In the differential diagnosis of inflammatory myelopathies, several entities must be taken into account, including those associated with specific antibodies primarily targeting CNS antigens, such as anti-aquaporin-4 antibodies (AQP4), anti-MOG antibodies and the paraneoplastic myelopathies, and those associated with systemic autoimmune disorders having secondary CNS involvement (e.g., systemic lupus erythematosus, Sjogren’s syndrome, and sarcoidosis) [66]. Here, AQP4 is linked to systemic lupus erythematosus.