Neuromyelitis optica spectrum disorders associated with systemic sclerosis: a case report and literature review

Neuromyelitis optica (NMO) is an autoimmune demyelinating disease of the central nervous system (CNS) afecting predominantly the spinal cord, brainstem, and optic nerves [1]. NMOSD may be associated with a variety of immunemediated disorders, such as systemic lupus erythematosus, Sjögren syndrome, and other organ-specifc autoimmune diseases [2], though accurate information about their prevalence is not available [3]. Systemic sclerosis (SSc) is characterized by vascular alterations, activation of the immune system, and tissue fbrosis [4]. Only a few cases of coexisting systemic sclerosis (SSc) and NMOSD are described [1, 5–9]. We report a case of an NMOSD AQP4-IgG antibodypositive patient associated with SSc and a review of the available evidence of the relationship between these autoimmune diseases