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

Arquivos de Neuro-Psiquiatria

Diogo C. Carvalho¹  Tauana S. Tironi¹  Denise S. Freitas¹  Rodrigo Kleinpaul¹  Natalia C. Talim¹  Marco A. Lana-peixoto¹ 

关键词: neuromyelitis optica spectrum disorders;    neuromyelitis optica;    Sjögren?s syndrome;    autoimmunity;    AQP4-IgG;    espectro de neuromielite óptica;    neuromielite óptica;    síndrome de Sjögren;    autoimunidade;    AQP4-IgG;   

DOI  :  10.1590/0004-282X20140097

来源: SciELO

【 摘 要 】

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.

【 授权许可】

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