Carla Tortorella, Alessandro Cruciani, Francesco Patti, Marco Capobianco, Pietro Iaffaldano, Massimiliano Mirabella, Matilde Inglese, Luisa Pastò, Shalom Haggiag, Luca Prosperini, Serena Ruggieri, Roberta Fantozzi, Matteo Foschi, Giovanna De Luca, Marika Vianello, Giuseppe Salemi, Sara Montepietra, Diana Ferraro, Paola Cavalla, Giorgia Teresa Maniscalco, Erica Curti, Alessandra Lugaresi, Girolama Alessandra Marfia, Paola Valentino, Alessia Di Sapio, Jessica Frau, Sabrina Realmuto, Salvatore Cottone, Maria A Rocca, Elena Colombo, Paola Perini, Sebastiano Crisafulli, Maria Teresa Ferrò, Silvia Romano, Alessandra Protti, Anna Chiara Landi, Maria Gabriella Coniglio, Pietro Annovazzi, Livia Pasquali, Elisabetta Ferraro, Ilaria Pesci, Marco Peresson, Roberta Lanzillo, Rocco Totaro, Giacomo Lus, Stefania Maria Bova, Roberto Cantello, Luigi Zuliani, Daniela Cargnelutti, Luca Massacesi, Ardito Bonaventura, Maria Trotta, Alberto Gajofatto, Umberto Aguglia, Giuseppe Santuccio, Maura Chiara Danni, Alessandro Burlina, Raffaella Clerici, Paola Gazzola, Sebastiano Bucello, Matteo Pizzorno, Paolo Barone, Antonella Conte, Giuseppe Santangelo, Fabiana Marinelli, Carlo Avolio, Alessandro Leone, Francesca Rossi, Maura Frigo, Nerina Mascoli, Marco Carrozzi, Maria Buccafusca, Francesco Sica, Augusto Rini, Mario Falcini, Giuseppe Ribizzi, Maria Letizia Bartolozzi, Claudio Gasperini, Maria Trojano, Massimo Filippi, Maria Pia Amato
{"title":"aqp4 - igg阳性NMOSD和MOGAD的临床表型和治疗反应：来自意大利I-MS&RD注册的见解","authors":"Carla Tortorella, Alessandro Cruciani, Francesco Patti, Marco Capobianco, Pietro Iaffaldano, Massimiliano Mirabella, Matilde Inglese, Luisa Pastò, Shalom Haggiag, Luca Prosperini, Serena Ruggieri, Roberta Fantozzi, Matteo Foschi, Giovanna De Luca, Marika Vianello, Giuseppe Salemi, Sara Montepietra, Diana Ferraro, Paola Cavalla, Giorgia Teresa Maniscalco, Erica Curti, Alessandra Lugaresi, Girolama Alessandra Marfia, Paola Valentino, Alessia Di Sapio, Jessica Frau, Sabrina Realmuto, Salvatore Cottone, Maria A Rocca, Elena Colombo, Paola Perini, Sebastiano Crisafulli, Maria Teresa Ferrò, Silvia Romano, Alessandra Protti, Anna Chiara Landi, Maria Gabriella Coniglio, Pietro Annovazzi, Livia Pasquali, Elisabetta Ferraro, Ilaria Pesci, Marco Peresson, Roberta Lanzillo, Rocco Totaro, Giacomo Lus, Stefania Maria Bova, Roberto Cantello, Luigi Zuliani, Daniela Cargnelutti, Luca Massacesi, Ardito Bonaventura, Maria Trotta, Alberto Gajofatto, Umberto Aguglia, Giuseppe Santuccio, Maura Chiara Danni, Alessandro Burlina, Raffaella Clerici, Paola Gazzola, Sebastiano Bucello, Matteo Pizzorno, Paolo Barone, Antonella Conte, Giuseppe Santangelo, Fabiana Marinelli, Carlo Avolio, Alessandro Leone, Francesca Rossi, Maura Frigo, Nerina Mascoli, Marco Carrozzi, Maria Buccafusca, Francesco Sica, Augusto Rini, Mario Falcini, Giuseppe Ribizzi, Maria Letizia Bartolozzi, Claudio Gasperini, Maria Trojano, Massimo Filippi, Maria Pia Amato","doi":"10.1007/s00415-026-14185-2","DOIUrl":null,"url":null,"abstract":"<p><strong>Background: </strong>Neuromyelitis optica spectrum disorders (NMOSD) and myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) are rare entities distinct from multiple sclerosis. We leveraged the Italian Multiple Sclerosis and Related Disorders (I-MS&RD) Registry to characterise clinical and treatment features of AQP4-positive NMOSD and MOGAD, identify predictors of rituximab treatment response, and assess relapse outcomes across treatment classes.