Long term Mortality in Neuromyelitis Optica Spectrum Disorder (P6.414)

Objective: To assess the long term outcomes of patients with neuromyelitis optica spectrum disorder (NMOSD). Background: Pre-aquaporin-4 IgG studies have reported a five year survival rate of 68% and median survival of 17.5 years (y). More contemporary studies report mortality rate of approximately 25% in those with disease duration of less than 10 years. African American race has been associated with greater mortality. Design/Methods: Retrospective study of patients evaluated at Mayo Clinic with onset prior to October, 2006. Results: 143 patients were identified; 22 (15%) were deceased, 4 men and 18 women; the median age of death was 58.5y (41–94). A comparison of deceased versus living patients in this study revealed the following: median follow up was 3y (range 0–8) versus 6y (0–21); median disease duration was 11y (3–28) versus 16y (9–49); median age of onset was 43y (30–83) versus 39y (21–71); median number of relapses was 6 (2–22) versus 5 (5–19); median last known Expanded Disability Status Scale (EDSS) score was 7.5y (4–8) versus 4 (0–8); 21 (95%) received immunotherapy for NMOSD versus 113 (93%); median time to initiation of treatment was 4y (0–21) in those deceased and 5y (0–39) in those living. Among the deceased patients, 16 (73%) were Caucasian, 4 (18%) African American and 2 (9%) of unknown ethnicity, whereas among living patients 72 (60%) Caucasian, 14 (12%) African American, 11 (9%) Hispanic, 8 (3%) Asian, 4 (2%) Native American, 2 (2%) Middle Eastern and 9 (7%) of unknown ethnicity. Conclusions: Patients in this study experienced a lower mortality rate than reported in previous studies. EDSS was identified as a risk factor for mortality. Disclosure: Dr. Marin Collazo has nothing to disclose. Dr. Weinshenker has received personal compensation for consulting, serving on a scientific advisory board, speaking, or other activities with Novartis, Alexion, MedImmune, Caladrius Biosciences, Brainstorm Therapeutics. Dr. Nasr has nothing to disclose. Dr. Lopez has nothing to disclose.