The Systemic Rheumatologic Disease and Neuromyelitis Optica

ABSTRACT

Neuromyelitis optica (NMO), an autoimmune inflammatory disease of the central nervous system (CNS), is related with autoantibodies for aquaporin-4, which is the most abundant water channel in CNS. The clinical syndromes of NMO, such as longitudinally extensive transverse myelitis and optic neuritis, can occur in the context of systemic rheumatologic diseases, such as systemic lupus erythematosus and Sjogren syndrome. It is debatable as to whether NMO is a feature of genetic tendency toward humoral autoimmunity or a CNS complication of systemic rheumatologic diseases. Current evidence suggests that NMO coexists with systemic rheumatologic diseases rather than a complication from them. Early immunosuppressive therapies should be considered in these patients since just one or two attacks can cause severe neurological disability.