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Background and objectives <p>Patients with late-onset neuromyelitis optica spectrum disorder (LO-NMOSD) have a worse prognosis than those with early onset NMOSD (EO-NMOSD), with limited immunotherapy data for patients with LO-NMOSD. We compared the efficacy of moderate and highly effective treatments for patients with LO-NMOSD.</p> Methods <p>We retrospectively included 695 aquaporin-4 immunoglobulin G-positive patients with EO-NMOSD (onset at age &lt; 50&#xa0;years) and LO-NMOSD (onset at age ≥ 50&#xa0;years), comparing their clinical and immunologic features. Patients with LO-NMOSD were divided into two subgroups: one using moderately effective treatments, and one using highly effective treatments, focusing on relapse rates and prognostic outcomes.</p> Results <p>In total, 208 (29.9%) patients had LO-NMOSD. The median disease duration was 4.21&#xa0;years for the LO-NMOSD group and 7.33&#xa0;years for the EO-NMOSD group. Patients with LO-NMOSD had a higher proportion of myelitis (47.6% vs. 31.2%) and higher Expanded Disability Status Scale (EDSS) scores at first attack [false discovery rate (FDR) corrected p = 0.001]. Severe attacks (EDSS ≥ 6) and motor disability at last follow-up was less common in EO-NMOSD. No difference in visual disability was observed between the two groups at last follow-up. Among patients with LO-NMOSD, 170 (81.7%) received moderately effective treatment and 38 (18.3%) received highly effective treatment. Patients using moderately effective treatments had a lower relapse-free rate than those using highly effective treatments (67.1% vs. 94.7%).</p> Discussion <p>Patients with LO-NMOSD had more severe attacks and worse prognoses than those with EO-NMOSD. Patients with LO-NMOSD may choose highly effective treatments as a first-line therapy.</p>

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Severe attacks and worse prognosis in patients with late-onset neuromyelitis optica spectrum disorder highlight the need for early highly effective treatment

  • 期刊论文

  • 发表日期：

    2025年11月19日

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  • Rui Wang,
  • Xiaofei Wang,
  • Ziyan Shi,
  • Lingyao Kong,
  • Qin Du,
  • Ying Zhang,
  • Hongxi Chen,
  • Nana Zhang,
  • Yangyang Zhang,
  • Hongyu Zhou

摘要

Background and objectives

Patients with late-onset neuromyelitis optica spectrum disorder (LO-NMOSD) have a worse prognosis than those with early onset NMOSD (EO-NMOSD), with limited immunotherapy data for patients with LO-NMOSD. We compared the efficacy of moderate and highly effective treatments for patients with LO-NMOSD.

Methods

We retrospectively included 695 aquaporin-4 immunoglobulin G-positive patients with EO-NMOSD (onset at age < 50 years) and LO-NMOSD (onset at age ≥ 50 years), comparing their clinical and immunologic features. Patients with LO-NMOSD were divided into two subgroups: one using moderately effective treatments, and one using highly effective treatments, focusing on relapse rates and prognostic outcomes.

Results

In total, 208 (29.9%) patients had LO-NMOSD. The median disease duration was 4.21 years for the LO-NMOSD group and 7.33 years for the EO-NMOSD group. Patients with LO-NMOSD had a higher proportion of myelitis (47.6% vs. 31.2%) and higher Expanded Disability Status Scale (EDSS) scores at first attack [false discovery rate (FDR) corrected p = 0.001]. Severe attacks (EDSS ≥ 6) and motor disability at last follow-up was less common in EO-NMOSD. No difference in visual disability was observed between the two groups at last follow-up. Among patients with LO-NMOSD, 170 (81.7%) received moderately effective treatment and 38 (18.3%) received highly effective treatment. Patients using moderately effective treatments had a lower relapse-free rate than those using highly effective treatments (67.1% vs. 94.7%).

Discussion

Patients with LO-NMOSD had more severe attacks and worse prognoses than those with EO-NMOSD. Patients with LO-NMOSD may choose highly effective treatments as a first-line therapy.