title: Sjögren syndrome and neuromyelitis optica spectrum disorder co-exist in a common autoimmune milieu.
abstract: 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.
authors.affiliation: Centro de Investigação de Esclerose Múltipla, Hospital das Clínicas, Universidade Federal de Minas Gerais, Belo Horizonte, MG, Brazil.
authors.forename: Diogo C
authors.initials: DC
authors.lastname: Carvalho
authors.affiliation: Centro de Investigação de Esclerose Múltipla, Hospital das Clínicas, Universidade Federal de Minas Gerais, Belo Horizonte, MG, Brazil.
authors.forename: Tauana S
authors.initials: TS
authors.lastname: Tironi
authors.affiliation: Centro de Investigação de Esclerose Múltipla, Hospital das Clínicas, Universidade Federal de Minas Gerais, Belo Horizonte, MG, Brazil.
authors.forename: Denise S
authors.initials: DS
authors.lastname: Freitas
authors.affiliation: Centro de Investigação de Esclerose Múltipla, Hospital das Clínicas, Universidade Federal de Minas Gerais, Belo Horizonte, MG, Brazil.
authors.forename: Rodrigo
authors.initials: R
authors.lastname: Kleinpaul
authors.affiliation: Centro de Investigação de Esclerose Múltipla, Hospital das Clínicas, Universidade Federal de Minas Gerais, Belo Horizonte, MG, Brazil.
authors.forename: Natalia C
authors.initials: NC
authors.lastname: Talim
authors.affiliation: Centro de Investigação de Esclerose Múltipla, Hospital das Clínicas, Universidade Federal de Minas Gerais, Belo Horizonte, MG, Brazil.
authors.forename: Marco A
authors.initials: MA
authors.lastname: Lana-Peixoto
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D051401:Aquaporin 4 / Q000097:blood / Q000276:immunology*; D001323:Autoantibodies / Q000097:blood / Q000276:immunology*; D002648:Child; D005260:Female; D006801:Humans; D007074:Immunoglobulin G / Q000097:blood / Q000276:immunology*; D008297:Male; D008875:Middle Aged; D009471:Neuromyelitis Optica / Q000150:complications / Q000175:diagnosis / Q000276:immunology*; D012859:Sjogren's Syndrome / Q000150:complications / Q000175:diagnosis / Q000276:immunology*; D055815:Young Adult
chemical_list: D051401:Aquaporin 4; D001323:Autoantibodies; D007074:Immunoglobulin G