Document Detail

Sjögren syndrome and neuromyelitis optica spectrum disorder co-exist in a common autoimmune milieu.
MedLine Citation:
PMID:  25098478     Owner:  NLM     Status:  In-Data-Review    
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.
Diogo C Carvalho; Tauana S Tironi; Denise S Freitas; Rodrigo Kleinpaul; Natalia C Talim; Marco A Lana-Peixoto
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Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  Arquivos de neuro-psiquiatria     Volume:  72     ISSN:  1678-4227     ISO Abbreviation:  Arq Neuropsiquiatr     Publication Date:  2014 Jun 
Date Detail:
Created Date:  2014-08-07     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  0125444     Medline TA:  Arq Neuropsiquiatr     Country:  Brazil    
Other Details:
Languages:  eng     Pagination:  619-24     Citation Subset:  IM    
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