FRI0687 NEUROMYELITIS SPECTRUM DISORDERS ASSOCIATED WITH AUTOIMMUNE DISEASES: DIFFERENCES IN CLINICAL CHARACTERISTICS AND MRI FINDINGS

Abstract

Background Neuromyelitis Optica Spectrum disorders (NMOSD) is a rare autoimmune disease characterized by optic neuritis (ON) and/or longitudinal extensive transverse myelitis (LETM). It is commonly associated with other autoimmune diseases (OAD). Recent reports suggested racial differences in clinical phenotype and presentation of NMOSD. However, data on Black population is scarce. Objectives We aim to characterize, in our largely Black population, the clinical, laboratory and radiologic features of patients with NMOSD and OAD. We also aim to ascertain differences in clinical presentation between NMOSD patients with and without OAD. Methods In a retrospective analysis, patients ≥ 18 years of age with a confirmed diagnosis of NMOSD as per the International Panel for NMOSD Diagnosis Criteria, seen at 2 NYC urban hospitals from 1/2005 to 4/2017 were identified. Demographic, clinical, and laboratory data were extracted together with expanded disability status scales (EDDS) and imaging studies. Brain magnetic resonance imaging (MRI) was reviewed by a neuro-radiologist who applied the NMOSD Radiological criteria to identify typical findings of the disease. Results Forty-one patients fulfilled NMOSD criteria. 85.4% were women with a mean age of 44.7±2.03 years. 82.9% of the patients were Black and 34.1% (14/41) had an associated OAD. Systemic lupus erythematosus (SLE) was the most common OAD present prior to NMOSD diagnosis, followed by thyroid disease and Sjogren’s syndrome. Aquaporin 4 immunoglobulin G (AQP4IgG) was positive in 82.9% of the entire cohort and in 76.9% (10/13) of patients with NMOSD and OAD. Hypertension (33.3% vs. 15.3%), and cardiovascular disease (13.3% vs. 4%) were more frequent in NMOSD with OAD, compared with the NMOSD only group. On initial presentation of the NMOSD only group, visual changes (40% vs. 28.5%) and ON (38.4% vs. 20%)were predominant. In the intial presentation of NMOSD with OAD group, sensory loss (78.5% vs. 57.7%), acute myelitis (40% vs. 23.1%), and elevated C reactive protein (CRP) (20.85±11.2 vs. 3.2±1.85mg/d/L) were more freuent. Disability scores (EDDS) were 5.5 for each group. Brain MRI revealed lesions affecting corpus callosum in a marble pattern, (21.4% vs. 13.6%), the hemispheres in a spindle like pattern(33%vs 22.7%), the dorsal medulla (50% vs. 39.1%), the area postrema (38.5% vs. 27.3%) and the pons (21.4% vs. 13.4) for NMOSD with OAD and without respectively. LETM with predilection for the thoracic region was (66.7% vs 54.5%), cord edema (69.2% vs. 40.9%) and gadolinium enhancement (69.2% vs. 59.1%) for NMOSD with OAD and NMOSD only patients respectively. Conclusion AQP4IgG-positivity was observed in most of the cases in our predominantly Black NMOSD population. Over a third of the NMOSD patients had OAD. SLE was the most commonly reported. NMOSD with OAD patients tended to present with sensory loss, acute myelitis, and elevated CRP, while in NMOSD without OAD presented more with visual changes and ON. The NMOSD with OAD group had more MRI lesions involving corpus callosum, hemispheres, brainstem and LETM, compared to those with NMOSD only group.