Case Report: Sjögren’s Syndrome Plus Neuromyelitis Optica Spectrum Disorder

NMOSD is a rare autoimmune demyelinating disease of the CNS. It is characterized by episodes of unilateral or bilateral optic neuritis & longitudinally extensive (three or more vertebral segments) transverse myelitis, as well as brain stem & hypothalamic lesions.

Conclusion

NMOSD is a rare, demyelinating, inflam­matory disorder that can coexist in patients with Sjögren’s syndrome. It is a separate clinical diagnosis and not a direct neurological manifestation of Sjögren’s syndrome. The presence of an anti-AQP4-IgG antibody helps in the diagnosis. Immunosuppressive therapy, such as mycophenolate, azathioprine and rituximab, prevents long-term neurological sequelae, but plasmapheresis is preferred if there is any concern for coexisting infections. IL-6 inhibitors can be used in patients with active or relapsing NMOSD.

Swosti Roka Magar, MD, is a rheumatology fellow at the University of Arkansas for Medical Sciences, Little Rock.

Gita Verma, MD, is a rheumatology fellow at the University of Arkansas for Medical Sciences, Little Rock.

Aaroop Haridas, MD, is an assistant professor in the Division of Rheumatology at the University of Arkansas for Medical Sciences, Little Rock.

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