Scholarlink

Background <p>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.</p> Methods <p>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.</p> Results <p>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%).</p> Conclusion <p>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.</p>

错误：搜索内容不能为空，请输入英文关键词
错误：关键词超出字数限制，请精简
高级检索

Progression independent of relapse activity and relapse-associated worsening in seronegative NMOSD: an international cohort study

  • 期刊论文

  • 出版模式：

    开放获取

  • 发表日期：

    2025年4月14日

查看全文
  • Pakeeran Siriratnam,
  • Saif Huda,
  • Anneke Van der Walt,
  • Paul Sanfilippo,
  • Sifat Sharmin,
  • Yi Chao Foong,
  • Wei Zhen Yeh,
  • Chao Zhu,
  • Samia J. Khoury,
  • Tunde Csepany,
  • Barbara Willekens,
  • Masoud Etemadifar,
  • Serkan Ozakbas,
  • Petra Nytrova,
  • Ayse Altintas,
  • Abdullah Al-Asmi,
  • Cristina Ramo-Tello,
  • Guy Laureys,
  • Francesco Patti,
  • Dana Horakova,
  • Matteo Foschi,
  • Cavit Boz,
  • Pamela McCombe,
  • Recai Turkoglu,
  • Izanne Roos,
  • Jeannette Lechner-Scott,
  • Tomas Kalincik,
  • Vilija Jokubaitis,
  • Helmut Butzkueven,
  • Mastura Monif

摘要

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.