Sjögren syndrome and neuromyelitis optica spectrum disorder co-exist in a common autoimmune milieu
| Autor: | Marco Aurélio Lana-Peixoto, Denise da Silva Freitas, Natália Talim, Tauana S. Tironi, Rodrigo Kleinpaul, Diogo C. Carvalho |
|---|---|
| Rok vydání: | 2014 |
| Předmět: |
Adult
Male Adolescent neuromyelitis optica Sjögren syndrome medicine.disease_cause neuromielite óptica Autoimmunity lcsh:RC321-571 Young Adult medicine Humans Spectrum disorder Young adult síndrome de Sjögren Child lcsh:Neurosciences. Biological psychiatry. Neuropsychiatry Aged Autoantibodies Sjögren?s syndrome Aquaporin 4 Neuromyelitis optica biology business.industry autoimmunity Neuromyelitis Optica Autoantibody espectro de neuromielite óptica Middle Aged medicine.disease Myasthenia gravis autoimunidade Sjogren's Syndrome Neurology AQP4-IgG Immunoglobulin G Immunology biology.protein Female Neurology (clinical) Antibody neuromyelitis optica spectrum disorders business |
| Zdroj: | Arquivos de Neuro-Psiquiatria, Vol 72, Iss 8, Pp 619-624 (2014) Arquivos de Neuro-Psiquiatria, Volume: 72, Issue: 8, Pages: 619-624, Published: JUN 2014 Arquivos de Neuro-Psiquiatria v.72 n.8 2014 Arquivos de neuro-psiquiatria Academia Brasileira de Neurologia instacron:ABNEURO |
| ISSN: | 1678-4227 |
| Popis: | 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. A relação entre síndrome de Sjögren (SS) e espectro da neuromielite óptica (ENMO) ainda não é bem compreendida. Relatamos dois pacientes com ambas as condições e revisamos 47 casos que preenchem critérios diagnósticos das duas doenças, descritos em 17 artigos extraídos da PubMed. Dos 44 pacientes cujo gênero foi informado 42 eram mulheres. A idade média ao início das manifestações neurológicas foi 36,2 anos (10-74). O anticorpo anti-AQP4 foi positivo em 32 dos 37 pacientes, em 1 foi “borderline”. Nosso Caso 1 era soronegativo para AQP4-IgG, não tinha autoanticorpos não-órgão específicos, exceto anti-SSB. O Caso 2 era soropositivo para anticorpos anti-AQP4, anti-SSA/SSB, anti-tireoglobulina, e anti-receptor da acetilcolina; apresentava hipotireoidismo, mas não havia evidêncas de miastenia gravis. Nossos casos e outros similares, previamente relatados na literatura, com resposta autoimune heterogênea à aquaporina-4 sugerem que a SS e o ENMO coexistem em meio de autoimunidade não dependente da aquaporina-4. |
| Databáze: | OpenAIRE |
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