones Sanitarias (FIS) (ISCIII/FEDER) (Programa Miguel Servet: CP10/00479 y CPII16/00017; PI13/00802 and PI14/00883) y Fundación Renal Íñigo Álvarez de Toledo (FRIAT) a JAM, Fundación Conchita Rábago a MGH, REDinREN (RD012/0021), FIS PI13/02502 y ICI14/00350 a MP, y FIS/FEDER PI14/00386 y Centro de Investigación Biomédica en Red de Diabetes y Enfermedades Metabólicas asociadas (CIBERDEM) a JE.

Published
2018

47. Improving treatment decisions using personalized risk assessment from the International IgA Nephropathy Prediction Tool

Author

Barbour, Sean J., primary, Canney, Mark, additional, Coppo, Rosanna, additional, Zhang, Hong, additional, Liu, Zhi-Hong, additional, Suzuki, Yusuke, additional, Matsuzaki, Keiichi, additional, Katafuchi, Ritsuko, additional, Induruwage, Dilshani, additional, Er, Lee, additional, Reich, Heather N., additional, Feehally, John, additional, Barratt, Jonathan, additional, Cattran, Daniel C., additional, Russo, M.L., additional, Troyanov, S., additional, Cook, H.T., additional, Roberts, I., additional, Tesar, V., additional, Maixnerova, D., additional, Lundberg, S., additional, Gesualdo, L., additional, Emma, F., additional, Fuiano, L., additional, Beltrame, G., additional, Rollino, C., additional, Amore, A., additional, Camilla, R., additional, Peruzzi, L., additional, Praga, M., additional, Feriozzi, S., additional, Polci, R., additional, Segoloni, G., additional, Colla, L., additional, Pani, A., additional, Piras, D., additional, Angioi, A., additional, Cancarini, G., additional, Ravera, S., additional, Durlik, M., additional, Moggia, E., additional, Ballarin, J., additional, Di Giulio, S., additional, Pugliese, F., additional, Serriello, I., additional, Caliskan, Y., additional, Sever, M., additional, Kilicaslan, I., additional, Locatelli, F., additional, Del Vecchio, L., additional, Wetzels, J.F.M., additional, Peters, H., additional, Berg, U., additional, Carvalho, F., additional, da Costa Ferreira, A.C., additional, Maggio, M., additional, Wiecek, A., additional, Ots-Rosenberg, M., additional, Magistroni, R., additional, Topaloglu, R., additional, Bilginer, Y., additional, D’Amico, M., additional, Stangou, M., additional, Giacchino, F., additional, Goumenos, D., additional, Kalliakmani, P., additional, Gerolymos, M., additional, Galesic, K., additional, Geddes, C., additional, Siamopoulos, K., additional, Balafa, O., additional, Galliani, M., additional, Stratta, P., additional, Quaglia, M., additional, Bergia, R., additional, Cravero, R., additional, Salvadori, M., additional, Cirami, L., additional, Fellstrom, B., additional, Kloster Smerud, H., additional, Ferrario, F., additional, Stellato, T., additional, Egido, J., additional, Martin, C., additional, Floege, J., additional, Eitner, F., additional, Lupo, A., additional, Bernich, P., additional, Menè, P., additional, Morosetti, M., additional, van Kooten, C., additional, Rabelink, T., additional, Reinders, M.E.J., additional, Boria Grinyo, J.M., additional, Cusinato, S., additional, Benozzi, L., additional, Savoldi, S., additional, Licata, C., additional, Mizerska-Wasiak, M., additional, Martina, G., additional, Messuerotti, A., additional, Dal Canton, A., additional, Esposito, C., additional, Migotto, C., additional, Triolo, G., additional, Mariano, F., additional, Pozzi, C., additional, Boero, R., additional, Bellur, S., additional, Mazzucco, G., additional, Giannakakis, C., additional, Honsova, E., additional, Sundelin, B., additional, Di Palma, A.M., additional, Gutiérrez, E., additional, Asunis, A.M., additional, Barratt, J., additional, Tardanico, R., additional, Perkowska-Ptasinska, A., additional, Arce Terroba, J., additional, Fortunato, M., additional, Pantzaki, A., additional, Ozluk, Y., additional, Steenbergen, E., additional, Soderberg, M., additional, Riispere, Z., additional, Furci, L., additional, Orhan, D., additional, Kipgen, D., additional, Casartelli, D., additional, Galesic Ljubanovic, D., additional, Gakiopoulou, H., additional, Bertoni, E., additional, Cannata Ortiz, P., additional, Karkoszka, H., additional, Groene, H.J., additional, Stoppacciaro, A., additional, Bajema, I., additional, Bruijn, J., additional, Fulladosa Oliveras, X., additional, Maldyk, J., additional, Ioachim, E., additional, Bavbek, N., additional, Cook, T., additional, Alpers, C., additional, Berthoux, F., additional, Bonsib, S., additional, D’Agati, V., additional, D’Amico, G., additional, Emancipator, S., additional, Emmal, F., additional, Fervenza, F., additional, Florquin, S., additional, Fogo, A., additional, Groene, H., additional, Haas, M., additional, Hill, P., additional, Hogg, R., additional, Hsu, S., additional, Hunley, T., additional, Hladunewich, M., additional, Jennette, C., additional, Joh, K., additional, Julian, B., additional, Kawamura, T., additional, Lai, F., additional, Leung, C., additional, Li, L., additional, Li, P., additional, Liu, Z., additional, Massat, A., additional, Mackinnon, B., additional, Mezzano, S., additional, Schena, F., additional, Tomino, Y., additional, Walker, P., additional, Wang, H., additional, Weening, J., additional, Yoshikawa N, N., additional, Zeng, C.-H., additional, Shi, S., additional, Nogi, C., additional, Suzuki, H., additional, Koike, K., additional, Hirano, K., additional, Yokoo, T., additional, Hanai, M., additional, Fukami, K., additional, Takahashi, K., additional, Yuzawa, Y., additional, Niwa, M., additional, Yasuda, Y., additional, Maruyama, S., additional, Ichikawa, D., additional, Suzuki, T., additional, Shirai, S., additional, Fukuda, A., additional, Fujimoto, S., additional, and Trimarchi, H., additional

