Advertisement

Neuroinflammation and Sjogren’s Syndrome

  • Pasquale AnnunziataEmail author
Chapter
Part of the Contemporary Clinical Neuroscience book series (CCNE)

Abstract

Sjogren’s syndrome (SS) is a chronic organ-specific autoimmune disease mainly involving exocrine glands such as lacrimal and salivary glands. SS may also involve central and peripheral nervous system with variable prevalence due to differences in diagnostic criteria and in time length to reach diagnosis. Clinical features of the central nervous involvement share similarities with multiple sclerosis (MS) and neuromyelitis optica spectrum disorders (NMOSD), two major neuroimmune disorders. SS may even coexist with MS or NMOSD. Sensory neuropathy, chronic polyradiculoneuropathy, cranial neuropathies as well as small fibre neuropathy are the main manifestations of the peripheral nervous system involvement. The pathogenic mechanism underlying neuro-SS is unclear even though molecular mimicry and epitope spreading have been hypothesized for central nervous involvement, whereas vasculitis with or without direct damage to nerve could account for peripheral nervous involvement. Treatment is mainly based on immunosuppressive therapies requiring a close cooperation between neurologists and rheumatologists to achieve the best management.

Keywords

Sjogren’s syndrome Multiple sclerosis NMOSD Polyneuropathy Autoimmunity 

References

  1. 1.
    Peri Y, Agmon-Levin N, Theodor E, Shoenfeld Y. Sjögren’s syndrome, the old and the new. Best Pract Res Clin Rheumatol. 2012;26:105–17.CrossRefGoogle Scholar
  2. 2.
    Vanderlugt CL, Miller SD. Epitope spreading in immune-mediated diseases: implications for immunotherapy. Nat Rev Immunol. 2002;2:85–95.CrossRefGoogle Scholar
  3. 3.
    Noseworthy JH, Bass BH, Vandervoort MK, et al. The prevalence of primary Sjögren’s syndrome in a multiple sclerosis population. Ann Neurol. 1989;25:95–8.CrossRefGoogle Scholar
  4. 4.
    Annunziata P, De Santi L, Di Rezze S, et al. Clinical features of Sjogren’s syndrome in patients with multiple sclerosis. Acta Neurol Scand. 2011;124:109–14.CrossRefGoogle Scholar
  5. 5.
    Mirò J, Peña-Sagredo JL, Berciano J, Insùa S, Leno C, Velarde R. Prevalence of primary Sjögren’s syndrome in patients with multiple sclerosis. Ann Neurol. 1990;27:582–4.CrossRefGoogle Scholar
  6. 6.
    Sandberg-Wollheim M, Axell T, Hansen B, et al. Primary Sjögren’s syndrome in patients with multiple sclerosis. Neurology. 1992;42:845–7.CrossRefGoogle Scholar
  7. 7.
    de Seze J, Devos D, Castelnovo G, et al. The prevalence of Sjögren syndrome in patients with primary progressive multiple sclerosis. Neurology. 2001;57:1359–63.CrossRefGoogle Scholar
  8. 8.
    Sène D, Jallouli M, Lefaucheur JP, et al. Peripheral neuropathies associated with primary Sjögren syndrome: immunologic profiles of nonataxic sensory neuropathy and sensorimotor neuropathy. Medicine (Baltimore). 2011;90:133–8.CrossRefGoogle Scholar
  9. 9.
    Delalande S, de Seze J, Fauchais AL, et al. Neurologic manifestations in primary Sjögren syndrome: a study of 82 patients. Medicine (Baltimore). 2004;83:280–91.CrossRefGoogle Scholar
  10. 10.
    Gono T, Kawaguchi Y, Katsumata Y, et al. Clinical manifestations of neurological involvement in primary Sjögren’s syndrome. Clin Rheumatol. 2011;30:485–90.CrossRefGoogle Scholar
  11. 11.
    Manthorpe R, Asmussen K, Oxholm P. Primary Sjogren’s syndrome: diagnostic criteria, clinical features, and disease activity. J Rheumatol. 1997;24(suppl):8–11.Google Scholar
  12. 12.
    Kassan SS, Moutsopoulos HM. Clinical manifestations and early diagnosis of Sjogren syndrome. Arch Intern Med. 2004;164:1275–84.CrossRefGoogle Scholar
  13. 13.
    Papiris SA, Maniati M, Constantopoulos SH, et al. Lung involvement in primary Sjogren’s syndrome is mainly related to the small airway disease. Ann Rheum Dis. 1999;58:61–4.CrossRefGoogle Scholar
  14. 14.
    Bailey RR, Swainson CP. Renal involvement in Sjogren’s syndrome. N Z Med J. 1986;99:579–80.PubMedGoogle Scholar
  15. 15.
    Skopouli FN, Dafni U, Ioannidis JP, Moutsopoulos HM. Clinical evolution, and morbidity and mortality of primary Sjogren’s syndrome. Semin Arthritis Rheum. 2000;29:296–304.CrossRefGoogle Scholar
  16. 16.
    Kassan SS. Managing dry eyes and dry mouth in Sjogren’s syndrome. Am J Manag Care. 2001;7(suppl):S444–50.PubMedGoogle Scholar
