Risk of relapse in patients with neuromyelitis optica spectrum disorder: Recognition and preventive strategy

Neuromyelitis optica spectrum disorder (NMOSD) is a rare autoimmune inflammatory disorder of the central nervous system (CNS) that is mainly associated with serum autoantibodies against aquaporin-4 (AQP4) in astrocytes. The relapsing clinical course of NMOSD, which can be blinding and disabling due to severe visual impairment, spinal cord lesions and a group of brain syndromes, suggests the importance of accurately evaluating the likelihood and severity of relapse at an early stage of the disease. To date, many risk factors have been revealed in association with relapse, and only some of them are supported by substantial evidence. Furthermore, while the clinical use of conventional immunosuppressants is mostly empirical, an increasing number of emerging therapies for monoclonal antibodies have been confirmed by several randomized placebo-controlled trials to be effective and safe for relapse prevention. In this review, we summarize the reported risk factors that may influence the frequency, symptoms, severity and prognosis of relapse in NMOSD, as well as the efficacy and safety of emerging therapies for relapse prevention. All of these results enable us to better recognize patients who are at higher risk of relapse and suggest more effective monoclonal antibody therapies for use in these patients.