Sjögren syndrome and neuromyelitis optica spectrum disorder co-exist in a common autoimmune milieu

The relationship between Sjögren's syndrome (SS) and neuromyelitis optica spectrum disorder (NMOSD) is not completely understood. We report two patients with both conditions and review 47 other previously reported cases meeting currently accepted diagnostic criteria, from 17 articles extracted...

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Bibliographic Details
Published in:Arquivos de neuro-psiquiatria 2014-06, Vol.72 (8), p.619-624
Main Authors: Carvalho, Diogo C, Tironi, Tauana S, Freitas, Denise S, Kleinpaul, Rodrigo, Talim, Natalia C, Lana-Peixoto, Marco A
Format: Article
Language:English
Subjects:
Adolescent
Adult
Aged
AQP4-IgG
Aquaporin 4 - blood
Aquaporin 4 - immunology
Autoantibodies - blood
Autoantibodies - immunology
autoimunidade
Child
espectro de neuromielite óptica
Female
Humans
Immunoglobulin G - blood
Immunoglobulin G - immunology
Male
Middle Aged
neuromielite óptica
Neuromyelitis Optica - complications
Neuromyelitis Optica - diagnosis
Neuromyelitis Optica - immunology
NEUROSCIENCES
PSYCHIATRY
Sjogren's Syndrome - complications
Sjogren's Syndrome - diagnosis
Sjogren's Syndrome - immunology
síndrome de Sjögren
Young Adult
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Summary:The relationship between Sjögren's syndrome (SS) and neuromyelitis optica spectrum disorder (NMOSD) is not completely understood. We report two patients with both conditions and review 47 other previously reported cases meeting currently accepted diagnostic criteria, from 17 articles extracted from PubMed. Out of 44 patients whose gender was informed, 42 were females. Mean age at onset of neurological manifestation was 36.2 years (10-74). Serum anti-AQP4-IgG was positive in 32 patients, borderline in 1, and negative in 4. Our Case 1 was seronegative for AQP4-IgG and had no non-organ-specific autoantibodies other than anti-SSB antibodies. Our Case 2 had serum anti-AQP4, anti-SSA/SSB, anti-thyreoglobulin and anti-acethylcholine-receptor antibodies, as well as clinical hypothyreoidism, but no evidence of myasthenia gravis. Our Cases and others, as previously reported in literature, with similar heterogeneous autoimmune response to aquaporin-4, suggest that SS and NMO co-exist in a common autoimmune milieu which is not dependent on aquaporin-4 autoimmunity.
ISSN:0004-282X
1678-4227
1678-4227
0004-282X
DOI:10.1590/0004-282x20140097