Published
2020
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48. OP0163 2019 UPDATE OF THE JOINT EUROPEAN LEAGUE AGAINST RHEUMATISM AND EUROPEAN RENAL ASSOCIATION–EUROPEAN DIALYSIS AND TRANSPLANT ASSOCIATION (EULAR/ERA-EDTA) RECOMMENDATIONS FOR THE MANAGEMENT OF LUPUS NEPHRITIS

Author

Fanouriakis, A., primary, Kostopoulou, M., additional, Cheema, K., additional, Anders, H. J., additional, Aringer, M., additional, Bajema, I., additional, Boletis, J. N., additional, Frangou, E., additional, Houssiau, F., additional, Hollis, J., additional, Karras, A., additional, Marchiori, F., additional, Marks, S., additional, Moroni, G., additional, Mosca, M., additional, Parodis, I., additional, Praga, M., additional, Schneider, M., additional, Smolen, J. S., additional, Tesar, V., additional, Trachana, M., additional, Vollenhoven, R. V., additional, Voskuyl, A., additional, Teng, Y. K. O., additional, Van Leeuw, B., additional, Bertsias, G., additional, Jayne, D., additional, and Boumpas, D., additional

Published
2020
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50. Reproducibility of the Oxford classification of immunoglobulin A nephropathy, impact of biopsy scoring on treatment allocation and clinical relevance of disagreements: Evidence from the VALidation of IGA study cohort

