Peripheral neuropathies in Sjogren syndrome: a new reappraisal

J Neurol Neurosurg Psychiatry. 2011 Jul;82(7):798-802. doi: 10.1136/jnnp.2010.222109. Epub 2010 Dec 16.

Abstract

Background: The prevalence of peripheral neuropathy in patients with Sjögren syndrome remains unclear owing to conflicting results in the published series, with numbers ranging from 2% to over 60% of Sjögren syndrome patients. Whether peripheral neuropathy is a feature of the systemic or glandular disease or whether it is related to a circulating antineuronal antibody remains also uncertain.

Methods: The authors reviewed the records of patients with primary Sjögren syndrome (pSS), fulfilling the Revised European-American Classification Criteria, seen in their department from 1992 to 2009. The patients with previously recorded neuropathic features were re-examined clinically and electrophysiologically. Other causes of polyneuropathy were excluded. The authors also searched for circulating antineural antibodies using immunofluorescence and western blot and for antibodies against muscarinic and nicotinic acetylcholine receptors as potential biomarkers.

Results: 509 cases met the diagnostic criteria for pSS. Among these, 44 patients were recorded as having neuropathic symptoms. After completing the evaluation, however, only nine (1.8%) had polyneuropathy with objective clinical signs and abnormal electrophysiological findings. The neuropathy was axonal in all, in five pure sensory and in four sensorimotor. The patients with peripheral neuropathy had extraglandular manifestations such as palpable purpura and vasculitis. No evidence of antineural autoimmunity was found, and no candidate biomarkers were identified.

Conclusion: Polyneuropathy is a rare manifestation of pSS occurring in 1.8% of patients. In the majority of patients, it is a late event and frequently associated with systemic disease or risk factors for lymphoma development.

MeSH terms

  • Aged
  • Aged, 80 and over
  • Autoantibodies / analysis
  • Axons / pathology
  • Biomarkers
  • Blotting, Western
  • Brain / immunology
  • Brain / pathology
  • Electrophysiological Phenomena
  • Female
  • Fluorescent Antibody Technique
  • Ganglia, Spinal / immunology
  • Ganglia, Spinal / pathology
  • Humans
  • Male
  • Middle Aged
  • Neurologic Examination
  • Neurons / immunology
  • Peripheral Nervous System Diseases / etiology
  • Peripheral Nervous System Diseases / immunology
  • Peripheral Nervous System Diseases / pathology*
  • Receptor, Muscarinic M1 / immunology
  • Receptor, Muscarinic M3 / immunology
  • Receptors, Muscarinic / immunology
  • Receptors, Muscarinic / metabolism
  • Receptors, Nicotinic / immunology
  • Receptors, Nicotinic / metabolism
  • Retrospective Studies
  • Sensory Receptor Cells / pathology
  • Sjogren's Syndrome / complications
  • Sjogren's Syndrome / immunology
  • Sjogren's Syndrome / pathology*

Substances

  • Autoantibodies
  • Biomarkers
  • Receptor, Muscarinic M1
  • Receptor, Muscarinic M3
  • Receptors, Muscarinic
  • Receptors, Nicotinic