Neuromyelitis optica spectrum standstill in rheumatic systemic autoimmune diseases

Abstract

Background: Neuromyelitis optica spectrum disorders (NMOSD) are considered as an autoantibody-mediated disorder that targets aquaporin-4 (AQP4); other autoantibodies could be detected in such spectrum of diseases, including anti-nuclear antibody and antibodies to extractable nuclear antigens. Systemic autoimmune diseases such as systemic lupus erythematosus (SLE), Sjogren’s syndrome (SS), and other autoimmune diseases can overlap with NMOSD. We aimed in this review to address the current evidence describing the relation of NMOSD to systemic autoimmunity diseases, its controversy of being co-association or the same etiology, and its practical implications. Main body: The current review was done using a search for related articles or case reports on PubMed until 2019. The keywords included neuromyelitis optica spectrum disorders in combination with autoimmune disease nomenclature. We described the literature background of this controversy, to summarize the evidence of NMOSD relationship to systemic autoimmune diseases. Conclusion: NMOSD associated with systemic autoimmune diseases is more common in SLE and Sjogren’s syndrome rather than other autoimmune diseases, frequently affects females more than males; AQP4 antibodies should be tested for all NMOSD like manifestations associated with an autoimmune disorder; however, the clinical diagnosis of NMOSD regardless of the cord lesion length and the presence of positive AQP4 antibody can occur in systemic autoimmune diseases.