ECTRIMS eLearning

Abstract: P1615

Type: LB Poster

Abstract Category: Late Breaking News

Background: Neuromyelitis Optica Spectrum Disorder (NMOSD) is a rare autoimmune disease frequently causing serious neurological disability. Pathogenic auto-antibodies (NMO-IgG) targeting the water channel, aquaporin-4 (AQP4), are frequently but not invariably detectable. Uncertainties in etiology, epidemiology, pathogenesis, and outcomes in NMOSD prompted investigation of differences between seropositive and seronegative phenotypes. The objective of this study was to explore clinical and epidemiological correlates with serostatus of patients enrolled in the Collaborative International Research in Clinical and Longitudinal Experience for NMOSD Studies (CIRCLES) registry.

Methods: Cases were recruited through geographically dispersed clinical centers in North America beginning in November 2013. NMOSD diagnosis (Wingerchuck 2006 or IPND 2015 criteria) and NMO-IgG serostatus were established at time of subject enrollment. Analyses compared age at first NMOSD episode, gender, race, time from first episode to NMOSD diagnosis, alternative diagnosis rate, number of relapses, and disability. Comparisons by NMO-IgG status were performed using descriptive statistics and chi-square or Wilcoxon rank sum tests.

Results: Of 330 cases analyzed, 256 (78%) were female. The median age at first episode was 37 years (IQR: 25,47). Median time from first episode to NMOSD diagnosis was 0.8 years (IQR 0.1,5.6) with a median of 3 relapses (IQR 2,7). NMO-IgG was detected in 257 (78%) cases; 73 (22%) were seronegative. Seronegative patients were more likely to be male (26% vs. 10%; p< 0.01); Caucasian (75 vs 61%); Asian (14 vs. 8%; p< 0.01); and younger at disease onset (median = 33 vs. 38 years; p = 0.02). Serostatus did not differentiate time from first episode to NMOSD diagnosis (median 0.8 vs. 0.7 years; p = 0.62); alternative diagnosis prior to NMOSD (57% vs. 53%; p = 0.60); or annualized relapse rate (ARR; median 0.7 vs. 0.6; p = 0.40).

Conclusion: Serostatus may reflect specific epidemiologic correlates in NMOSD. Understanding these relationships should facilitate diagnosis and shorten the interval to appropriate therapy. Such knowledge may also reveal insights into disease etiology, pathogenesis, relapse frequency & severity, and therapeutic outcomes. This study illustrates how CIRCLES represents a valuable resource for accelerating advances to define and solve NMOSD.

Disclosure: Lawrence J Cook: nothing to disclose

John Rose: nothing to disclose

Anna Marie Jolley: nothing to disclose

Renee Kuhn: nothing to disclose

Melissa Pederson: nothing to disclose

Rene Enriquez: nothing to disclose

Jeff Yearley: nothing to disclose

J. Michael Dean: nothing to disclose

May Han: nothing to disclose

Mike Feng: nothing to disclose

Toni Ganaway: nothing to disclose

Michael Levy: nothing to disclose

Maureen Mealy: nothing to disclose

Jessica Coleman: nothing to disclose

Michelle Salvatore: nothing to disclose

Jeffery Bennett Bennett serves as a consultant for Novartis Pharmaceuticals, MedImmune, Chugai Pharmaceuticals, EMD Serono, Abbvie, Genentech, TEVA, and Genzyme; receives license and royalties for a patent re Compositions and Methods for the Treatment of Neuromyelitis Optica (Aquaporumab); has stock options and serves on the scientific advisory board of Apsara Therapeutics; receives research support from Mallinckrodt Pharmaceuticals, Novartis pharmaceuticals, National Institutes of Health (R01 EY022936), and Guthy-Jackson Foundation; and serves on the editorial boards of the Multiple Sclerosis Journal, Neurology: Neuroimmunology & Neuroinflammation, and Journal of Neuro-ophthalmology.

Ruth Johnson: nothing to disclose

Elaine Hsu: nothing to disclose

Tony Traboulsee is a consultant for Novartis, Genzyme, Roche and a principal investigator on clinical trials with Biogen, Genzyme, Roche, and Chugai..

Robert Carruthers speakers bureau Biogen, Genzyme, Teva; grant support Novartis, MedImmune, Teva, Guthy-Jackson Charitable Foundation; consulting fees Novartis, Serono, Genzyme.

Alice Lee: nothing to disclose

Julia Shubert: nothing to disclose

Katrina McMullen: nothing to disclose

Ilya Kister: nothing to disclose

Zoe Rimler: nothing to disclose

Allyson Reid: nothing to disclose

Rivka Green: nothing to disclose

Megan Kenneally: nothing to disclose

Renee Rodriguez: nothing to disclose

Daniel Behne: nothing to disclose

Derek Blackway: nothing to disclose

Brian Coords: nothing to disclose

Terry Blaschke: nothing to disclose

Terry Smith: received research support from River Vision, NIH, University of South Denmark, Bell Charitable Foundation, Research to Prevent Blindness Foundation; he is an advisor to the Guthy-Jackson Charitable Foundation. He is scientific and medical director of the Graves" disease and Thyroid Foundation, and has been a paid consultant for Lipothera, River Vision, Wyeth, Merck, and Novartis. He holds patents for anti-IGF-1 receptor monoclonal therapy in autoimmune diseases.

Michael Yeaman is an associate editor for PLoS Pathogens, and study section reviewer for the U.S. National Institutes of Health; holds patents for vaccines and immunotherapies targeting drug-resistant pathogens & infections, anti-infective biologicals, and anti-infective small molecules; is Founder and 170 shareholder of NovaDigm Therapeutics; is Founder and shareholder of Metacin, Inc.; has received research support from the United States Department of Defense, and National Institutes of Health; receives license fees or royalty payments for vaccines (NovaDigm); holds U.S. copyrights and receives license fees or royalty payments for original music compositions and performances in neuroscience research (Metacin); is an advisor to the Guthy-Jackson Charitable Foundation focusing on its mission to solve NMO.

Jacinta Behne: nothing to disclose

with The Guthy-Jackson Charitable Foundation International Clinical Consortium (GJCF-ICC)