PDF(Pubmed)
Abstract：
BACKGROUND: We sought insights into the classification of and factors associated with relapse severity and disease stability in neuromyelitis optica spectrum disorder (NMOSD) clinical practice worldwide.
METHODS: Neurologists recruited from six countries (the USA, Germany, Italy, Brazil, South Korea, and China) participated in a 30-60 minute online survey and submitted two to four clinical records for aquaporin-4-immunoglobulin G (AQP4-IgG)-seropositive adults with NMOSD, which included patient demographics, diagnosis, maintenance treatment history, relapse occurrence, and severity. Separately, patients with NMOSD receiving maintenance therapy were interviewed over the telephone about their treatment journey, as well as perceptions of relapse severity and disease stability, and their potential influence on treatment decisions.
RESULTS: Clinical records for 1185 patients with AQP4-IgG-seropositive NMOSD were provided by 389 neurologists (July-August 2020); 33 patients were interviewed (October-November 2020). There was no clear consensus on how relapse severity was defined in clinical practice, with geographical variations in relapse classification also found. Neurologists tended to rely on clinical assessments when determining severity, viewing each relapse in isolation, whereas patients had a more subjective view based on the changes in their daily lives and comparisons with prior relapses. Similarly, there was a disconnect in the definition of disease stability: the complete absence of relapses was more important for patients than for neurologists.
CONCLUSIONS: A clear consensus on how to assess relapse severity and disease stability is needed to ensure that patients receive appropriate and timely treatment. In the future, clinical measures should be combined with patient-focused assessments.
METHODS: Neurologists recruited from six countries (the USA, Germany, Italy, Brazil, South Korea, and China) participated in a 30-60 minute online survey and submitted two to four clinical records for aquaporin-4-immunoglobulin G (AQP4-IgG)-seropositive adults with NMOSD, which included patient demographics, diagnosis, maintenance treatment history, relapse occurrence, and severity. Separately, patients with NMOSD receiving maintenance therapy were interviewed over the telephone about their treatment journey, as well as perceptions of relapse severity and disease stability, and their potential influence on treatment decisions.
RESULTS: Clinical records for 1185 patients with AQP4-IgG-seropositive NMOSD were provided by 389 neurologists (July-August 2020); 33 patients were interviewed (October-November 2020). There was no clear consensus on how relapse severity was defined in clinical practice, with geographical variations in relapse classification also found. Neurologists tended to rely on clinical assessments when determining severity, viewing each relapse in isolation, whereas patients had a more subjective view based on the changes in their daily lives and comparisons with prior relapses. Similarly, there was a disconnect in the definition of disease stability: the complete absence of relapses was more important for patients than for neurologists.
CONCLUSIONS: A clear consensus on how to assess relapse severity and disease stability is needed to ensure that patients receive appropriate and timely treatment. In the future, clinical measures should be combined with patient-focused assessments.
摘要：
背景：我们在全球范围内寻求对视神经脊髓炎谱系障碍（NMOSD）临床实践的分类和与复发严重程度和疾病稳定性相关的因素的见解。
方法：从六个国家（美国，德国,意大利,巴西,韩国,和中国）参加了30-60分钟的在线调查，并提交了2至4份水通道蛋白-4-免疫球蛋白G（AQP4-IgG）-血清阳性成人NMOSD的临床记录，包括病人的人口统计，诊断,维持治疗史,复发发生，和严重性。分别,接受维持治疗的NMOSD患者通过电话采访了他们的治疗旅程，以及对复发严重程度和疾病稳定性的看法，以及它们对治疗决策的潜在影响。
结果：389名神经科医生提供了1185例AQP4-IgG血清阳性NMOSD患者的临床记录（2020年7月至8月）；采访了33例患者（2020年10月至11月）。在临床实践中如何定义复发严重程度尚无明确共识，还发现了复发分类的地理差异。神经学家在确定严重程度时倾向于依靠临床评估，孤立地观察每一次复发，而患者根据日常生活的变化以及与既往复发的比较，有更主观的观点。同样，疾病稳定性的定义存在脱节:对于患者而言,完全没有复发比神经科医师更重要.
结论：需要就如何评估复发严重程度和疾病稳定性达成明确共识，以确保患者得到适当和及时的治疗。在未来,临床措施应与以患者为中心的评估相结合.
方法：从六个国家（美国，
结果：389名神经科医生提供了1185例AQP4-IgG血清阳性NMOSD患者的临床记录（2020年7月至8月）；采访了33例患者（2020年10月至11月）。
结论：需要就如何评估复发严重程度和疾病稳定性达成明确共识，以确保患者得到适当和及时的治疗。
