{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,7,8]],"date-time":"2025-07-08T06:10:08Z","timestamp":1751955008316,"version":"3.41.2"},"reference-count":49,"publisher":"Frontiers Media SA","license":[{"start":{"date-parts":[[2025,7,8]],"date-time":"2025-07-08T00:00:00Z","timestamp":1751932800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["frontiersin.org"],"crossmark-restriction":true},"short-container-title":["Front. Immunol."],"abstract":"<jats:sec><jats:title>Introduction<\/jats:title><jats:p>Neuromyelitis optica spectrum disorder (NMOSD) is an immune-mediated, 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\u201330% of adult NMOSD cases, such associations remain rarely reported in pediatric populations.<\/jats:p><\/jats:sec><jats:sec><jats:title>Case Report<\/jats:title><jats:p>We present a 14-year-old girl with NMOSD coexisting with primary Sj\u00f6gren\u2019s 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 \u22651 focus\/4 mm\u00b2), 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.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>This case highlights the rare but clinically important co-occurrence of NMOSD and pSS in children. Routine screening for autoantibodies (e.g., ANA, organ-specific 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.<\/jats:p><\/jats:sec>","DOI":"10.3389\/fimmu.2025.1559825","type":"journal-article","created":{"date-parts":[[2025,7,8]],"date-time":"2025-07-08T05:28:16Z","timestamp":1751952496000},"update-policy":"https:\/\/doi.org\/10.3389\/crossmark-policy","source":"Crossref","is-referenced-by-count":0,"title":["A Chinese girl with neuromyelitis optica spectrum disorder coexisting with primary Sjogren\u2019s syndrome: a case report and literature review"],"prefix":"10.3389","volume":"16","author":[{"given":"Guo-qin","family":"Zhu","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Rong-xuan","family":"Hu","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Yan","family":"Peng","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Yao","family":"Yao","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Guo-min","family":"Li","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1965","published-online":{"date-parts":[[2025,7,8]]},"reference":[{"key":"B1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMra1904655","article-title":"Neuromyelitis optica spectrum disorder","volume":"387","author":"Wingerchuk","year":"2022","journal-title":"N Engl J Med"},{"key":"B2","doi-asserted-by":"publisher","first-page":"103465","DOI":"10.1016\/j.autrev.2023.103465","article-title":"A comprehensive review of the advances in neuromyelitis optica spectrum disorder","volume":"22","author":"Siriratnam","year":"2023","journal-title":"Autoimmun Rev"},{"key":"B3","doi-asserted-by":"publisher","DOI":"10.1007\/s10072-023-06617-y","article-title":"Worldwide prevalence of neuromyelitis optica spectrum disorder (NMOSD) and neuromyelitis optica (NMO): a systematic review and meta-analysis","volume":"44","author":"Bagherieh","year":"2023","journal-title":"Neurol Sci"},{"key":"B4","doi-asserted-by":"publisher","DOI":"10.1038\/ncpneuro0764","article-title":"Mechanisms of disease: aquaporin-4 antibodies in neuromyelitis optica","volume":"4","author":"Jarius","year":"2008","journal-title":"Nat Clin Pract Neurol"},{"key":"B5","doi-asserted-by":"publisher","first-page":"77","DOI":"10.1016\/j.ncl.2023.06.009","article-title":"Updates in NMOSD and MOGAD diagnosis and treatment: A tale of two central nervous system autoimmune inflammatory disorders","volume":"42","author":"Cacciaguerra","year":"2024","journal-title":"Neurol Clin"},{"key":"B6","doi-asserted-by":"publisher","first-page":"107","DOI":"10.1186\/s12969-024-01045-4","article-title":"A Chinese child with both systemic lupus erythematosus coexisting with neuromyelitis optica spectrum disorder: a case report","volume":"22","author":"Hu","year":"2024","journal-title":"Pediatr Rheumatol Online J"},{"key":"B7","doi-asserted-by":"publisher","first-page":"85","DOI":"10.1038\/s41572-020-0214-9","article-title":"Neuromyelitis optica","volume":"6","author":"Jarius","year":"2020","journal-title":"Nat Rev Dis Primers"},{"key":"B8","doi-asserted-by":"publisher","DOI":"10.1007\/s00415-023-11634-0","article-title":"Update on the diagnosis and treatment of neuromyelits optica spectrum disorders (NMOSD) - revised recommendations of the Neuromyelitis Optica Study Group (NEMOS). 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