Background/Purpose: Neuromyelitis optica (NMO) and neuromyelitis optica spectrum disorders (NMOSD) are immune-mediated demyelinating disorders of the central nervous system, primarily characterized by optic neuritis and longitudinal extensive transverse myelitis. Antibodies to aquaporin-4 (AQP4-IgG) are highly specific markers of NMOSD. The coexistence of NMOSD with systemic rheumatologic autoimmune disease has been well documented, especially systemic lupus erythematosus (SLE) and Sjogren syndrome (SS) [1]. Several recent studies have demonstrated that NMOSD may disproportionately affect black patients [2,3]. Our objective was to characterize African-Americans diagnosed with NMO at our academic medical center, and identify any associated systemic rheumatologic autoimmune diseases.

Methods: We conducted a retrospective chart review of adult African-American patients diagnosed with NMO at a single academic medical center between 2011 and 2017. The charts reviewed were identified using ICD codes for NMO, transverse myelitis and optic neuritis. The diagnosis of NMO was then confirmed as per the 2015 International Consensus Diagnostic Criteria. The diagnosis of SLE and SS were also confirmed based on existing diagnostic criteria.

Results: Of the 60 charts reviewed, 25 patients (41.7%) met criteria for NMO, and of those, 21 patients (84%) were African-American. Of the 21 African-American NMO patients, 89.5% (n=19) were AQP4-IgG positive, 95.2% were women, and 38.1% had an associated systemic rheumatologic autoimmune disease (SLE 62.5% and primary SS 37.5%).  In 60% of the NMO patients with SLE, the diagnosis of NMO preceded the SLE diagnosis. Conversely, in 100% of the NMO patients with SS, the diagnosis of NMO either followed, or was made simultaneous to, the SS diagnosis. Notably, 80% of the SLE patients had primarily hematologic manifestations. ANA was positive in 78.6% of the African-American patients in whom ANA testing was performed (n=14), with anti-SSA (64.3%, n=14) and anti-SSB (50%, n=11) the next most prevalent autoantibodies. Mean age of NMO onset was 46.7 years (SD 13.6). Transverse myelitis was the most common presenting manifestation (71.4%), followed by optic neuritis (61.9%).

Conclusion: Our population had a higher mean age of onset (46.7 vs 33 years) compared with other cohorts of seropositive NMO African-Americans [2]. Additionally, African-Americans with NMO at our institution had a higher female:male distribution than has been documented[3]. Not surprisingly, SLE was the most common associated systemic rheumatic disease in our cohort, however the frequency of SLE and ANA positivity in our NMO cohort was higher than has been reported (23.8% and 78.6%, respectively) [4]. Additional larger studies are needed to further explore how NMOSD in African-Americans may differ from other populations.     

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/neuromyelitis-optica-overlaps-frequently-with-systemic-rheumatic-diseases-in-african-americans-experience-at-a-large-us-academic-medical-center/