Neuromyelitis optica spectrum disorders: Comparison according to the phenotype and serostatus.
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Sepúlveda Maria, Armangué Thaís, Sola-Valls Nuria, Arrambide Georgina, Meca-Lallana José E, Oreja-Guevara Celia, Mendibe Mar, Alvarez de Arcaya Amaya, Aladro Yolanda, Casanova Bonaventura, Olascoaga Javier, Jiménez-Huete Adolfo, Fernández-Fournier Mireya, Ramió-Torrentà Lluis, Cobo-Calvo Alvaro, Viñals Montserrat, de Andrés Clara, Meca-Lallana Virginia, Cervelló Angeles, Calles Carmen, Rubio Manuel Barón, Ramo-Tello Cristina, Caminero Ana, Munteis Elvira, Antigüedad Alfredo R, Blanco Yolanda, Villoslada Pablo, Montalban Xavier, Graus Francesc, Saiz Albert
Аннотация
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 presented more 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.