</p><p><strong>Methods: </strong>We identified 664 patients from 97 Italian centres: 406 with AQP4+ NMOSD and 258 with MOGAD. Demographic, clinical, and treatment characteristics were compared between groups. Time to first relapse was assessed according to treatment class, comparing monoclonal antibodies with other therapies. Predictors of relapse-free status were evaluated using multivariable logistic regression.</p><p><strong>Results: </strong>Female sex was more common in AQP4+ NMOSD than in MOGAD patients (p < 0.001), whereas paediatric onset was more common in MOGAD (p < 0.001). Patients with AQP4+ NMOSD had greater disability at diagnosis than patients with MOGAD (p < 0.001). Rituximab was the most prescribed therapy. Treatment with monoclonal antibodies was associated with longer relapse-free survival in AQP4+ NMOSD (log-rank p = 0.021), but not in MOGAD. In AQP4+ NMOSD patients treated with rituximab, male sex and myelitis at onset were independently associated with a lower likelihood of remaining relapse-free (OR 0.37, 95% CI 0.14-0.94; p = 0.038 and OR 0.36, 95% CI 0.15-0.83; p = 0.017, respectively). In contrast, among rituximab-treated patients with MOGAD, optic neuritis at onset was the only predictor of relapse-free status at follow-up (OR 0.20, 95% CI 0.04-0.90; p = 0.038).</p><p><strong>Conclusions: </strong>This nationwide, large-scale analysis, derived from the newly established I-MS&RD Registry, characterises a large Italian cohort of patients with AQP4+ NMOSD and MOGAD, identifies clinically relevant predictors of response to rituximab, and provides real-world evidence on treatment-associated relapse outcomes in these two disorders.</p>","PeriodicalId":16558,"journal":{"name":"Journal of Neurology","volume":"273 11","pages":""},"PeriodicalIF":5.4000,"publicationDate":"2026-10-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":"{\"title\":\"Clinical phenotype and treatment response in AQP4-IgG-positive NMOSD and MOGAD: insights from the Italian I-MS&RD registry.\",\"authors\":\"Carla Tortorella, Alessandro Cruciani, Francesco Patti, Marco Capobianco, Pietro Iaffaldano, Massimiliano Mirabella, Matilde Inglese, Luisa Pastò, Shalom Haggiag, Luca Prosperini, Serena Ruggieri, Roberta Fantozzi, Matteo Foschi, Giovanna De Luca, Marika Vianello, Giuseppe Salemi, Sara Montepietra, Diana Ferraro, Paola Cavalla, Giorgia Teresa Maniscalco, Erica Curti, Alessandra Lugaresi, Girolama Alessandra Marfia, Paola Valentino, Alessia Di Sapio, Jessica Frau, Sabrina Realmuto, Salvatore Cottone, Maria A Rocca, Elena Colombo, Paola Perini, Sebastiano Crisafulli, Maria Teresa Ferrò, Silvia Romano, Alessandra Protti, Anna Chiara Landi, Maria Gabriella Coniglio, Pietro Annovazzi, Livia Pasquali, Elisabetta Ferraro, Ilaria Pesci, Marco Peresson, Roberta Lanzillo, Rocco Totaro, Giacomo Lus, Stefania Maria Bova, Roberto Cantello, Luigi Zuliani, Daniela Cargnelutti, Luca Massacesi, Ardito Bonaventura, Maria Trotta, Alberto Gajofatto, Umberto Aguglia, Giuseppe Santuccio, Maura Chiara Danni, Alessandro Burlina, Raffaella Clerici, Paola Gazzola, Sebastiano Bucello, Matteo Pizzorno, Paolo Barone, Antonella Conte, Giuseppe Santangelo, Fabiana Marinelli, Carlo Avolio, Alessandro Leone, Francesca Rossi, Maura Frigo, Nerina Mascoli, Marco Carrozzi, Maria Buccafusca, Francesco Sica, Augusto Rini, Mario Falcini, Giuseppe Ribizzi, Maria Letizia