Neuromyelitis Optica Spectrum Disorders (NMOSD) and Connective Tissue Disease (CTD): an Update for the Rheumatologist

Curr Rheumatol Rep. 2021 Apr 28;23(6):33. doi: 10.1007/s11926-021-01000-2.


Purpose of review: To review the pathophysiology, presentation, and treatment of neuromyelitis optica spectrum disorder (NMOSD) and its association with systemic lupus erythematosus (SLE) and Sjogren's syndrome (SS).

Recent findings: NMOSD is an autoimmune disorder of the central nervous system that primarily targets astrocytes. Although the prevalence is unknown, the coexistence of NMOSD and SLE/SS is well-recognized. Patients with both NMOSD and SLE or SS require may require unique approaches to diagnosis and management. Coexistence of NMOSD and SLE/SS is important for the rheumatologist and neurologist to be able to recognize. For the rheumatologist, NMOSD and its neurologic symptoms represent a distinct disease process from neurologic complications of the patient's underlying connective tissue disease, and it requires distinct acute and chronic management. For the neurologist, the coexistence of SLE and SS can help to establish a diagnosis of NMOSD, or in some situations, the development of neurologic symptoms secondary to NMOSD can lead to the diagnosis of connective tissue disease.

Keywords: Connective tissue disease; Devic’s disease; NMO; NMOSD; Sjogren’s syndrome; Systemic lupus disease.

Publication types

  • Review

MeSH terms

  • Connective Tissue Diseases* / complications
  • Humans
  • Lupus Erythematosus, Systemic* / complications
  • Neuromyelitis Optica* / complications
  • Neuromyelitis Optica* / diagnosis
  • Rheumatologists
  • Sjogren's Syndrome* / complications