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Primary Sjögren’s syndrome: new clinical and therapeutic concepts
  1. M Ramos-Casals1,
  2. A G Tzioufas2,
  3. J Font1
  1. 1Department of Autoimmune Diseases, Hospital Clinic, Barcelona, Spain
  2. 2Department of Pathophysiology, School of Medicine, University of Athens, Greece
  1. Correspondence to:
    Dr M Ramos-Casals
    Servei de Malalties Autoimmunes, Hospital Clínic, C/Villarroel, 170, 08036-Barcelona, Spain;


Sicca features are the central clinical manifestations of Sjögren’s syndrome (SS), but recent studies have confirmed that primary SS has a systemic expression, including extraglandular manifestations. Patients with a predominantly extraepithelial expression should be managed differently from patients with predominantly periepithelial or sicca limited disease. In coming years treatment will be based on muscarinic agonists for sicca features and immunosuppressive/biological agents for extraglandular features.

  • ANA, antinuclear antibodies
  • CNS, central nervous system
  • ESR, erythrocyte sedimentation rate
  • GMN, glomerulonephritis
  • IFNα, interferon α
  • PSN, pure sensory neuropathy
  • RA, rheumatoid arthritis
  • RP, Raynaud’s phenomenon
  • SHL, sensorineural hearing loss
  • SLE, systemic lupus erythematosus
  • SS, Sjögren’s syndrome
  • SSc, systemic sclerosis
  • TIN, tubulointerstitial nephritis
  • Sjögren’s syndrome
  • extraglandular features
  • lymphoma
  • muscarinic agonists

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