Otolaryngologic Manifestations of Sjögren’s Syndrome



Sjögren’s syndrome (SS) is an insidious progressive autoimmune disease characterized by exocrinopathy and a predilection for women. It affects many organ systems; however, head and neck manifestations predominate and are frequently the initial presenting complaints. A high index of suspicion should be maintained to permit early diagnosis and minimize long-term complications of the disease. This chapter aims to review the otolaryngologic manifestations of Sjögren’s syndrome, current diagnostic tools, and treatment.


Sjögren’s syndrome Oral Nasal Laryngeal Esophageal Esophageal dysmotility Laryngopharyngeal reflux Xerostomia Xerotrachea Hyposalivation Dental caries Salivary gland lymphoma Sialography Salivary scintigraphy Salivary MRI Lip biopsy Parotid biopsy Autoimmune Extra-glandular manifestations 


  1. 1.
    Kassan SS, Moutsopoulos HM. Clinical manifestations and early diagnosis of Sjögren’s syndrome. Arch Intern Med. 2004;164:1275–84.PubMedCrossRefGoogle Scholar
  2. 2.
    Mahoney EJ, Speigel JH. Sjögren’s disease. Otol Clin N Am. 2003;36:733–45.CrossRefGoogle Scholar
  3. 3.
    Ng KP, Isenberg DA. Sjögren’s syndrome. Diagnosis and therapeutic challenges in the elderly. Drugs Aging 2008;25(1):19–33.PubMedCrossRefGoogle Scholar
  4. 4.
    Al-Hashimi I, Khuder S, Haghighat N, Zipp M. Frequency and predictive value of the clinical manifestations in Sjögren’s syndrome. J Oral Pathol Med. 2001;30:1–6.PubMedCrossRefGoogle Scholar
  5. 5.
    Manthorpe R. Sjögren’s syndrome criteria. Ann Rheum Dis. 2002;61:482–4.PubMedCrossRefGoogle Scholar
  6. 6.
    Vitali C, Bombardieri S, Jonsson R, et al. Classification criteria for Sjögren’s syndrome: a revised version of the European criteria proposed by the American–European Consensus Group. Ann Rheum Dis. 2002; 61:554–8.PubMedCrossRefGoogle Scholar
  7. 7.
    Fox RI. Clinical features, pathogenesis, and treatment of Sjögren’s syndrome. Curr Op Rheum. 1996;8:438–45.CrossRefGoogle Scholar
  8. 8.
    Jonsson R, Bolstad AI, Brokstad KA, Brun JG. Sjögren’s syndrome—a plethora of clinical and immunological phenotypes with a complex genetic background. Ann NY Acad Sci. 2007;1108:433–47.PubMedCrossRefGoogle Scholar
  9. 9.
    Srinivasan S, Slomovic AR. Sjögren syndrome. Comp Ophthamol Update. 2007;8:205–12.Google Scholar
  10. 10.
    Soy M, Piskin S. Cutaneous findings in patients with primary Sjögren’s syndrome. Clin Rheum. 2007;26(8):1350–2.CrossRefGoogle Scholar
  11. 11.
    Tucci M, Quatraro C, Silvestris F. Sjögren’s syndrome: an autoimmune disorder with otolaryngological involvement. Acta Oto Ital. 2005;25:139–44.Google Scholar
  12. 12.
    McGuirt WF, Whang C, Moreland W. The role of parotid biopsy in the diagnosis of pediatric Sjögren’s syndrome. Arch Oto Head Neck Surg. 2002;128:1279–81.Google Scholar
  13. 13.
    Pijpe J, Kalk WWI, Bootsma H, Spijkervet FKL, Kallenberg CGM, Vissink A. Progression of salivary gland dysfunction in patients with Sjögren’s syndrome. Ann Rheum Dis. 2007;66:107–12.PubMedCrossRefGoogle Scholar
  14. 14.