  17. 17.
    Ostuni P, Botsios C, Sfriso P, et al. Prevalence and clinical features of fibromyalgia in systemic lupus erythematosus, systemic sclerosis, and Sjogren’s syndrome. Minerva Med. 2002;93:203–9.PubMedGoogle Scholar
  18. 18.
    Tsokos M, Lazarou SA, Moutsopoulos HM. Vasculitis in primary Sjogren’s syndrome: histologic classification and clinical presentation. Am J Clin Pathol. 1987;88:26–31.CrossRefGoogle Scholar
  19. 19.
    Lafitte C. Neurologic manifestations of primary Gougerot-Sjögren syndrome. Rev Neurol. 1998;154:658–73.PubMedGoogle Scholar
  20. 20.
    Massara A, Bonazza S, Castellino G, et al. Central nervous system involvement in Sjögren’s syndrome: unusual, but not unremarkable-clinical, serological characteristics and outcomes in a large cohort of Italian patients. Rheumatology (Oxford). 2010;49:1540–9.CrossRefGoogle Scholar
  21. 21.
    Alexander EL, Arnett FC, Provost TT, Stevens MB. Sjögren’s syndrome: association of anti-Ro(SS-A) antibodies with vasculitis, hematologic abnormalities, and serologic hyperreactivity. Ann Intern Med. 1983;98:155–9.CrossRefGoogle Scholar
  22. 22.
    Alexander GE, Provost TT, Stevens MB, Alexander EL. Sjögren syndrome: central nervous system manifestations. Neurology. 1981;31:1391–6.CrossRefGoogle Scholar
  23. 23.
    Flachenecker P. Autonomic dysfunction in Guillain-Barré syndrome and multiple sclerosis. J Neurol. 2007;254:96–101.CrossRefGoogle Scholar
  24. 24.
    Tsai KY, Tsai CP, Liao N. Sjögren’s syndrome with central nervous system involvement presenting as multiple sclerosis with failure response to beta-interferon. Eur Neurol. 2001;45:59–60.CrossRefGoogle Scholar
  25. 25.
    De Santi L, Costantini MC, Annunziata P. Long time interval between multiple sclerosis onset and occurrence of primary Sjögren’s syndrome in a woman treated with interferon-beta. Acta Neurol Scand. 2005;112:194–6.CrossRefGoogle Scholar
  26. 26.
    Wingerchuk DM, Banwell B, Bennett JL, et al. International consensus diagnostic criteria for neuromyelitis optica spectrum disorders. Neurology. 2015;85:177–89.CrossRefGoogle Scholar
  27. 27.
    Pavlakis PP, Alexopoulos H, Kosmidis ML, et al. Peripheral neuropathies in Sjogren syndrome: a new reappraisal. J Neurol Neurosurg Psychiatry. 2011;82:798–802.CrossRefGoogle Scholar
  28. 28.
    Yamamoto K. Pathogenesis of Sjögren’s syndrome. Autoimmun Rev. 2003;2:13–8.CrossRefGoogle Scholar
  29. 29.
    Gemignani F, Marbini A, Pavesi G, et al. Peripheral neuropathy associated with primary Sjogren’s syndrome. J Neurol Neurosurg Psychiatry. 1994;57:983–6.CrossRefGoogle Scholar
  30. 30.
    Vitali C, Bombardieri S, Jonsson R, et al. Classification criteria for Sjögren’s syndrome: a revised version of the European criteria proposed by the American-European Consensus Group. Ann Rheum Dis. 2002;61:554–8.CrossRefGoogle Scholar
  31. 31.
    Coates T, Slavotinek JP, Rischmueller M, et al. Cerebral white matter lesions in primary Sjogren’s syndrome: a controlled study. J Rheumatol. 1999;26:1301–5.PubMedGoogle Scholar
  32. 32.
    De Andrés C, Guillem A, Rodriguez-Mahou M, et al. Frequency and significance of anti-Ro (SSA) antibodies in multiple sclerosis patients. Acta Neurol Scand. 2001;104:83–7.CrossRefGoogle Scholar
  33. 33.
    Masi G, Annunziata P. Sjogren’s syndrome and multiple sclerosis: two sides of the same coin? Autoimmun Rev. 2016;15:457–61.CrossRefGoogle Scholar
  34. 34.
    Milo R, Miller A. Revised diagnostic criteria of multiple sclerosis. Autoimmun Rev. 2014;13:518–24.CrossRefGoogle Scholar
  35. 35.
    Drori T, Chapman J. Diagnosis and classification of neuromyelitis optica (Devic’s syndrome). Autoimmun Rev. 2014;13:531–3.CrossRefGoogle Scholar
  36. 36.
    Psianou K, Panagoulias I, Papanastasiou AD, et al. Clinical and immunological parameters of Sjogren’s syndrome. Autoimmun Rev. 2018;17:1053–64.CrossRefGoogle Scholar
  37. 37.
    Bruscolini A, Sacchetti M, La Cava M, et al. Diagnosis and management of neuromyelitis optica disorders – an update. Autoimmun Rev. 2018;17:195–200.CrossRefGoogle Scholar

Copyright information

© Springer Nature Switzerland AG 2019

Authors and Affiliations

  1. 1.Department of Medicine, Surgery and Neurosciences, Clinical Neuroimmunology UnitUniversity of SienaSienaItaly

Personalised recommendations