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Otolaryngologic Manifestations of Sjögren’s Syndrome

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Sjögren’s Syndrome

Abstract

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.

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References

  1. Kassan SS, Moutsopoulos HM. Clinical manifestations and early diagnosis of Sjögren’s syndrome. Arch Intern Med. 2004;164:1275–84.

    Article  PubMed  Google Scholar 

  2. Mahoney EJ, Speigel JH. Sjögren’s disease. Otol Clin N Am. 2003;36:733–45.

    Article  Google Scholar 

  3. Ng KP, Isenberg DA. Sjögren’s syndrome. Diagnosis and therapeutic challenges in the elderly. Drugs Aging 2008;25(1):19–33.

    Article  PubMed  CAS  Google Scholar 

  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.

    Article  PubMed  CAS  Google Scholar 

  5. Manthorpe R. Sjögren’s syndrome criteria. Ann Rheum Dis. 2002;61:482–4.

    Article  PubMed  CAS  Google Scholar 

  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.

    Article  PubMed  CAS  Google Scholar 

  7. Fox RI. Clinical features, pathogenesis, and treatment of Sjögren’s syndrome. Curr Op Rheum. 1996;8:438–45.

    Article  CAS  Google Scholar 

  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.

    Article  PubMed  CAS  Google Scholar 

  9. Srinivasan S, Slomovic AR. Sjögren syndrome. Comp Ophthamol Update. 2007;8:205–12.

    Google Scholar 

  10. Soy M, Piskin S. Cutaneous findings in patients with primary Sjögren’s syndrome. Clin Rheum. 2007;26(8):1350–2.

    Article  Google Scholar 

  11. Tucci M, Quatraro C, Silvestris F. Sjögren’s syndrome: an autoimmune disorder with otolaryngological involvement. Acta Oto Ital. 2005;25:139–44.

    CAS  Google Scholar 

  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. 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.

    Article  PubMed  CAS  Google Scholar 

  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.

    Article  PubMed  CAS  Google Scholar 

  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.

    Article  PubMed  Google Scholar 

  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.

    PubMed  Google Scholar 

  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.

    Article  PubMed  CAS  Google Scholar 

  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. 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.

    PubMed  Google Scholar 

  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.

    Article  PubMed  Google Scholar 

  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.

    Article  Google Scholar 

  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. Soto-Rojas AE, Kraus A. The oral side of Sjögren’s syndrome. Diagnosis and treatment. Arch Med Res. 2002;33:95–106.

    Article  PubMed  Google Scholar 

  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.

    PubMed  CAS  Google Scholar 

  25. Mathews SA, Kurien BT, Scofield RH. Oral manifestations of Sjögren’s syndrome. J Dent Res. 2008;87(4):308–18.

    Article  PubMed  CAS  Google Scholar 

  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.

    Article  PubMed  CAS  Google Scholar 

  27. Larché M. A short review of the pathogenesis of Sjögren’s syndrome. Autoimmun Rev. 2006;5:132–5.

    Article  PubMed  Google Scholar 

  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.

    PubMed  CAS  Google Scholar 

  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.

    Article  PubMed  CAS  Google Scholar 

  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.

    Article  PubMed  CAS  Google Scholar 

  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.

    PubMed  CAS  Google Scholar 

  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.

    Article  Google Scholar 

  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.

    Article  CAS  Google Scholar 

  34. Voulgarelis M, Dafni UG, Isenberg DA, Moutsopoulos HM. Malignant lymphoma in primary Sjögren’s syndrome. Arth Rheum. 1999;42(8):1765–72.

    Article  CAS  Google Scholar 

  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.

    Article  PubMed  CAS  Google Scholar 

  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.

    Article  Google Scholar 

  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.

    CAS  Google Scholar 

  38. Tumiati B, Casoli P, Parmeggiani A. Hearing loss in the Sjögren syndrome. Ann Int Med. 1997;126(6):450–3.

    PubMed  CAS  Google Scholar 

  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.

