The Mayo Clinic Glial Autoimmunity Study: AQP4/MOG-IgG serostatus and outcomes in 228 patients presenting with recurrent optic neuritis (S13.007)

Objective: Determine aquaporin-4 (AQP4) and myelin oligodendrocyte glycoprotein (MOG)-IgG serostatus and visual outcomes in patients with recurrent optic neuritis (rON). Background: AQP4-IgG and MOG-IgG are biomarkers of autoimmune monophasic and rON. Serology may predict visual outcomes and relapse propensity. Design/Methods: The patients were identified at Mayo Clinic 2000–2017, presented with 2 ON attacks, and had stored serum available. AQP4-IgG and MOG-IgG1 serostatus was determined by validated flow cytometry assay utilizing M1-AQP4-transfected and full length MOG-transfected live HEK293 cells. Results: Among 228 patients presenting with rON, 179 had rON alone (rON) and 49 had rON with sequential other inflammatory demyelinating attacks (rON+). Serostatus for rON cohort: 16 (9%) AQP4-IgG+, 25 (14%) MOG-IgG1+ (4 CRION), 26 (14%) multiple sclerosis (MS), 112 (63%) double negative/idiopathic (DN). Serostatus for rON+ cohort: 15 (31%) AQP4-IgG+, 4 (8%) MOG-IgG1+, 19 (39%) MS and 11 (22%) DN. The annualized relapse rates of rON was 1.2 for MOG-IgG1+, 0.67 for DN, 0.64 for AQP4-IgG+, and 0.43 for MS (MOG-IgG1+ vs MS [ p=0.009 ] , other comparisons not statistically significant or pu003e0.05). The mean visual acuity (VA) at last follow up of affected eyes was 20/400 for AQP4-IgG+, 20/100 for DN, 20/50 for MS and 20/40 for MOG-IgG1+ groups ( p p ). Only 3 of 29 MOG-IgG1+ group developed severe vision loss. Cumulative probabilities to develop severe vision loss at 5 years was 48% in AQP4-IgG+, 21% in DN, 8% in MS and MOG-IgG1+ groups ( p Conclusions: Most patients with recurrent ON lack a definitive biomarker. AQP4-IgG positive patients have worse visual outcome compared with MOG-IgG1+, MS, and double negative cases. MOG-IgG+ patients have highest relapse rate, but better visual outcome. Disclosure: Dr. Jitprapaikulsan has nothing to disclose. Dr. Chen has nothing to disclose. Dr. Fryer 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. McKeon has received research support from Medimmune, Euroimmun, Grifols and Alexion. Dr. Lennon has received royalty, license fees, or contractual rights payments from RSR, royalties from sale kits for AQP4 IgG detetction and from clinical service assays performed outside Mayo clinic. Dr. Leavitt has nothing to disclose. Dr Flanagan has nothing to disclose. Dr. Tobin has nothing to disclose. Dr. Lopez has nothing to disclose. Dr. Keegan has nothing to disclose. Dr. Lucchinetti has nothing to disclose. Dr. Kantarci has nothing to disclose. Dr. Sagen has nothing to disclose. Dr. Pittock has nothing to disclose.