Phenotypic and immunological profile divergence between SLE-associated myelitis and SLE-NMOSD overlap syndrome: a retrospective case series
- 1Department of Neurology, First Affiliated Hospital of Dalian Medical University
Abstract
Background: Systemic lupus erythematosus-associated myelitis (SLE-AM) is a devastating neuroimmunological complication. Increasing evidence suggests that myelitis occurring in patients with SLE is heterogeneous and may include non-overlap SLE-AM and SLE-neuromyelitis optica spectrum disorder (NMOSD) overlap syndrome. We aimed to describe the clinical, radiological, CSF inflammatory and systemic immunological profiles of patients with these conditions and to explore their potential therapeutic implications.
Methods: We conducted a retrospective, single-center case series of six patients with SLE-associated myelitis treated at the First Affiliated Hospital of Dalian Medical University. Two patients were classified as having non-overlap SLE-AM and four as having SLE-NMOSD overlap syndrome according to clinical, radiological, and serological findings.
Results: Among the six patients, five were women and one was a man; Patients 1 and 2 had non-overlap SLE-AM, whereas Patients 3–6 had SLE-NMOSD overlap syndrome. In Patients 1 and 2, myelitis occurred concurrently with SLE onset and was accompanied by marked cerebrospinal fluid (CSF) inflammation, high anti-dsDNA antibody levels, and complement consumption. Patients 3–6 had more heterogeneous temporal courses and generally lower routine CSF inflammatory indices; serum AQP4-IgG was positive in Patients 4–6 and was not tested in Patient 3. Despite intensive immunosuppressive treatment, several patients had substantial residual neurological disability during follow-up. Notably, Patient 4 showed improvement in left-leg pain and remained clinically stable, with no recurrence over nearly 7 months of follow-up after inebilizumab treatment.
Conclusion: Non-overlap SLE-AM and SLE-NMOSD overlap syndrome may show differing clinical and CSF profiles. Detailed serological and CSF characterization may help distinguish these clinical patterns and inform more individualized therapeutic strategies.
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BibTeX
@article{zhang2026phenotypic,
title = {{Phenotypic and immunological profile divergence between SLE-associated myelitis and SLE-NMOSD overlap syndrome: a retrospective case series}},
author = {Tingting Zhang and Qingmiao Liu and Hui Cong and Yiling Huang and Hui Zhang},
journal = {Frontiers in Immunology},
year = {2026},
month = oct,
doi = {10.3389/fimmu.2026.1925485},
url = {https://www.frontiersin.org/articles/10.3389/fimmu.2026.1925485/full},
note = {CC BY 4.0},
}
RIS
TY - JOUR
TI - Phenotypic and immunological profile divergence between SLE-associated myelitis and SLE-NMOSD overlap syndrome: a retrospective case series
AU - Tingting Zhang
AU - Qingmiao Liu
AU - Hui Cong
AU - Yiling Huang
AU - Hui Zhang
PY - 2026
DA - 2026/10/20
JO - Frontiers in Immunology
DO - 10.3389/fimmu.2026.1925485
UR - https://www.frontiersin.org/articles/10.3389/fimmu.2026.1925485/full
AB - Background: Systemic lupus erythematosus-associated myelitis (SLE-AM) is a devastating neuroimmunological complication. Increasing evidence suggests that myelitis occurring in patients with SLE is heterogeneous and may include non-overlap SLE-AM and SLE-neuromyelitis optica spectrum disorder (NMOSD) overlap syndrome. We aimed to describe the clinical, radiological, CSF inflammatory and systemic immunological profiles of patients with these conditions and to explore their potential therapeutic implications. Methods: We conducted a retrospective, single-center case series of six patients with SLE-associated myelitis treated at the First Affiliated Hospital of Dalian Medical University. Two patients were classified as having non-overlap SLE-AM and four as having SLE-NMOSD overlap syndrome according to clinical, radiological, and serological findings. Results: Among the six patients, five were women and one was a man; Patients 1 and 2 had non-overlap SLE-AM, whereas Patients 3–6 had SLE-NMOSD overlap syndrome. In Patients 1 and 2, myelitis occurred concurrently with SLE onset and was accompanied by marked cerebrospinal fluid (CSF) inflammation, high anti-dsDNA antibody levels, and complement consumption. Patients 3–6 had more heterogeneous temporal courses and generally lower routine CSF inflammatory indices; serum AQP4-IgG was positive in Patients 4–6 and was not tested in Patient 3. Despite intensive immunosuppressive treatment, several patients had substantial residual neurological disability during follow-up. Notably, Patient 4 showed improvement in left-leg pain and remained clinically stable, with no recurrence over nearly 7 months of follow-up after inebilizumab treatment. Conclusion: Non-overlap SLE-AM and SLE-NMOSD overlap syndrome may show differing clinical and CSF profiles. Detailed serological and CSF characterization may help distinguish these clinical patterns and inform more individualized therapeutic strategies.
SN - 1664-3224
ER -