Ear, Nose, and Throat Manifestations of Sjögren’s Syndrome

  • Rohan R. Walvekar
  • Francis Marchal


Sjögren’s syndrome (SjS) is an autoimmune disease that primarily affects the secretory function of lacrimal and salivary glands [1]. Dry mouth (xerostomia) is the most common presenting complaint [2], but SjS can affect multiple organs including the kidneys, lungs, liver, blood vessels, and lymph nodes, resulting in a myriad of clinical manifestations [3]. This chapter provides an overview of the key manifestations of SjS associated with the ear, nose, and throat region. We review the otologic, sinus and nasal, laryngopharyngeal, and tracheal manifestations of this disease.


Hearing Loss Sensorineural Hearing Loss Vocal Fold Laryngeal Lesion Vocal Nodule 



The authors would like to acknowledge the Louisiana State University (LSU) Department of Otolaryngology Head & Neck Surgery, New Orleans, Louisiana, USA for its support. In particularly, we wish to thank Virginia Plauche (LSU School of Medicine) and Neelima Tammareddi (PGY-2 Otolaryngology Resident), Esther Phelps and Dane Blanchard (academic staff) with LSU Department of Otolaryngology Head Neck Surgery.

The authors would like to thank Eugene New for his help with the illustrations contained in this chapter (medart4u@yahoo.com).


