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Clinical aspects

 

Primary Sjögren's syndrome: central and peripheral nervous system involvements


1, 2, 3, 4, 5, 6, 7, 8, 9, 10

 

  1. Sorbonne Université, AP-HP, Hôpital Saint-Antoine, Service de Médecine Interne and Inflammation-Immunopathology-Biotherapy Department (DMU i3), Paris, France. arsene.mekinian@aphp.fr
  2. Sorbonne Université, AP-HP, Hôpital Saint-Antoine, Service de Médecine Interne and Inflammation-Immunopathology-Biotherapy Department (DMU i3), Paris, France.
  3. Sorbonne Université, AP-HP, Hôpital Saint-Antoine, Service de Médecine Interne and Inflammation-Immunopathology-Biotherapy Department (DMU i3), Paris, France.
  4. Hôpital Montfermeil, Service de Médecine Interne et Rhumatologie, Montfermeil, France.
  5. Hôpital de Melun, Service de Médecine Interne, Melun, France.
  6. Sorbonne Université, AP-HP, Hôpital Saint-Antoine, Service de Neurologie, Paris, France.
  7. Sorbonne Université, AP-HP, Hôpital Saint-Antoine, Service de Neurologie, Paris, France.
  8. Sorbonne Université, AP-HP, Hôpital Saint-Antoine, Service de Neurologie, Paris, France.
  9. Sorbonne Université, AP-HP, Hôpital Saint-Antoine, Service de Neurologie, Paris, France.
  10. Sorbonne Université, AP-HP, Hôpital Saint-Antoine, Service de Médecine Interne and Inflammation-Immunopathology-Biotherapy Department (DMU i3), Paris, France.

CER13916
2020 Vol.38, N°4 ,Suppl.126
PI 0103, PF 0109
Clinical aspects

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PMID: 33095149 [PubMed]

Received: 10/08/2020
Accepted : 14/09/2020
In Press: 22/10/2020
Published: 22/10/2020

Abstract

Primary Sjögren’s syndrome (pSS) is a common systemic autoimmune disease characterised by exocrinopathy resulting in dryness of the mouth and eyes, unexplained fatigue and diffuse pain. Neurological involvement is uncommon in pSS, involving the central nervous system in 2–5% of cases and more frequently the peripheral nervous system in 5–15% of cases. The diagnosis of pSS is to be considered when confronted with symptoms such as mouth and eye dryness, fatigue and pain, the most frequent of pSS symptoms. Objective measures of oral and eye dryness may help assert the diagnosis of pSS, as well as ACR/EULAR criteria. Differential diagnoses have to be excluded in patients exhibiting neurological symptoms, such as cryoglobulinaemic vasculitis or multiple sclerosis, before considering a neurological involvement specific to pSS. The treatment of these neurological manifestations takes into account different parameters, such as the presence of cryoglobulinaemic vasculitis, the severity of the symptoms, a rapidly progressing evolution and the failure of previous symptomatic treatments.

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