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.

You Might Also Like

• Fellows Forum Case Report: Neuromyelitis Optica
• Diagnostic Criteria Released for Neuromyelitis Optica Spectrum Disorders
• Fibromyalgia & Bipolar Spectrum Disorder: A Shared Pathophysiology May Lead to Better Drug Targets

Explore This Issue

June 2019

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.

References

1. Wingerchuk DM, Banwell B, Bennett JL, et al. International consensus diagnostic criteria for neuromyelitis optica spectrum disorders. Neurology. 2015 Jul 14;85(2):177–189.
2. Jarius S, Wildemann B. The history of neuromyelitis optica. J Neuroinflammation. 2013 Jan 15;10:8.
3. Ratelade J, Verkman AS. Neuromyelitis optica: Aquaporin-4 based patho­genesis mechanisms and new therapies. Int J Biochem Cell Biol. 2012 Sep;44(9):1519–1530.
4. Weinshenker BG, Wingerchuk DM, Vukusic S, et al. Neuromyelitis optica IgG predicts relapse after longitudinally extensive transverse myelitis. Ann Neurol. 2006 Mar;59(3):566–569.
5. Pandit L. Neuromyelitis optica spectrum disorders: An update. Ann Indian Acad Neurol. 2015 Sep;18(Suppl 1):S11–S15.
6. Birnbaum J, Atri NM, Baer AN, et al. Relationship between neuromyelitis optica spectrum disorder and Sjögren’s syndrome: Central nervous system extraglandular disease or unrelated, co-occurring autoimmunity? Arthritis Care Res (Hoboken). 2017 Jul;69(7):1069–1075.
7. Zekeridou A, Lennon VA. Aquaporin-4 autoimmunity. Neurol Neuroimmunol Neuroinflamm. 2015 May 21;2(4):e110.
8. Kitley J, Waters P, Woodhall M, et al. Neuromyelitis optica spectrum disorders with aquaporin-4 and myelin-oligodendrocyte glycoprotein antibodies: A comparative study. JAMA Neurol. 2014 Mar;71(3):276–283.
9. Jarius S, Paul F, Franciotta D, et al. Cerebrospinal fluid findings in aquaporin-4 antibody positive neuromyelitis optica: Results from 211 lumbar punctures. J Neurol Sci. 2011 Jul 15;306(1–2):82–90.
10. Kim HJ, Paul F, Lana-Peixoto MA, et al. MRI characteristics of neuromyelitis optica spectrum disorder: An international update. Neurology. 2015 Mar 17;84(11): 1165–1173.
11. Kimbrough DJ, Fujihara K, Jacob A, et al. Treatment of neuromyelitis optica: Review and recommendations. Mult Scler Relat Disord. 2012 Oct;1(4):180–187.
12. Kessler RA, Mealy MA, Levy M. Treatment of neuromyelitis optica spectrum disorder: Acute, preventive and symptomatic. Curr Treat Options Neurol. 2016 Jan;18(1):2.
13. Sherman E, Han MH. Acute and chronic management of neuromyelitis optica spectrum disorder. Curr Treat Options Neurol. 2015 Nov;17(11):48.
14. Içöz S, Tüzün E, Kürtüncü M, et al. Enhanced IL-6 production in aquaporin-4 antibody positive neuromyelitis optica patients. Int J Neurosci. 2010 Jan;120(1):71–75.
15. Araki M, Matsuoka T, Miyamoto K, et al. Efficacy of the anti-IL-6 receptor antibody tocilizumab in neuromyelitis optica: A pilot study. Neurology. 2014 Apr 15;82(15):1302–1306.
16. Ringelstein M, Ayzenberg I, Harmel J, et al. Long-term therapy with interleukin 6 receptor blockade in highly active neuromyelitis optica spectrum disorder. JAMA Neurol. 2015 Jul;72(7):756–763.