CASE REPORT article
Front. Immunol.
Sec. Autoimmune and Autoinflammatory Disorders : Autoimmune Disorders
Volume 16 - 2025 | doi: 10.3389/fimmu.2025.1559825
This article is part of the Research TopicAdvances in Ocular Autoimmune DiseasesView all 5 articles
A Chinese girl with neuromyelitis optica spectrum disorder coexisting with primary Sjogren's syndrome: A case report and literature review
Provisionally accepted
- Wuxi Children’s Hospital, Wuxi, Jiangsu Province, China
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Neuromyelitis optica spectrum disorder (NMOSD) is an immunemediated, typically relapsing central nervous system demyelinating disorder characterized by optic neuritis (ON) and transverse myelitis (TM). While systemic or organ-specific autoimmune comorbidities are well-documented in 20-30% of adult NMOSD cases, such associations remain rarely reported in pediatric populations.Case Report: We present a 14-year-old girl with NMOSD coexisting with primary Sjögren's syndrome (pSS). At 11 years of age, she presented with acute right-sided headache, painful eye movements, and vision loss. Diagnostic workup confirmed AQP4-IgG seropositivity, ON, and corresponding T2-hyperintense lesions on optic nerve MRI, meeting 2023 Neuromyelitis Optica Study Group (NEMOS) revised recommendations. Acute-phase treatment included intravenous methylprednisolone and intravenous immunoglobulin, followed by maintenance therapy with oral prednisone and mycophenolate mofetil (MMF), with gradual prednisolone tapering. Right-eye vision normalized after intervention. Initial workup revealed positive antinuclear antibody (ANA), anti-Ro/SSA, anti-La/SSB, and elevated alanine aminotransferase (ALT)/aspartate aminotransferase (AST). Aged 12.5 years, labial salivary gland biopsy for persistent transaminitis showed focal lymphocytic sialadenitis (focus score ≥1 focus/4 mm²), satisfying the 2016 ACR/EULAR criteria for pSS. At 13.5 years, MMF was switched to tacrolimus due to persistent ALT/AST elevation, leading to biochemical normalization. No NMOSD relapses occurred post-initial episode. Conclusion: This case highlights the rare but clinically important co-occurrence of NMOSD and pSS in children. Routine screening for autoantibodies (e.g., ANA, organspecific antibodies) in pediatric NMOSD is warranted to detect comorbid autoimmune disorders. Targeted therapy for concurrent connective tissue diseases can optimize clinical outcomes and quality of life.
Keywords: Aquaporin 4, Children, neuromyelitis optica spectrum disorders, Optic Neuritis, Sjögren syndrome
Received: 13 Jan 2025; Accepted: 25 Jun 2025.
Copyright: © 2025 Zhu, Hu, Peng, Yao and Li. This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) or licensor are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.
* Correspondence: Guo-min Li, Wuxi Children’s Hospital, Wuxi, 214023, Jiangsu Province, China
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