Neuromyelitis optica spectrum disorders Comparison according to the phenotype and serostatus

Fecha de publicación: 01/06/2016

Autores de IIS La Fe

Bonaventura Casanova Estruch

Autor
Angeles Cervello Donderis

Autor
Francesc Graus Ribas

Autor

Participantes ajenos a IIS La Fe

Sepúlveda M
Armangué T
Sola-Valls N
Arrambide G
Meca-Lallana JE
Oreja-Guevara C
Mendibe M
Alvarez de Arcaya A
Aladro Y
Olascoaga J
Jiménez-Huete A
Fernández-Fournier M
Ramió-Torrentà L
Cobo-Calvo A
Viñals M
de Andrés C
Meca-Lallana V
Calles C
Rubio MB
Ramo-Tello C
Caminero A
Munteis E
Antigüedad AR
Blanco Y
Villoslada P
Montalban X
Saiz A

Grupos

Investigación en Neuroinmunología y Esclerosis Múltiple

Leader

Bonaventura Casanova Estruch

Abstract

Objective: To (1) determine the value of the recently proposed criteria of neuromyelitis optica (NMO) spectrum disorder (NMOSD) that unify patients with NMO and those with limited forms (NMO/LF) with aquaporin-4 immunoglobulin G (AQP4-IgG) antibodies; and (2) investigate the clinical significance of the serologic status in patients with NMO. Methods: This was a retrospective, multicenter study of 181 patients fulfilling the 2006 NMO criteria (n = 127) or NMO/LF criteria with AQP4-IgG (n = 54). AQP4-IgG and myelin oligodendrocyte glycoprotein immunoglobulin G (MOG-IgG) antibodies were tested using cell-based assays. Results: Patients were mainly white (86%) and female (ratio 6.5:1) with median age at onset 39 years (range 10-77). Compared to patients with NMO and AQP4-IgG (n = 94), those with NMO/LF presentedmore often with longitudinally extensive transverse myelitis (LETM) (p<0.001), and had lower relapse rates (p = 0.015), but similar disability outcomes. Nonwhite ethnicity and optic neuritis presentation doubled the risk for developing NMO compared with white race (p = 0.008) or LETM presentation (p = 0.008). Nonwhite race (hazard ratio [HR] 4.3, 95% confidence interval [CI] 1.4-13.6) and older age at onset were associated with worse outcome (for every 10-year increase, HR 1.7, 95% CI 1.3-2.2). Patients with NMO and MOG-IgG (n = 9) had lower female: male ratio (0.8:1) and better disability outcome than AQP4-IgG-seropositive or double-seronegative patients (p<0.001). Conclusions: In patients with AQP4-IgG, the similar outcomes regardless of the clinical phenotype support the unified term NMOSD; nonwhite ethnicity and older age at onset are associated with worse outcome. Double-seronegative and AQP4-IgG-seropositive NMO have a similar clinical outcome. The better prognosis of patients with MOG-IgG and NMO suggests that phenotypic and serologic classification is useful.

Datos de la publicación

ISSN/ISSNe:: 2332-7812, 2332-7812
Tipo:: Article
Páginas:: -
DOI:: 10.1212/NXI.0000000000000225
Factor de Impacto:: 2,374 SCImago ℠
Cuartil:: Q1 SCImago ℠

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