Bartolozzi, Claudio Gasperini, Maria Trojano, Massimo Filippi, Maria Pia Amato\",\"doi\":\"10.1007/s00415-026-14185-2\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"<p><strong>Background: </strong>Neuromyelitis optica spectrum disorders (NMOSD) and myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) are rare entities distinct from multiple sclerosis. We leveraged the Italian Multiple Sclerosis and Related Disorders (I-MS&RD) Registry to characterise clinical and treatment features of AQP4-positive NMOSD and MOGAD, identify predictors of rituximab treatment response, and assess relapse outcomes across treatment classes.</p><p><strong>Methods: </strong>We identified 664 patients from 97 Italian centres: 406 with AQP4+ NMOSD and 258 with MOGAD. Demographic, clinical, and treatment characteristics were compared between groups. Time to first relapse was assessed according to treatment class, comparing monoclonal antibodies with other therapies. Predictors of relapse-free status were evaluated using multivariable logistic regression.</p><p><strong>Results: </strong>Female sex was more common in AQP4+ NMOSD than in MOGAD patients (p < 0.001), whereas paediatric onset was more common in MOGAD (p < 0.001). Patients with AQP4+ NMOSD had greater disability at diagnosis than patients with MOGAD (p < 0.001). Rituximab was the most prescribed therapy. Treatment with monoclonal antibodies was associated with longer relapse-free survival in AQP4+ NMOSD (log-rank p = 0.021), but not in MOGAD. In AQP4+ NMOSD patients treated with rituximab, male sex and myelitis at onset were independently associated with a lower likelihood of remaining relapse-free (OR 0.37, 95% CI 0.14-0.94; p = 0.038 and OR 0.36, 95% CI 0.15-0.83; p = 0.017, respectively). In contrast, among rituximab-treated patients with MOGAD, optic neuritis at onset was the only predictor of relapse-free status at follow-up (OR 0.20, 95% CI 0.04-0.90; p = 0.038).</p><p><strong>Conclusions: </strong>This nationwide, large-scale analysis, derived from the newly established I-MS&RD Registry, characterises a large Italian cohort of patients with AQP4+ NMOSD and MOGAD, identifies clinically relevant predictors of response to rituximab, and provides real-world evidence on treatment-associated relapse outcomes in these two disorders.</p>\",\"PeriodicalId\":16558,\"journal\":{\"name\":\"Journal of Neurology\",\"volume\":\"273 11\",\"pages\":\"\"},\"PeriodicalIF\":5.4000,\"publicationDate\":\"2026-10-16\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"Journal of Neurology\",\"FirstCategoryId\":\"3\",\"ListUrlMain\":\"https://doi.org/10.1007/s00415-026-14185-2\",\"RegionNum\":2,\"RegionCategory\":\"医学\",\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"\",\"PubModel\":\"\",\"JCR\":\"Q1\",\"JCRName\":\"CLINICAL NEUROLOGY\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"Journal of Neurology","FirstCategoryId":"3","ListUrlMain":"https://doi.org/10.1007/s00415-026-14185-2","RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q1","JCRName":"CLINICAL NEUROLOGY","Score":null,"Total":0}
引用次数: 0
摘要
