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The First Case Report of Preschool-Onset SS/SLE Coexisting With NMOSD of Chinese Origin

Systemic lupus erythematosus (SLE) is an autoimmune connective tissue disease (CTD), the main features of which are multiple serum autoantibodies and extensive involvement of multiple systems. The onset age of patients varies from childhood to middle age, with nearly 1/5 in childhood. Sjogren’s synd...

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Autores principales: Liu, Liqun, Tang, Li, Zhang, Lu, Li, Xingfang, Huang, Peng, Xiong, Jie, Xiao, Yangyang, Liu, Lingjuan
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Frontiers Media S.A. 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9108358/
https://www.ncbi.nlm.nih.gov/pubmed/35585974
http://dx.doi.org/10.3389/fimmu.2022.887041
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author Liu, Liqun
Tang, Li
Zhang, Lu
Li, Xingfang
Huang, Peng
Xiong, Jie
Xiao, Yangyang
Liu, Lingjuan
author_facet Liu, Liqun
Tang, Li
Zhang, Lu
Li, Xingfang
Huang, Peng
Xiong, Jie
Xiao, Yangyang
Liu, Lingjuan
author_sort Liu, Liqun
collection PubMed
description Systemic lupus erythematosus (SLE) is an autoimmune connective tissue disease (CTD), the main features of which are multiple serum autoantibodies and extensive involvement of multiple systems. The onset age of patients varies from childhood to middle age, with nearly 1/5 in childhood. Sjogren’s syndrome (SS) is also an autoimmune disease characterized by high-degree lymphocytic infiltration of exocrine glands, usually occurring in middle-aged and older women, and rarely in childhood. Neuromyelitis optica spectrum disorder (NMOSD) is an immune-mediated inflammatory demyelinating disease of the central nervous system (CNS) mainly involving the optic nerve and spinal cord. The coexistence of NMOSD and SLE and/or SS is well recognized by both neurologists and rheumatologists, but cases in children have been rarely reported. In this paper, we reported a case of a girl with onset at age 5 clinically featured by recurrent parotid gland enlargement, pancytopenia, hypocomplementemia, multiple positive serum antibodies, and cirrhosis. She was initially diagnosed with SS/SLE overlap syndrome at age 5. Four years later, the patient suffered a sudden vision loss and was examined to have positive AQP4 antibodies in serum and cerebrospinal fluid (CSF), and long segmental spinal swelling, in line with the diagnostic criteria for NMOSD. Up to now, the current patient is of the youngest onset age to develop SS/SLE coexisting with NMOSD, also with cirrhosis. It is important for clinicians to be aware of the possibility of CTDs coexisting with NMOSD in children, especially in those with positive anti-multiple autoantibodies, and to decrease the rate of missed diagnosis.
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spelling pubmed-91083582022-05-17 The First Case Report of Preschool-Onset SS/SLE Coexisting With NMOSD of Chinese Origin Liu, Liqun Tang, Li Zhang, Lu Li, Xingfang Huang, Peng Xiong, Jie Xiao, Yangyang Liu, Lingjuan Front Immunol Immunology Systemic lupus erythematosus (SLE) is an autoimmune connective tissue disease (CTD), the main features of which are multiple serum autoantibodies and extensive involvement of multiple systems. The onset age of patients varies from childhood to middle age, with nearly 1/5 in childhood. Sjogren’s syndrome (SS) is also an autoimmune disease characterized by high-degree lymphocytic infiltration of exocrine glands, usually occurring in middle-aged and older women, and rarely in childhood. Neuromyelitis optica spectrum disorder (NMOSD) is an immune-mediated inflammatory demyelinating disease of the central nervous system (CNS) mainly involving the optic nerve and spinal cord. The coexistence of NMOSD and SLE and/or SS is well recognized by both neurologists and rheumatologists, but cases in children have been rarely reported. In this paper, we reported a case of a girl with onset at age 5 clinically featured by recurrent parotid gland enlargement, pancytopenia, hypocomplementemia, multiple positive serum antibodies, and cirrhosis. She was initially diagnosed with SS/SLE overlap syndrome at age 5. Four years later, the patient suffered a sudden vision loss and was examined to have positive AQP4 antibodies in serum and cerebrospinal fluid (CSF), and long segmental spinal swelling, in line with the diagnostic criteria for NMOSD. Up to now, the current patient is of the youngest onset age to develop SS/SLE coexisting with NMOSD, also with cirrhosis. It is important for clinicians to be aware of the possibility of CTDs coexisting with NMOSD in children, especially in those with positive anti-multiple autoantibodies, and to decrease the rate of missed diagnosis. Frontiers Media S.A. 2022-05-02 /pmc/articles/PMC9108358/ /pubmed/35585974 http://dx.doi.org/10.3389/fimmu.2022.887041 Text en Copyright © 2022 Liu, Tang, Zhang, Li, Huang, Xiong, Xiao and Liu https://creativecommons.org/licenses/by/4.0/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) and the copyright owner(s) 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.
spellingShingle Immunology
Liu, Liqun
Tang, Li
Zhang, Lu
Li, Xingfang
Huang, Peng
Xiong, Jie
Xiao, Yangyang
Liu, Lingjuan
The First Case Report of Preschool-Onset SS/SLE Coexisting With NMOSD of Chinese Origin
title The First Case Report of Preschool-Onset SS/SLE Coexisting With NMOSD of Chinese Origin
title_full The First Case Report of Preschool-Onset SS/SLE Coexisting With NMOSD of Chinese Origin
title_fullStr The First Case Report of Preschool-Onset SS/SLE Coexisting With NMOSD of Chinese Origin
title_full_unstemmed The First Case Report of Preschool-Onset SS/SLE Coexisting With NMOSD of Chinese Origin
title_short The First Case Report of Preschool-Onset SS/SLE Coexisting With NMOSD of Chinese Origin
title_sort first case report of preschool-onset ss/sle coexisting with nmosd of chinese origin
topic Immunology
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9108358/
https://www.ncbi.nlm.nih.gov/pubmed/35585974
http://dx.doi.org/10.3389/fimmu.2022.887041
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