RESUMO
BACKGROUND: Studies on the prevalence of neuromyelitis optica spectrum disorder (NMOSD) are still scarce. The aim of the current study was to determine the prevalence rate of NMOSD in Belo Horizonte, southeast Brazil, where the prevalence rate of multiple sclerosis (MS) has already been established. METHODS: For this observational study, eligible patients had to meet the 2015 International Panel for Neuromyelitis Optica Diagnosis, be seen at the study center between January 2000 and February 2019 and live in Belo Horizonte. The prevalence rate of NMOSD was estimated based on the number of MS and NMOSD patients seen at same Center during the same period, and the previously established prevalence of MS in Belo Horizonte. RESULTS: During the study period, there were 69 patients with NMOSD, 60 (87.0%) of whom were females, and 44 (63.8%) non-whites. The median age at disease onset was 36.7 (4-72) years, the mean EDSS score 4.78±2.36, and the mean ARR 0.57±0.43. Anti-aquaporin-4 immunoglobulin testing was available for 61 (88.4%) patients, of whom 41 (67.2%) had a positive result. During the same period, 280 MS patients were seen. Considering the local known prevalence rate of MS of 18.1/100,000 inhabitants, the estimated NMOSD prevalence rate in Belo Horizonte was 4.52/100,000 (95% CI 3.72-5.43) inhabitants. CONCLUSION: The prevalence rate of NMOSD in Belo Horizonte is high as compared with those found in most of the studies reported to date.
Assuntos
Esclerose Múltipla , Neuromielite Óptica , Aquaporina 4 , Brasil/epidemiologia , Feminino , Humanos , Masculino , Esclerose Múltipla/epidemiologia , Neuromielite Óptica/epidemiologia , PrevalênciaRESUMO
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.
Assuntos
Aquaporina 4/imunologia , Autoanticorpos/imunologia , Imunoglobulina G/imunologia , Neuromielite Óptica/imunologia , Síndrome de Sjogren/imunologia , Adolescente , Adulto , Idoso , Aquaporina 4/sangue , Autoanticorpos/sangue , Criança , Feminino , Humanos , Imunoglobulina G/sangue , Masculino , Pessoa de Meia-Idade , Neuromielite Óptica/complicações , Neuromielite Óptica/diagnóstico , Síndrome de Sjogren/complicações , Síndrome de Sjogren/diagnóstico , Adulto JovemRESUMO
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.