Sjögren’s Syndrome: Interface of Immunology and Neurology

  • Robert I. Fox
  • Julius Birnbaum


Sjögren’s syndrome (SS) is characterized by dry eyes and dry mouth, due to focal lymphocytic infiltrates of the lacrimal and salivary glands. SS patients also have a spectrum of extraglandular manifestations including skin, joint, pulmonary, renal, cardiovascular, hematopoietic, neurological, and lymphoid proliferation. Indeed, the neurological manifestations are among the most challenging perplexities for the clinical rheumatologists and other specialists involved in their care. In addition, SS patients have a high incidence of fatigue, self-described cognitive impairment, and myalgias that baffle the rheumatologist because they do not correlate closely with either glandular or extraglandular manifestations. An understanding of the neuropathies in SS seems a good starting point in approaching the unmet needs of the SS patient. In particular, it is necessary to move beyond the narrowly defined “innate” and “adaptive” immune response (i.e., T-cell-directed B-cell production of autoantibodies) to the more general “danger hypothesis” that includes interactions of the immune system with the brain (hypothalamic and adrenal axis), midbrain amplification of neurokines and pain pathways, and prostaglandin pathways that play a role in vascular tone and blood pressure regulation.


Sjögren’s syndrome Neuropathies Central nervous system Danger hypothesis Dry eyes Dry mouth Sicca symptoms Brain fog 



Dr. Robert I. Fox and Dr. Julius Birnbaum would like to acknowledge Dr. Carla Martin Fox for her contribution toward this chapter.


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Copyright information

© Springer Nature Switzerland AG 2019

Authors and Affiliations

  • Robert I. Fox
    • 1
  • Julius Birnbaum
    • 2
    • 3
  1. 1.Rheumatology Clinic, Department of RheumatologyScripps Memorial Hospital and Research FoundationLa JollaUSA
  2. 2.Division of Rheumatology, Department of MedicineThe Jerome Greene Sjogren’s CenterBaltimoreUSA
  3. 3.Department of NeurologyBayview Medical CenterBaltimoreUSA

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