背景：视神经脊髓炎谱系障碍（NMOSD）和髓鞘少突胶质细胞糖蛋白抗体相关疾病（MOGAD）是与多发性硬化症不同的罕见疾病。我们利用意大利多发性硬化症及相关疾病登记处（I-MS&RD）来描述aqp4阳性NMOSD和MOGAD的临床和治疗特征，确定利妥昔单抗治疗反应的预测因素，并评估不同治疗类别的复发结果。方法：我们从97个意大利中心确定了664例患者：406例AQP4+ NMOSD， 258例MOGAD。组间比较人口学、临床和治疗特征。根据治疗类别评估首次复发时间，并将单克隆抗体与其他治疗方法进行比较。使用多变量逻辑回归评估无复发状态的预测因子。结果：AQP4+ NMOSD患者中女性比MOGAD患者更常见（p < 0.001），而MOGAD患者中儿科发病更常见（p < 0.001）。AQP4+ NMOSD患者诊断时的残疾程度高于MOGAD患者（p < 0.001）。利妥昔单抗是最常用的治疗方法。单克隆抗体治疗与AQP4+ NMOSD患者更长的无复发生存期相关（log-rank p = 0.021），但与MOGAD患者无关。在接受利妥昔单抗治疗的AQP4+ NMOSD患者中，男性和发病时的脊髓炎与较低的无复发可能性独立相关（OR分别为0.37,95% CI 0.14-0.94; p = 0.038和OR 0.36, 95% CI 0.15-0.83; p = 0.017）。相比之下，在接受利妥昔单抗治疗的MOGAD患者中，发病时视神经炎是随访时无复发状态的唯一预测因素（OR 0.20, 95% CI 0.04-0.90; p = 0.038）。结论：这项全国性的、大规模的分析来自新建立的I-MS&RD Registry，研究了意大利AQP4+ NMOSD和MOGAD患者的大型队列，确定了对利妥昔单抗反应的临床相关预测因素，并提供了这两种疾病治疗相关复发结果的真实证据。
Clinical phenotype and treatment response in AQP4-IgG-positive NMOSD and MOGAD: insights from the Italian I-MS&RD registry.
Background: Neuromyelitis optica spectrum disorders (NMOSD) and myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) are rare entities distinct from multiple sclerosis. We leveraged the Italian Multiple Sclerosis and Related Disorders (I-MS&RD) Registry to characterise clinical and treatment features of AQP4-positive NMOSD and MOGAD, identify predictors of rituximab treatment response, and assess relapse outcomes across treatment classes.
Methods: We identified 664 patients from 97 Italian centres: 406 with AQP4+ NMOSD and 258 with MOGAD. Demographic, clinical, and treatment characteristics were compared between groups. Time to first relapse was assessed according to treatment class, comparing monoclonal antibodies with other therapies. Predictors of relapse-free status were evaluated using multivariable logistic regression.
Results: Female sex was more common in AQP4+ NMOSD than in MOGAD patients (p < 0.001), whereas paediatric onset was more common in MOGAD (p < 0.001). Patients with AQP4+ NMOSD had greater disability at diagnosis than patients with MOGAD (p < 0.001). Rituximab was the most prescribed therapy. Treatment with monoclonal antibodies was associated with longer relapse-free survival in AQP4+ NMOSD (log-rank p = 0.021), but not in MOGAD. In AQP4+ NMOSD patients treated with rituximab, male sex and myelitis at onset were independently associated with a lower likelihood of remaining relapse-free (OR 0.37, 95% CI 0.14-0.94; p = 0.038 and OR 0.36, 95% CI 0.15-0.83; p = 0.017, respectively). In contrast, among rituximab-treated patients with MOGAD, optic neuritis at onset was the only predictor of relapse-free status at follow-up (OR 0.20, 95% CI 0.04-0.90; p = 0.038).
Conclusions: This nationwide, large-scale analysis, derived from the newly established I-MS&RD Registry, characterises a large Italian cohort of patients with AQP4+ NMOSD and MOGAD, identifies clinically relevant predictors of response to rituximab, and provides real-world evidence on treatment-associated relapse outcomes in these two disorders.
期刊介绍：
The Journal of Neurology is an international peer-reviewed journal which provides a source for publishing original communications and reviews on clinical neurology covering the whole field.
In addition, Letters to the Editors serve as a forum for clinical cases and the exchange of ideas which highlight important new findings. A section on Neurological progress serves to summarise the major findings in certain fields of neurology. Commentaries on new developments in clinical neuroscience, which may be commissioned or submitted, are published as editorials.
Every neurologist interested in the current diagnosis and treatment of neurological disorders needs access to the information contained in this valuable journal.
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