  1. 1.
    Doig JA et al. Otolaryngological aspects of Sjogren’s syndrome. Br Med J. 1971;4(5785):460–3.PubMedCrossRefGoogle Scholar
  2. 2.
    Carsons S. Sjogren’s syndrome. In: Firestein G, Budd RC, Harris ED, McInnes IB, Ruddy S, Sergent JS, editors. Firestein: Kelley’s textbook of rheumatology. 8th ed. Philadelphia: W.B. Saunders Company; 2008. p. 1148–69.Google Scholar
  3. 3.
    Tucci M, Quatraro C, Silvestris F. Sjogren’s syndrome: an autoimmune disorder with ­otolaryngological involvement. Acta Otorhinolaryngol Ital. 2005;25(3):139–44.PubMedGoogle Scholar
  4. 4.
    Freeman SR et al. Ear, nose, and throat manifestations of Sjogren’s syndrome: retrospective review of a multidisciplinary clinic. J Otolaryngol. 2005;34(1):20–4.PubMedCrossRefGoogle Scholar
  5. 5.
    Mahoney EJ, Spiegel JH. Sjogren’s disease. Otolaryngol Clin North Am. 2003;36(4):733–45.PubMedCrossRefGoogle Scholar
  6. 6.
    Trune DR. Cochlear immunoglobulin in the C3H/lpr mouse model for autoimmune hearing loss. Otolaryngol Head Neck Surg. 1997;117(5):504–8.PubMedCrossRefGoogle Scholar
  7. 7.
    Nariuchi H et al. Mechanisms of hearing disturbance in an autoimmune model mouse NZB/kl. Acta Otolaryngol Suppl. 1994;514:127–31.PubMedCrossRefGoogle Scholar
  8. 8.
    Moysan JF et al. Is the target of anti-cardiolipin antibodies the same in Gougerot-Sjogren syndrome and lupus erythematosus disseminatus? Rev Med Interne. 1987;8(2):163–8.PubMedCrossRefGoogle Scholar
  9. 9.
    Campbell SM, Montanaro A, Bardana EJ. Head and neck manifestations of autoimmune disease. Am J Otolaryngol. 1983;4(3):187–216.PubMedCrossRefGoogle Scholar
  10. 10.
    Vitali C et al. Classification criteria for Sjogren’s syndrome: a revised version of the European criteria proposed by the American-European Consensus Group. Ann Rheum Dis. 2002;61(6):554–8.PubMedCrossRefGoogle Scholar
  11. 11.
    Takeuchi K et al. Nasal mucociliary clearance in Sjogren’s syndrome. Dissociation in flow between sol and gel layers. Acta Otolaryngol. 1989;108(1–2):126–9.PubMedCrossRefGoogle Scholar
  12. 12.
    Rasmussen N, Brofeldt S, Manthorpe R. Smell and nasal findings in patients with primary Sjogren’s syndrome. Scand J Rheumatol Suppl. 1986;61:142–5.PubMedGoogle Scholar
  13. 13.
    Jacobsen H, Johannessen AC, et al. Sjogren’s syndrome in Bergen during a two-year period. ENT symptoms in Sjogren’s syndrome. In: Transactions of the XXV Congress of the Scandinavian Oto-Laryngological Society. Bergen. 1993.Google Scholar
  14. 14.
    Henkin RI et al. Abnormalities of taste and smell in Sjogren’s syndrome. Ann Intern Med. 1972;76(3):375–83.PubMedGoogle Scholar
  15. 15.
    Doty RL, Shaman P, Dann M. Development of the University of Pennsylvania Smell Identification Test: a standardized microencapsulated test of olfactory function. Physiol Behav. 1984;32(3):489–502.PubMedCrossRefGoogle Scholar
  16. 16.
    Doty RL et al. University of Pennsylvania Smell Identification Test: a rapid quantitative olfactory function test for the clinic. Laryngoscope. 1984;94(2 Pt 1):176–8.PubMedCrossRefGoogle Scholar
  17. 17.
    Lin DF et al. Clinical and prognostic characteristics of 573 cases of primary Sjogren’s syndrome. Chin Med J (Engl). 2010;123(22):3252–7.Google Scholar
  18. 18.
    Garcia-Carrasco M et al. Primary Sjogren syndrome: clinical and immunologic disease patterns in a cohort of 400 patients. Medicine (Baltimore). 2002;81(4):270–80.CrossRefGoogle Scholar
  19. 19.
    Skopouli FN et al. Clinical evolution, and morbidity and mortality of primary Sjogren’s syndrome. Semin Arthritis Rheum. 2000;29(5):296–304.PubMedCrossRefGoogle Scholar
  20. 20.
    Davidson BK, Kelly CA, Griffiths ID. Primary Sjogren’s syndrome in the North East of England: a long-term follow-up study. Rheumatology (Oxford). 1999;38(3):245–53.CrossRefGoogle Scholar
  21. 21.
    Alamanos Y et al. Epidemiology of primary Sjogren’s syndrome in north-west Greece, ­1982–2003. Rheumatology (Oxford). 2006;45(2):187–91.CrossRefGoogle Scholar
  22. 22.
    Kjellen G et al. Esophageal function, radiography, and dysphagia in Sjogren’s syndrome. Dig Dis Sci. 1986;31(3):225–9.PubMedCrossRefGoogle Scholar
  23. 23.
    Anselmino M et al. Esophageal motor function in primary Sjogren’s syndrome: correlation with dysphagia and xerostomia. Dig Dis Sci. 1997;42(1):113–8.PubMedCrossRefGoogle Scholar
  24. 24.
    Bloch KB, Buchanan WW, Wohl MJ, et al. Sjogren’s syndrome: a clinical, pathological, and serological study of 62 cases. Medicine. 1965;44:187–231.PubMedCrossRefGoogle Scholar
  25. 25.
    Prytz S. Vocal nodules in Sjogren’s syndrome. J Laryngol Otol. 1980;94(2):197–203.PubMedCrossRefGoogle Scholar
  26. 26.
    Valter HR, Pillon J, Kosugi EM, Fujita R, Pontes P. Laryngeal assessment in reumatic disease patients. Rev Bras Otorrinolaringol. 2005;71(4):499–503.CrossRefGoogle Scholar
  27. 27.
    Murano E et al. Bamboo node: primary vocal fold lesion as evidence of autoimmune disease. J Voice. 2001;15(3):441–50.PubMedCrossRefGoogle Scholar
  28. 28.
    Cooke TD et al. The pathogenesis of chronic inflammation in experimental antigen-induced arthritis. II. Preferential localization of antigen-antibody complexes to collagenous tissues. J Exp Med. 1972;135(2):323–38.PubMedCrossRefGoogle Scholar
  29. 29.
    Gilliam JN, Cheatum DE. Immunoglobulins in the larynx in systemic lupus erythematosus. Arch Dermatol. 1973;108(5):696–7.PubMedCrossRefGoogle Scholar
  30. 30.
    Grundy DJ. Sjogren’s disease with widespread lymphoid deposits. Proc R Soc Med. 1972;65(2):167–8.PubMedGoogle Scholar
  31. 31.
    Barrs DM, McDonald TJ, Duffy J. Sjogren’s syndrome involving the larynx: report of a case. J Laryngol Otol. 1979;93(9):933–6.PubMedCrossRefGoogle Scholar

Copyright information

© Springer-Verlag London Limited 2011

Authors and Affiliations

  • Rohan R. Walvekar
    • 1
  • Francis Marchal
    • 2
  1. 1.Department of Otolaryngology Head and Neck Surgery, LSU Health Sciences CenterSalivary Endoscopy Service and Clinical ResearchNew OrleansUSA
  2. 2.Department of Otolaryngology Head and Neck Surgery, Sialendoscopy Unit, European Sialendoscopy Training Center (ETSC)University Hospital of GenevaGenevaSwitzerland

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