    Mignogna MD, Fedele S, Lo Russo L, Lo Muzio L, Wolff A. Sjögren’s syndrome: the diagnostic potential of early oral manifestations preceding hyposalivation/xerostomia. J Oral Pathol Med. 2005;34:1–6.PubMedCrossRefGoogle Scholar
  15. 15.
    Hocevar A, Rainer S, Rozman B, Zor P, Tomšic M. Ultrasonographic changes of major salivary glands in primary Sjögren’s syndrome. Evaluation of a novel scoring system. Eur J Radiol. 2007;63:379–83.PubMedCrossRefGoogle Scholar
  16. 16.
    Wernicke D, Hess H, Gromnica-Ihle E, Krause A, Schmidt WA. Ultrasonography of salivary glands—a highly specific imaging procedure for diagnosis of Sjögren’s syndrome. J Rheum. 2008;35:285–93.PubMedGoogle Scholar
  17. 17.
    Salaffi F, Carotti M, Iagnocco A, et al. Ultrasonography of salivary glands in primary Sjögren’s syndrome: a comparison with contrast sialography and scintigraphy. Rheumatology 2008;47:1244–9.PubMedCrossRefGoogle Scholar
  18. 18.
    Booker J, Howarth D, Taylor L, Voutnis D, Sutherland D. Appropriate utilization of semi-quantitative analysis in salivary scintigraphy. Nuc Med. 2004;25:1203–10.Google Scholar
  19. 19.
    Decuzzi M, Tatulli F, Giampaolo M, et al. Sialocintigraphy versus ultrasonography of the salivary glands in patients first diagnosed with Sjögren’s syndrome. Hell J Nucl Med. 2006;9(2):103–5.PubMedGoogle Scholar
  20. 20.
    Niemelä RK, Takalo R, Pääko E, et al. Ultrasonography of salivary glands in primary Sjögren’s syndrome. A comparison with magnetic resonance imaging and magnetic resonance sialography of parotid glands. Rheumatology 2004;43:875–9.PubMedCrossRefGoogle Scholar
  21. 21.
    Takagi Y, Sumi M, Van Cauteren M, Nakamura T. Fast and high-resolution MR sialography using a small surface coil. J Mag Res Imag. 2005;22:29–37.CrossRefGoogle Scholar
  22. 22.
    Morimoto Y, Habu M, Tomoyose T, et al. Dynamic magnetic resonance sialography as a new diagnostic technique for patients with Sjögren’s syndrome. Oral Dis. 1006;12:408–14.Google Scholar
  23. 23.
    Soto-Rojas AE, Kraus A. The oral side of Sjögren’s syndrome. Diagnosis and treatment. Arch Med Res. 2002;33:95–106.PubMedCrossRefGoogle Scholar
  24. 24.
    Soto-Rojas AE, Villa AR, Sifuentes-Osornio J, Alarcón-Segovia D, Kraus A. Oral manifestations in patients with Sjögren’s syndrome. J Rheumatol. 1998;25:906–10.PubMedGoogle Scholar
  25. 25.
    Mathews SA, Kurien BT, Scofield RH. Oral manifestations of Sjögren’s syndrome. J Dent Res. 2008;87(4):308–18.PubMedCrossRefGoogle Scholar
  26. 26.
    Pijpe J, Kalk WWI, van der Wal JE, et al. Parotid gland biopsy compared with labial biopsy in the diagnosis of patients with primary Sjögren’s syndrome. Rheumatology 2007;46:335–41.PubMedCrossRefGoogle Scholar
  27. 27.
    Larché M. A short review of the pathogenesis of Sjögren’s syndrome. Autoimmun Rev. 2006;5:132–5.PubMedCrossRefGoogle Scholar
  28. 28.
    Lisi S, Sisto M, Scagliusi P, Mitolo V, D’Amore M. Sjögren’s syndrome: anti-Ro and anti-La autoantibodies trigger apoptotic mechanism in the human salivary gland cell line, A-253. Panminerva Med. 2007;49:103–8.PubMedGoogle Scholar
  29. 29.
