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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

Abstract

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.

Keywords

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

Notes

Conflict of interest

The authors declare that they have no conflict of interest.

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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

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