Pakeeran Siriratnam, Saif Huda, Anneke Van Der Walt, Paul G Sanfilippo, Sifat Sharmin, Yi Chao Foong, Wei Z Yeh, Chao Zhu, Samia J Khoury, Tunde Csepany, Barbara Willekens, Masoud Etemadifar, Serkan Ozakbas, Petra Nytrova, Ayse Altintas, Abdullah Al-Asmi, Cristina M Ramo-Tello, Guy Laureys, Francesco Patti, Dana Horakova, Matteo Foschi, Cavit Boz, Pamela A Mccombe, Recai Turkoglu, Jeannette Lechner-Scott, Izanne Roos, Tomas Kalincik, Vilija G Jokubaitis, Helmut Butzkueven, Mastura Monif
{"title":"与 AQP4-IgG 阳性 NMOSD 患者的复发活动和复发相关恶化无关的病情进展发生率。","authors":"Pakeeran Siriratnam, Saif Huda, Anneke Van Der Walt, Paul G Sanfilippo, Sifat Sharmin, Yi Chao Foong, Wei Z Yeh, Chao Zhu, Samia J Khoury, Tunde Csepany, Barbara Willekens, Masoud Etemadifar, Serkan Ozakbas, Petra Nytrova, Ayse Altintas, Abdullah Al-Asmi, Cristina M Ramo-Tello, Guy Laureys, Francesco Patti, Dana Horakova, Matteo Foschi, Cavit Boz, Pamela A Mccombe, Recai Turkoglu, Jeannette Lechner-Scott, Izanne Roos, Tomas Kalincik, Vilija G Jokubaitis, Helmut Butzkueven, Mastura Monif","doi":"10.1212/WNL.0000000000209940","DOIUrl":null,"url":null,"abstract":"<p><strong>Objectives: </strong>In aquaporin-4 antibody-positive neuromyelitis optica spectrum disorder (AQP4-IgG NMOSD), disability accrual is mostly attributed to relapses. This study aimed to assess the prevalence of progression independent of relapse activity (PIRA) and relapse-associated worsening (RAW) in AQP4-IgG NMOSD.</p><p><strong>Methods: </strong>This was a retrospective cohort study of patients with AQP4-IgG NMOSD enrolled in the MSBase international data registry. Patients required a minimum of 3 recorded Expanded Disability Status Scale (EDSS) scores: baseline, event, and a 6-month confirmation score. Presence and absence of relapses between the baseline and event EDSS scores determined RAW and PIRA, respectively. Descriptive statistics were used to present the results.</p><p><strong>Results: </strong>A total of 181 patients followed for a median of 4.5 years (Q1 1.7, Q3 7.8) were included. Most patients were female (88.4%), and the median age at disease onset was 38.1 years. Overall, 4 patients (2.2%) developed 5 incidences of PIRA and 13 patients developed RAW (7.2%).</p><p><strong>Discussion: </strong>This multicenter study highlights that PIRA is very rare in AQP4-IgG NMOSD. Limitations of this study include the sole focus of overall EDSS to measure disability, lack of requirement for a second EDSS score to confirm baseline EDSS, and the absence of magnetic resonance imaging information for all patients.</p>","PeriodicalId":19256,"journal":{"name":"Neurology","volume":"103 12","pages":"e209940"},"PeriodicalIF":7.7000,"publicationDate":"2024-12-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":"{\"title\":\"Prevalence of Progression Independent of Relapse Activity and Relapse-Associated Worsening in Patients With AQP4-IgG-Positive NMOSD.\",\"authors\":\"Pakeeran Siriratnam, Saif Huda, Anneke Van Der Walt, Paul G Sanfilippo, Sifat Sharmin, Yi Chao Foong, Wei Z Yeh, Chao Zhu, Samia J Khoury, Tunde Csepany, Barbara Willekens, Masoud Etemadifar, Serkan Ozakbas, Petra Nytrova, Ayse Altintas, Abdullah Al-Asmi, Cristina M Ramo-Tello, Guy Laureys, Francesco Patti, Dana Horakova, Matteo Foschi, Cavit Boz, Pamela A Mccombe, Recai Turkoglu, Jeannette Lechner-Scott, Izanne Roos, Tomas Kalincik, Vilija G Jokubaitis, Helmut Butzkueven, Mastura Monif\",\"doi\":\"10.1212/WNL.0000000000209940\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"<p><strong>Objectives: </strong>In aquaporin-4 antibody-positive neuromyelitis optica spectrum disorder (AQP4-IgG NMOSD), disability accrual is mostly attributed to relapses. This study aimed to assess the prevalence of progression independent of relapse activity (PIRA) and relapse-associated worsening (RAW) in AQP4-IgG NMOSD.