    Sisto M, Lisi S, Lofrumento D, D’Amore M, Scagliusi P, Mitolo V. Autoantibodies from Sjögren’s syndrome trigger apoptosis in salivary gland cell line. Ann NY Acad Sci. 2007;1108:418–25.PubMedCrossRefGoogle Scholar
  30. 30.
    Theander E, Henriksson G, Ljungberg O, Mandl T, Manthorpe R, Jacobsson LTH. Lymphoma and other malignancies in primary Sjögren’s syndrome: a cohort study on cancer incidence and lymphoma predictors. Ann Rheum Dis. 2006;65:796–803.PubMedCrossRefGoogle Scholar
  31. 31.
    Pariente D, Anaya JM, Combe B, et al. Non-Hodgkin’s lymphoma associated with primary Sjögren’s syndrome. Eur J Med. 1992;1:337–42.PubMedGoogle Scholar
  32. 32.
    Ioannidis JPA, Vassiliou V, Moutsopoulos HM. Long-term risk of mortality and lymphoproliferative disease and predictive classification of primary Sjögren’s syndrome. Arth Rheum. 2002;46(3):741–7.CrossRefGoogle Scholar
  33. 33.
    Mackay F, Groom JR, Tangye SG. An important role for B-cell activation factor and B cells in the pathogenesis of Sjögren’s syndrome. Curr Opin Rheum. 2007;19(5):406–13.CrossRefGoogle Scholar
  34. 34.
    Voulgarelis M, Dafni UG, Isenberg DA, Moutsopoulos HM. Malignant lymphoma in primary Sjögren’s syndrome. Arth Rheum. 1999;42(8):1765–72.CrossRefGoogle Scholar
  35. 35.
    Kauppi M, Pukkala E, Isomäki H. Elevated incidence of hematologic malignancies in patients with Sjögren’s syndrome compared with patients with rheumatoid arthritis (Finland). Cancer Causes Control 1997;8:201–4.PubMedCrossRefGoogle Scholar
  36. 36.
    Freeman SRM, Sheehan PZ, Thorpe MA, Rutka JA. Ear, nose and throat manifestations of Sjögren’s syndrome: retrospective review of a multidisciplinary clinic. J Otol. 2005;34:20–4.CrossRefGoogle Scholar
  37. 37.
    Ziavra N, Politi EN, Kastanioudakis I, Skevas A, Drosos AA. Hearing loss in Sjögren’s syndrome patients. A comparative study. Clin Exp Rheum. 2000;18:725–8.Google Scholar
  38. 38.
    Tumiati B, Casoli P, Parmeggiani A. Hearing loss in the Sjögren syndrome. Ann Int Med. 1997;126(6):450–3.PubMedGoogle Scholar
  39. 39.
    Boki KA, Ioannidis JPA, Segas JV, et al. How significant is sensorineural hearing loss in primary Sjögren’s syndrome? An individually matched case–control study. J Rheumatol. 2001;28:798–801.PubMedGoogle Scholar
  40. 40.
    Alobid I, Guilemany JM, Mullol J. Nasal manifestations of systemic illnesses. Curr All Asth Rep. 2004;4:208–16.CrossRefGoogle Scholar
  41. 41.
    Takeuchi K, Sakakura Y, Murai S, Majima Y. Nasal mucociliary clearance in Sjögren’s syndrome. Dissociation in flow between sol and gel layers. Acta Otolaryngol. 1989;108:126–9.PubMedCrossRefGoogle Scholar
  42. 42.
    Papiris SA, Maniati M, Constantopoulos SH, Roussos C, Moutsopoulos HM, Skopouli FN. Lung involvement in primary Sjögren’s syndrome is mainly related to the small airway disease. Ann Rheum Dis. 1999;58:61–4.PubMedCrossRefGoogle Scholar
  43. 43.
