A Chinese girl with neuromyelitis optica spectrum disorder coexisting with primary Sjogren’s syndrome: a case report and literature review
IntroductionNeuromyelitis 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 20–3...
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Frontiers Media S.A.
2025-07-01
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| author | Guo-qin Zhu Guo-qin Zhu Rong-xuan Hu Rong-xuan Hu Yan Peng Yan Peng Yao Yao Yao Yao Guo-min Li Guo-min Li |
| author_facet | Guo-qin Zhu Guo-qin Zhu Rong-xuan Hu Rong-xuan Hu Yan Peng Yan Peng Yao Yao Yao Yao Guo-min Li Guo-min Li |
| author_sort | Guo-qin Zhu |
| collection | DOAJ |
| description | IntroductionNeuromyelitis 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 20–30% of adult NMOSD cases, such associations remain rarely reported in pediatric populations.Case ReportWe 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.ConclusionThis 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. |
| format | Article |
| id | doaj-art-9cf1c53687fb4e29b15eba84a9b64cdc |
| institution | Matheson Library |
| issn | 1664-3224 |
| language | English |
| publishDate | 2025-07-01 |
| publisher | Frontiers Media S.A. |
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| series | Frontiers in Immunology |
| spelling | doaj-art-9cf1c53687fb4e29b15eba84a9b64cdc2025-07-08T05:26:43ZengFrontiers Media S.A.Frontiers in Immunology1664-32242025-07-011610.3389/fimmu.2025.15598251559825A Chinese girl with neuromyelitis optica spectrum disorder coexisting with primary Sjogren’s syndrome: a case report and literature reviewGuo-qin Zhu0Guo-qin Zhu1Rong-xuan Hu2Rong-xuan Hu3Yan Peng4Yan Peng5Yao Yao6Yao Yao7Guo-min Li8Guo-min Li9Department of Nephrology, Rheumatology and Immunology, Childrens Hospital of Jiangnan University, Wuxi, Jiangsu, ChinaDepartment of Nephrology, Rheumatology and Immunology, Wuxi Childrens Hospital, Wuxi, Jiangsu, ChinaDepartment of Nephrology, Rheumatology and Immunology, Childrens Hospital of Jiangnan University, Wuxi, Jiangsu, ChinaDepartment of Nephrology, Rheumatology and Immunology, Wuxi Childrens Hospital, Wuxi, Jiangsu, ChinaDepartment of Nephrology, Rheumatology and Immunology, Childrens Hospital of Jiangnan University, Wuxi, Jiangsu, ChinaDepartment of Nephrology, Rheumatology and Immunology, Wuxi Childrens Hospital, Wuxi, Jiangsu, ChinaDepartment of Nephrology, Rheumatology and Immunology, Childrens Hospital of Jiangnan University, Wuxi, Jiangsu, ChinaDepartment of Nephrology, Rheumatology and Immunology, Wuxi Childrens Hospital, Wuxi, Jiangsu, ChinaDepartment of Nephrology, Rheumatology and Immunology, Childrens Hospital of Jiangnan University, Wuxi, Jiangsu, ChinaDepartment of Nephrology, Rheumatology and Immunology, Wuxi Childrens Hospital, Wuxi, Jiangsu, ChinaIntroductionNeuromyelitis 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 20–30% of adult NMOSD cases, such associations remain rarely reported in pediatric populations.Case ReportWe 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.ConclusionThis 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.https://www.frontiersin.org/articles/10.3389/fimmu.2025.1559825/fullaquaporin 4childrenneuromyelitis optica spectrum disordersoptic neuritisSjögren syndrome |
| spellingShingle | Guo-qin Zhu Guo-qin Zhu Rong-xuan Hu Rong-xuan Hu Yan Peng Yan Peng Yao Yao Yao Yao Guo-min Li Guo-min Li A Chinese girl with neuromyelitis optica spectrum disorder coexisting with primary Sjogren’s syndrome: a case report and literature review Frontiers in Immunology aquaporin 4 children neuromyelitis optica spectrum disorders optic neuritis Sjögren syndrome |
| title | A Chinese girl with neuromyelitis optica spectrum disorder coexisting with primary Sjogren’s syndrome: a case report and literature review |
| title_full | A Chinese girl with neuromyelitis optica spectrum disorder coexisting with primary Sjogren’s syndrome: a case report and literature review |
| title_fullStr | A Chinese girl with neuromyelitis optica spectrum disorder coexisting with primary Sjogren’s syndrome: a case report and literature review |
| title_full_unstemmed | A Chinese girl with neuromyelitis optica spectrum disorder coexisting with primary Sjogren’s syndrome: a case report and literature review |
| title_short | A Chinese girl with neuromyelitis optica spectrum disorder coexisting with primary Sjogren’s syndrome: a case report and literature review |
| title_sort | chinese girl with neuromyelitis optica spectrum disorder coexisting with primary sjogren s syndrome a case report and literature review |
| topic | aquaporin 4 children neuromyelitis optica spectrum disorders optic neuritis Sjögren syndrome |
| url | https://www.frontiersin.org/articles/10.3389/fimmu.2025.1559825/full |
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