Systemic Autoantibodies in Neuromyelitis Optica: Is There Any Impact on Clinical Outcome? (P07.069)

Abstract

Objective: To characterize the SS/SLE autoantibody profile and its impact on clinical features and disease severity of NMO/NMOSD patients. Background Neuromyelitis optica (NMO) and its spectrum disorders (NMOSD) have been associated with systemic inflammatory disorders, in particular Sjogren9s syndrome (SS) and systemic lupus erythromatosus (SLE). It is presently unknown if concomitant SS/SLE or systemic autoantibody seropositivity confers a worse prognosis in NMO/NMOSD patients. Design/Methods: We retrospectively reviewed patients who fulfilled diagnosis for NMO/NMOSD and underwent SS/SLE autoantibody evaluations, and compared autoantibody seropositivity to clinical characteristics and disease severity. Results: Twenty-nine patients (25 female, 4 male; 18 NMO, 11 NMOSD) were analysed. Mean disease duration was 84 months (range: 9 - 233 months). Overall systemic autoantibody seropositivity was high: anti-nuclear antibodies (48.3% [14/29]), anti-double stranded DNA (anti-dsDNA) (22.2% [6/27]) and anti-Ro and/or anti-La (21.4% [6/28]). Of those who tested for NMO-IgG, 83.3% (20/24) were positive; 55% (11/20) of these patients also had SS and/or SLE autoantibodies. All eleven patients with concomitant NMO-IgG and SS/SLE autoantibody seropositivity were female. Mean annualized relapse rate was not significantly different between systemic autoantibody-positive and autoantibody-negative patients (0.51 vs 0.91 relapse/year, p =0.089). Median Expanded Disability Status Scale (EDSS) score was similar in NMO/NMOSD patients with or without concomitant SS/SLE autoantibody positivity (3.0 vs 3.0, p=0.579). Similarly, there were no differences in ambulation (p=0.238), visual (p=0.702) and bladder/bowel dysfunction (p=0.192) scores in NMO/NMOSD patients with or without concomitant SS/SLE autoantibody positivity. Conclusions: Concomitant systemic inflammatory diseases such as SS and SLE and systemic autoantibody positivity in NMO/NMOSD are common and not mutually exclusive. Concomitant systemic autoantibody seropositivity does not translate to a worse clinical outcome in NMO/NMOSD patients, hence neurological prognosis appears to be exclusively mediated by NMO/NMOSD. Disclosure: Dr. See has nothing to disclose. Dr. Peh has nothing to disclose. Dr. Tan has received personal compensation for activities with Biogen Idec and Merck Serono as a consultant and/or speaker.