</p><p><strong>Methods: </strong>This was a retrospective cohort study of patients with AQP4-IgG NMOSD enrolled in the MSBase international data registry. Patients required a minimum of 3 recorded Expanded Disability Status Scale (EDSS) scores: baseline, event, and a 6-month confirmation score. Presence and absence of relapses between the baseline and event EDSS scores determined RAW and PIRA, respectively. Descriptive statistics were used to present the results.</p><p><strong>Results: </strong>A total of 181 patients followed for a median of 4.5 years (Q1 1.7, Q3 7.8) were included. Most patients were female (88.4%), and the median age at disease onset was 38.1 years. Overall, 4 patients (2.2%) developed 5 incidences of PIRA and 13 patients developed RAW (7.2%).</p><p><strong>Discussion: </strong>This multicenter study highlights that PIRA is very rare in AQP4-IgG NMOSD. 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Prevalence of Progression Independent of Relapse Activity and Relapse-Associated Worsening in Patients With AQP4-IgG-Positive NMOSD.
Objectives: In aquaporin-4 antibody-positive neuromyelitis optica spectrum disorder (AQP4-IgG NMOSD), disability accrual is mostly attributed to relapses. This study aimed to assess the prevalence of progression independent of relapse activity (PIRA) and relapse-associated worsening (RAW) in AQP4-IgG NMOSD.
Methods: This was a retrospective cohort study of patients with AQP4-IgG NMOSD enrolled in the MSBase international data registry. Patients required a minimum of 3 recorded Expanded Disability Status Scale (EDSS) scores: baseline, event, and a 6-month confirmation score. Presence and absence of relapses between the baseline and event EDSS scores determined RAW and PIRA, respectively. Descriptive statistics were used to present the results.
Results: A total of 181 patients followed for a median of 4.5 years (Q1 1.7, Q3 7.8) were included. Most patients were female (88.4%), and the median age at disease onset was 38.1 years. Overall, 4 patients (2.2%) developed 5 incidences of PIRA and 13 patients developed RAW (7.2%).
Discussion: This multicenter study highlights that PIRA is very rare in AQP4-IgG NMOSD. Limitations of this study include the sole focus of overall EDSS to measure disability, lack of requirement for a second EDSS score to confirm baseline EDSS, and the absence of magnetic resonance imaging information for all patients.
期刊介绍：
Neurology, the official journal of the American Academy of Neurology, aspires to be the premier peer-reviewed journal for clinical neurology research. Its mission is to publish exceptional peer-reviewed original research articles, editorials, and reviews to improve patient care, education, clinical research, and professionalism in neurology.
As the leading clinical neurology journal worldwide, Neurology targets physicians specializing in nervous system diseases and conditions. It aims to advance the field by presenting new basic and clinical research that influences neurological practice. The journal is a leading source of cutting-edge, peer-reviewed information for the neurology community worldwide. Editorial content includes Research, Clinical/Scientific Notes, Views, Historical Neurology, NeuroImages, Humanities, Letters, and position papers from the American Academy of Neurology. The online version is considered the definitive version, encompassing all available content.
Neurology is indexed in prestigious databases such as MEDLINE/PubMed, Embase, Scopus, Biological Abstracts®, PsycINFO®, Current Contents®, Web of Science®, CrossRef, and Google Scholar.
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