    Belafksy PC, Postma GN. The laryngeal and esophageal manifestations of Sjögren’s syndrome. Curr Rheum Rep. 2003;5:297–303.CrossRefGoogle Scholar
  44. 44.
    Belafsky PC, Postma GN, Koufman JA. Validity and reliability of the reflux symptom index (RSI). J Voice. 2002;16(2):274–7.PubMedCrossRefGoogle Scholar
  45. 45.
    Ford CN. Evaluation and management of laryngopharyngeal reflux. JAMA 2005;294:1534–40.PubMedCrossRefGoogle Scholar
  46. 46.
    Ogut F, Midilli R, Oder G, Engin EZ, Karci B, Kabasakal Y. Laryngeal findings and voice quality in Sjögren’s syndrome. Auris Nas Larynx. 2005;32:375–80.CrossRefGoogle Scholar
  47. 47.
    Murano E, Hosako-Naito Y, Oka T, Miyaji M, Kumada M, Niimi S. Bamboo node: primary vocal fold lesion as evidence of autoimmune disease. J Voice. 2001;15:441–50.PubMedCrossRefGoogle Scholar
  48. 48.
    Volter F, Fain O, Mathieu E, Thomas M. Esophageal function and Sjögren’s syndrome. Dig Dis Sci. 2004;49(2):248–53.PubMedCrossRefGoogle Scholar
  49. 49.
    Palma R, Freire A, Freitas J, et al. Esophageal motility disorders in patients with Sjögren’s syndrome. Dig Dis Sci. 1994;39(4):758–61.PubMedCrossRefGoogle Scholar
  50. 50.
    Kjellén G, Fransson SG, Lindström F, Sökjer H, Tibbling L. Esophageal function, radiography, and dysphagia in Sjögren’s syndrome. Dig Dis Sci. 1986;31(3):225–9.PubMedCrossRefGoogle Scholar
  51. 51.
    Mandl T, Ekberg O, Wollmer P, Manthorpe R, Jacobsson LTH. Dysphagia and dysmotility of the pharynx and oesophagus in patients with primary Sjögren’s syndrome. Scand J Rheumatol. 2007;36:394–401.PubMedCrossRefGoogle Scholar
  52. 52.
    Türk T, Pirildar T, Tunç, Bor S, Doganavsargil E. Manometric assessment of esophageal motility in patients with primary Sjögren’s syndrome. Rheumatol Int. 2005;25:246–9.PubMedCrossRefGoogle Scholar
  53. 53.
    Dent J. Patterns of lower esophageal sphincter function associated with gastroesophageal reflux. Am J Med. 1997;103(5A):29S–32S.PubMedCrossRefGoogle Scholar
  54. 54.
    Pope CE. The esophagus for the nonesophagologist. Am J Med. 1997;103(5A):19S–22S.PubMedCrossRefGoogle Scholar
  55. 55.
    Biancani P, Sohn UD, Rich HG, Harnett KM, Behar J. Signal transduction pathways in esophageal and lower esophageal sphincter circular muscle. Am J Med. 1997;103(5A):23S–8S.PubMedCrossRefGoogle Scholar
  56. 56.
    Pasricha PJ. Effect of sleep on gastroesophageal physiology and airway protective mechanisms. Am J Med. 2003;115(3A):114S–8S.PubMedCrossRefGoogle Scholar
  57. 57.
    Shaker R, Hogan WJ. Reflex-mediated enhancement of airway protective mechanisms. Am J Med. 2000; 108(4A):8S–14S.PubMedCrossRefGoogle Scholar
  58. 58.
    Tsianos EB, Chiras CD, Drosos AA, Moutsopoulos HM. Esophageal dysfunction in patients with primary Sjögren’s syndrome. Ann Rheum Dis. 1985;44:610–13.PubMedCrossRefGoogle Scholar
  59. 59.
