Central and peripheral neurological complications of primary Sjögren's syndrome

Presse Med. 2012 Sep;41(9 Pt 2):e485-93. doi: 10.1016/j.lpm.2012.06.002. Epub 2012 Jul 26.


Primary Sjögren's syndrome (pSS) is an autoimmune inflammatory disorder characterized by lymphocytic infiltration of exocrine glands, mainly the lacrimal and salivary glands leading to a chronic sicca syndrome. However, extraglandular organ systems may frequently be involved, including both central and peripheral nervous systems. Clinically significant neurologic manifestations affect approximately 20% of patients and may be the first manifestation of the disease in at least 25% of the cases. The spectrum of pSS-related neuropathies is wide including sensory neuropathies, neuronopathies, sensory-motor neuropathies, mononeuritis multiplex related to vasculitis… Central nervous system involvement is composed by multiple sclerosis-like manifestations including acute and chronic myelopathies and by more diffuse manifestations (cognitive dysfunction, subacute aseptic meningitis, encephalopathy, psychiatric symptoms, chorea, seizures…). The diagnosis and treatment of such pSS-related manifestations must be optimized in order to avoid severe disability.

Publication types

  • Review

MeSH terms

  • Autoimmune Diseases of the Nervous System / etiology*
  • Brain Diseases / etiology
  • Central Nervous System Diseases / etiology*
  • Humans
  • Mononeuropathies / etiology
  • Motor Neurons / pathology
  • Multiple Sclerosis / etiology
  • Peripheral Nervous System Diseases / etiology*
  • Polyneuropathies / etiology
  • Sensory Receptor Cells / pathology
  • Sjogren's Syndrome / complications*
  • Spinal Cord Diseases / etiology
  • Vasculitis / etiology