Rheumatology International

, Volume 35, Issue 2, pp 289–294 | Cite as

Frequent involvement of central nervous system in primary Sjögren syndrome

  • Isabel MoreiraEmail author
  • Filipa Teixeira
  • Ana Martins Silva
  • Carlos Vasconcelos
  • Fátima Farinha
  • Ernestina Santos
Short Communication


Primary Sjögren syndrome (pSS) is a systemic autoimmune disease characterized by lymphocytic infiltration of the salivary and tear glands, and autoantibody secretion, in the absence of other systemic autoimmune disorder. Among autoimmune diseases, it is a relatively common disease, but the burden of central nervous system (CNS) involvement is controversial. This retrospective study evaluates the prevalence, clinical patterns and outcomes of CNS involvement in a cohort of patients with primary Sjögren syndrome. We evaluated 93 patients with pSS diagnosed according to American-European Consensus Group criteria. Fourteen patients (15.1 %) had CNS involvement. All were women with an average age of onset of the disease of 42.1 ± 14.7 years (average ± SD) and an average age of onset of neurological involvement of 47.29 ± 16 years. Three had parkinsonian syndrome, two epilepsy, two motor and sensory deficits, two headache with brain magnetic resonance abnormalities, two neuromyelitis optica, two chronic progressive myelitis and one aseptic meningitis. Neurological involvement preceded Sjögren syndrome diagnosis in nine of the patients (64 %), and neurological outcome was good in 11 patients (78.6 %). Central nervous involvement was not as rare as expected, and the frequency was similar to the frequency of peripheral nervous system involvement. In half of the patients, this was the first symptom of the disease, emphasizing the importance of considering this diagnosis, especially in young female with neurological symptoms without other evident cause.


Sjögren syndrome Sicca syndrome Autoimmune diseases Central nervous system Neuroimmune process Keratoconjunctivitis sicca 


Conflict of interest

The authors declare that they have no conflict of interest.


  1. 1.
    Tzioufas AG, Mitsias DI, Moutsopoulos HM (2008) Sjögren’s syndrome. In: Hochberg MC, Silman AJ, Smolen JS et al (eds) Rheumatology. Elsevier, Philadelphia, pp 1348–1349Google Scholar
  2. 2.
    Mavragani CP, Moutsopoulos HM (2010) The geoepidemiology of Sjögren’s syndrome. Autoimmun Rev 9(5):A305–A310PubMedCrossRefGoogle Scholar
  3. 3.
    Delalande S, de Seze J, Fauchais AL et al (2004) Neurologic manifestations in primary Sjögren syndrome: a study of 82 patients. Medicine (Baltimore) 83(5):280–291CrossRefGoogle Scholar
  4. 4.
    Vitali C, Bombardieri S, Jonsson R et al (2002) Classification criteria for Sjögren’s syndrome: a revised version of the European criteria proposed by the American-European Consensus Group. Ann Rheum Dis 61(6):554–558PubMedCentralPubMedCrossRefGoogle Scholar
  5. 5.
    Teixeira F, Moreira I, Silva A et al (2013) Neurological involvement in primary Sjögren’s syndrome. Acta Reumatol Port 38(1):29–36PubMedGoogle Scholar
  6. 6.
    Gono T, Kawaguchi Y, Katsumata Y et al (2011) Clinical manifestations of neurological involvement in primary Sjogren’s syndrome. Clin Rheumatol 30(4):485–490PubMedCrossRefGoogle Scholar
  7. 7.
    Massara A, Bonazza S, Castellino G et al (2010) 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) 49(8):1540–1549CrossRefGoogle Scholar
  8. 8.
    Ramos-Casals M, Solans R, Rosas J et al (2008) Primary Sjögren syndrome in Spain: clinical and immunologic expression in 1010 patients. Medicine (Baltimore) 87(4):210–219CrossRefGoogle Scholar
  9. 9.
    Baldini C, Tavoni A, Merlini G et al (2005) Primary Sjogren’s syndrome: clinical and serological feature of a single centre. Reumatismo 57(4):256–261PubMedGoogle Scholar
  10. 10.
    Muñoz Mda G, Castelão WC, Saraiva FM et al (2004) Primary Sjögren’s syndrome: exocrine and non-exocrine manifestations. Rev Bras Reumatol 44(2):129–138PubMedCrossRefGoogle Scholar
  11. 11.
    García-Carrasco M, Ramos-Casals M, Rosas J et al (2002) Primary Sjogren Syndrome: clinical and immunologic disease patterns in a cohort of 400 patients. Medicine (Baltimore) 81(4):270–280CrossRefGoogle Scholar
  12. 12.
    Dalakas MC (2006) B cells in the pathophysiology of autoimmune neurological disorders: a credible therapeutic target. Pharmacol Ther 112(1):57–70PubMedCrossRefGoogle Scholar
  13. 13.
    Jarius S, Aboul-Enein F, Waters P et al (2008) Antibody to aquaporin-4 in the long-term course of neuromyelitis optica. Brain 131(Pt. 11):3072–3080PubMedCentralPubMedCrossRefGoogle Scholar
  14. 14.
    Walker RH, Spiera H, Brin MF et al (1999) Parkinsonism associated with Sjögren’s syndrome: three cases and a review of the literature. Mov Disord 14(2):262–268PubMedCrossRefGoogle Scholar
  15. 15.
    Ishida K, Uchihara T, Mizusawa H (2007) Recurrent aseptic meningitis: a new CSF complication of Sjogren’s syndrome. J Neurol 254(6):806–807PubMedCrossRefGoogle Scholar

Copyright information

© Springer-Verlag Berlin Heidelberg 2014

Authors and Affiliations

  • Isabel Moreira
    • 1
    Email author
  • Filipa Teixeira
    • 2
  • Ana Martins Silva
    • 1
  • Carlos Vasconcelos
    • 3
  • Fátima Farinha
    • 3
  • Ernestina Santos
    • 1
  1. 1.Neurology DepartmentHospital Santo António, Centro Hospitalar do PortoPortoPortugal
  2. 2.Rheumatology DepartmentUnidade Local de Saude do Alto MinhoPonte de LimaPortugal
  3. 3.Clinical Immunology UnitHospital Santo António, Centro Hospitalar do PortoPortoPortugal

Personalised recommendations