Journal of neurology · 2025
Progression independent of relapse activity and relapse-associated worsening in seronegative NMOSD: an international cohort study.
СтатьяМногоцентровое исследованиеНазвание и аннотация: Перевод в очереди
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Siriratnam Pakeeran, Huda Saif, Van der Walt Anneke, Sanfilippo Paul, Sharmin Sifat, Foong Yi Chao, Yeh Wei Zhen, Zhu Chao, Khoury Samia J, Csepany Tunde, Willekens Barbara, Etemadifar Masoud, Ozakbas Serkan, Nytrova Petra, Altintas Ayse, Al-Asmi Abdullah, Ramo-Tello Cristina, Laureys Guy, Patti Francesco, Horakova Dana, Foschi Matteo, Boz Cavit, McCombe Pamela, Turkoglu Recai, Roos Izanne, Lechner-Scott Jeannette, Kalincik Tomas, Jokubaitis Vilija, Butzkueven Helmut, Monif Mastura
Аннотация
BACKGROUND: Previous studies have indicated that progression independent of relapse activity (PIRA) is uncommon in patients with aquaporin- 4 antibody-positive (AQP4-IgG) neuromyelitis optica spectrum disorder (NMOSD). However, the patterns of disability accumulation in seronegative NMOSD are unknown. This study aimed to evaluate the prevalence of PIRA and relapse-associated worsening (RAW) in seronegative NMOSD.
METHODS: We conducted a retrospective, multicentre cohort study of seronegative NMOSD patients from the MSBase registry. Inclusion criteria required at least three recorded expanded disability status scale (EDSS) scores: baseline, progression, and 6 months confirmed disability progression (CDP). For those with 6-month CDP, the presence or absence of relapse between baseline and progression determined the classification as RAW or PIRA, respectively. Descriptive statistics were employed to present the data.
RESULTS: This study included 93 patients, with a median follow-up duration of 5.0 years (Q1 2.8, Q3 8.4). The cohort predominantly consisted of female patients (77.4%), with a median age of onset of 33.9 years (Q1 26.1, Q3 41.2). PIRA was observed in 1 case (1.1%), whilst RAW was documented in 7 cases (7.5%).
CONCLUSION: This international cohort study confirms that CDP is uncommon in seronegative NMOSD. Given more than three quarters of CDP occur due to RAW, therapeutic strategies should focus primarily on preventing relapses.
METHODS: We conducted a retrospective, multicentre cohort study of seronegative NMOSD patients from the MSBase registry. Inclusion criteria required at least three recorded expanded disability status scale (EDSS) scores: baseline, progression, and 6 months confirmed disability progression (CDP). For those with 6-month CDP, the presence or absence of relapse between baseline and progression determined the classification as RAW or PIRA, respectively. Descriptive statistics were employed to present the data.
RESULTS: This study included 93 patients, with a median follow-up duration of 5.0 years (Q1 2.8, Q3 8.4). The cohort predominantly consisted of female patients (77.4%), with a median age of onset of 33.9 years (Q1 26.1, Q3 41.2). PIRA was observed in 1 case (1.1%), whilst RAW was documented in 7 cases (7.5%).
CONCLUSION: This international cohort study confirms that CDP is uncommon in seronegative NMOSD. Given more than three quarters of CDP occur due to RAW, therapeutic strategies should focus primarily on preventing relapses.