    Grande L, Lacima G, Ros E, Font J, Pera C. Esophageal motor function in primary Sjögren’s syndrome. Am J Gastroenterol. 1993;88(3):378–81.PubMedGoogle Scholar
  60. 60.
    Sheikh SH, Shaw-Stiffel TA. The gastrointestinal manifestations of Sjögren’s syndrome. Am J Gastroenterol. 1995;90(1):9–14.PubMedGoogle Scholar
  61. 61.
    Rosztóczy A, Kovács L, Wittmann T, Lonovics J, Pokorny G. Manometric assessment of impaired esophageal motor function in primary Sjögren’s syndrome. Clin Exper Rheum. 2001;19:147–52.Google Scholar
  62. 62.
    Poglio F, Mongini T, Cocito D. Sensory ataxic neuropathy and esophageal achalasia in a patient with Sjögren’s syndrome. Muscle Nerve 2007;35:532–5.PubMedCrossRefGoogle Scholar
  63. 63.
    Vela MF, Tutuian R, Katz PO, Castell DO. Baclofen decreases acid and non-acid post-prandial gastroesophageal reflux measured by combined multichannel intraluminal impedance and pH. Aliment Pharmacol Ther. 2003;17(2):243–51.PubMedCrossRefGoogle Scholar
  64. 64.
    Richter JE. Novel medical therapies for gastroesophageal reflux disease beyond proton pump inhibitors. Gastroenterol Clin North Am. 2002;31(4 Suppl):S111–6.PubMedCrossRefGoogle Scholar
  65. 65.
    Holloway RH. Systemic pharmacomodulation of transient lower esophageal sphincter relaxations. Am J Med. 2001;(111 Suppl 8A):178S–85S.PubMedCrossRefGoogle Scholar
  66. 66.
    Lehmann A, Antonsson M, Bremner-Danielson M, Flärd M, Hansson-Bränden L, Kärrberg L. Activation of the GABA(B) receptor inhibits transient lower esophageal sphincter relaxation in dogs. Gastroenterology 1999;117(5):1147–54.PubMedCrossRefGoogle Scholar
  67. 67.
    Fox RI. Sjögren’s syndrome. Lancet 2005;366:321–31.PubMedCrossRefGoogle Scholar
  68. 68.
    Fife RS, Chase WF, Dore RK, et al. Cevimeline for the treatment of xerostomia in patients with Sjögren’s syndrome. Arch Int Med. 2002;162:1293–300.CrossRefGoogle Scholar
  69. 69.
    Vivino FB, Al-Hashimi I, Khan Z, et al. Pilocarpine tablets for the treatment of dry mouth and dry eye symptoms in patients with Sjögren’s syndrome. Arch Int Med. 1999;159:174–81.CrossRefGoogle Scholar
  70. 70.
    Petrone D, Condemi JJ, Fife R, Gluck O, Cohen S, Dalgin P. A double-blind, randomized, placebo-controlled study of cevimeline in Sjögren’s syndrome patients with xerostomia and keratoconjunctivitis sicca. Arth Rheum. 2002;46(3):748–54.CrossRefGoogle Scholar
  71. 71.
    Shiozawa S, Cummins JM, Fox PC. Opening the flood gates—interferon alpha treatment for Sjögren’s syndrome. Biodrugs 2000;13(5):305–11.PubMedCrossRefGoogle Scholar
  72. 72.
    Sankar V, Brennan MT, Kok MR, et al. Etanercept in Sjögren’s syndrome. A twelve-week randomized, double-blind, placebo-controlled pilot clinical trial. Arth Rheum. 2004;50(7):2240–5.CrossRefGoogle Scholar

Copyright information

© Springer Science+Business Media, LLC 2011

Authors and Affiliations

  1. 1.Department of OtolaryngologyUniversity of AucklandAucklandNew Zealand
  2. 2.The Center for Voice and Swallowing, University of California at DavisDavisUSA

Personalised recommendations