Neuromyelitis Optica Spectrum Disorders with and Without Associated Autoimmune Diseases

Objectives We aimed to determinate the frequency of this association and compare the features of neuromyelitis optica spectrum disorder (NMOSD) with and without associated autoimmune diseases (AD) in a Latin American (LATAM) population in clinical practice. Methods We retrospectively reviewed the medical records of patients with NMOSD according to the 2015 diagnostic criteria. Patients from Argentina ( n =77), Brazil ( n =46), and Venezuela ( n =17) were enrolled and classified into two groups as follows: with AD or without AD. Clinical, paraclinical (including aquaporin-4 antibodies (AQP4-ab) status), magnetic resonance imaging (MRI), and prognosis data were analyzed and compared. Kaplan-Meier (KM) and the Nelson-Aalen estimator analyses were performed to estimate both time and the cumulative hazard risk of disability reaching an EDSS≥4; and time for the first recurrence. Results Out of 140 patients, 33 (23.5%) patients had associated an AD at presentation. The most frequent associated AD was Hashimoto disease ( n =10) followed by lupus ( n =7) and Sjogren’s syndrome ( n =6). However, rituximab use (42.4% vs. 21.5%, p =0.02), female gender (82.2% vs. 100%, p =0.006), corticospinal lesions on MRI (0% vs. 12.5%, p =0.01) at onset, and positivity for antinuclear antibodies (21.2% vs. 48.4%, p =0.03) were significantly associated with NMOSD patients with AD in comparison to NMOSD patients without AD. No differences were found in other clinical and paraclinical aspects between groups. KM and Nelson-Aalen estimator analyses did not show differences between groups. Conclusion NMOSD patients associated with AD were observed in 23.5%. In addition, NMOSD patients with and without associated AD were similar in most evaluated features.