Author

Bellur, S.S. Roberts, I.S.D. Troyanov, S. Royal, V. Coppo, R. Cook, H.T. Cattran, D. Terroba, Y.A. Asunis, A.M. Bajema, I. Bertoni, E. Bruijn, J.A. Cannata-Ortiz, P. Casartelli, D. Di Palma, A.M. Ferrario, F. Fortunato, M. Furci, L. Gakiopoulou, H. Ljubanovic, D.G. Giannakakis, K. Gomá, M. Gröne, H.-J. Gutiérrez, E. Haider, S.A. Honsova, E. Ioachim, E. Karkoszka, H. Kipgen, D. Maldyk, J. Mazzucco, G. Orhan, D. Ozluk, Y. Pantzaki, A. Perkowska-Ptasinska, A. Riispere, Z. Soderberg, M.P. Steenbergen, E. Stoppacciaro, A. Von Feilitzen, B.S. Tardanico, R. Tesar, V. Maixnerova, D. Lundberg, S. Gesualdo, L. Emma, F. Fuiano, L. Beltrame, G. Rollino, C. Coppo, R. Amore, A. Camilla, R. Peruzzi, L. Praga, M. Feriozzi, S. Polci, R. Segoloni, G. Colla, L. Pani, A. Angioi, A. Piras, L. Feehally, J. Barratt, J. Cancarini, G. Ravera, S. Durlik, M. Moggia, E. Ballarin, J. Di Giulio, S. Pugliese, F. Serriello, I. Caliskan, Y. Sever, M. Locatelli, F. Del Vecchio, L. Wetzels, J.F.M. Peters, H. Berg, U. Carvalho, F. da Costa Ferreira, A.C. Maggio, M. Wiecek, A. Ots-Rosenberg, M. Magistroni, R. Topaloglu, R. Bilginer, Y. D'Amico, M. Stangou, M. Giacchino, F. Goumenos, D. Kalliakmani, P. Gerolymos, M. Galesic, K. Geddes, C. Siamopoulos, K. Balafa, O. Galliani, M. Stratta, P. Quaglia, M. Bergia, R. Cravero, R. Salvadori, M. Cirami, L. Fellstrom, B. Kloster Smerud, H. Ferrario, F. Stellato, T. Egido, J. Martin, C. Floege, J. Eitner, F. Lupo, A. Bernich, P. Menè, P. Morosetti, M. van Kooten, C. Rabelink, T. Reinders, M.E.J. Grinyo, J.M. Fulladosa, X. Cusinato, S. Benozzi, L. Savoldi, S. Licata, C. Mizerska-Wasiak, M. Roszkowska-Blaim, M. Martina, G. Messuerotti, A. Dal Canton, A. Esposito, C. Migotto, C. Triolo, G. Mariano, F. Pozzi, C. Boero, R. Kilicaslan, I.

Abstract

Background: The VALidation of IGA (VALIGA) study investigated the utility of the Oxford Classification of immunoglobulin A nephropathy (IgAN) in 1147 patients from 13 European countries. Methods. Biopsies were scored by local pathologists followed by central review in Oxford. We had two distinct objectives: to assess how closely pathology findings were associated with the decision to give corticosteroid/immunosuppressive (CS/IS) treatments, and to determine the impact of differences in MEST-C scoring between central and local pathologists on the clinical value of the Oxford Classification. We tested for each lesion the associations between the type of agreement (local and central pathologists scoring absent, local present and central absent, local absent and central present, both scoring present) with the initial clinical assessment, as well as long-term outcomes in those patients who did not receive CS/IS. Results: All glomerular lesions (M, E, C and S) assessed by local pathologists were independently associated with the decision to administer CS/IS therapy, while the severity of tubulointerstitial lesions was not. Reproducibility between local and central pathologists was moderate for S (segmental sclerosis) and T (tubular atrophy/interstitial fibrosis), and poor for M (mesangial hypercellularity), E (endocapillary hypercellularity) and C (crescents). Local pathologists found statistically more of each lesion, except for the S lesion, which was more frequent with central review. Disagreements were more likely to occur when the proportion of glomeruli affected was low. The M lesion, assessed by central pathologists, correlated better with the severity of the disease at presentation and discriminated better with outcomes. In contrast, the E lesion, evaluated by local pathologists, correlated better with the clinical presentation and outcomes when compared with central review. Both C and S lesions, when discordant between local and central pathologists, had a clinical phenotype intermediate to double absent lesions (milder disease) and double present (more severe). Conclusion: We conclude that differences in the scoring of MEST-C criteria between local pathologists and a central reviewer have a significant impact on the prognostic value of the Oxford Classification. Since the decision to offer immunosuppressive therapy in this cohort was intimately associated with the MEST-C score, this study indicates a need for a more detailed guidance for pathologists in the scoring of IgAN biopsies. © 2018 The Author(s) 2018. Published by Oxford University Press on behalf of ERA-EDTA. All rights reserved.

Published
2019

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