    PubMed  CAS  Google Scholar 

  40. Alobid I, Guilemany JM, Mullol J. Nasal manifestations of systemic illnesses. Curr All Asth Rep. 2004;4:208–16.

    Article  Google Scholar 

  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.

    Article  PubMed  CAS  Google Scholar 

  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.

    Article  PubMed  CAS  Google Scholar 

  43. Belafksy PC, Postma GN. The laryngeal and esophageal manifestations of Sjögren’s syndrome. Curr Rheum Rep. 2003;5:297–303.

    Article  Google Scholar 

  44. Belafsky PC, Postma GN, Koufman JA. Validity and reliability of the reflux symptom index (RSI). J Voice. 2002;16(2):274–7.

    Article  PubMed  Google Scholar 

  45. Ford CN. Evaluation and management of laryngopharyngeal reflux. JAMA 2005;294:1534–40.

    Article  PubMed  CAS  Google Scholar 

  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.

    Article  Google Scholar 

  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.

    Article  PubMed  CAS  Google Scholar 

  48. Volter F, Fain O, Mathieu E, Thomas M. Esophageal function and Sjögren’s syndrome. Dig Dis Sci. 2004;49(2):248–53.

    Article  PubMed  Google Scholar 

  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.

    Article  PubMed  CAS  Google Scholar 

  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.

    Article  PubMed  Google Scholar 

  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.

    Article  PubMed  CAS  Google Scholar 

  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.

    Article  PubMed  Google Scholar 

  53. Dent J. Patterns of lower esophageal sphincter function associated with gastroesophageal reflux. Am J Med. 1997;103(5A):29S–32S.

    Article  PubMed  CAS  Google Scholar 

  54. Pope CE. The esophagus for the nonesophagologist. Am J Med. 1997;103(5A):19S–22S.

    Article  PubMed  Google Scholar 

  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.

    Article  PubMed  CAS  Google Scholar 

  56. Pasricha PJ. Effect of sleep on gastroesophageal physiology and airway protective mechanisms. Am J Med. 2003;115(3A):114S–8S.

    Article  PubMed  Google Scholar 

  57. Shaker R, Hogan WJ. Reflex-mediated enhancement of airway protective mechanisms. Am J Med. 2000; 108(4A):8S–14S.

    Article  PubMed  Google Scholar 

  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.

    Article  PubMed  CAS  Google Scholar 

  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.

    PubMed  CAS  Google Scholar 

  60. Sheikh SH, Shaw-Stiffel TA. The gastrointestinal manifestations of Sjögren’s syndrome. Am J Gastroenterol. 1995;90(1):9–14.

    PubMed  CAS  Google Scholar 

  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. 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.

    Article  PubMed  Google Scholar 

  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.

    Article  PubMed  CAS  Google Scholar 

  64. Richter JE. Novel medical therapies for gastroesophageal reflux disease beyond proton pump inhibitors. Gastroenterol Clin North Am. 2002;31(4 Suppl):S111–6.

    Article  PubMed  Google Scholar 

  65. Holloway RH. Systemic pharmacomodulation of transient lower esophageal sphincter relaxations. Am J Med. 2001;(111 Suppl 8A):178S–85S.

    Article  PubMed  Google Scholar 

  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.

    Article  PubMed  CAS  Google Scholar 

  67. Fox RI. Sjögren’s syndrome. Lancet 2005;366:321–31.

    Article  PubMed  CAS  Google Scholar 

  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.

    Article  CAS  Google Scholar 

  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.

    Article  CAS  Google Scholar 

  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.

    Article  CAS  Google Scholar 

  71. Shiozawa S, Cummins JM, Fox PC. Opening the flood gates—interferon alpha treatment for Sjögren’s syndrome. Biodrugs 2000;13(5):305–11.

    Article  PubMed  CAS  Google Scholar 

  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.

    Article  CAS  Google Scholar 

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Author information

Authors and Affiliations

  1. Department of Otolaryngology, University of Auckland, Auckland, New Zealand

    Jacqui E. Allen

  2. The Center for Voice and Swallowing, University of California at Davis, Davis, CA, USA

    Peter C. Belafsky

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  1. Jacqui E. Allen
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  2. Peter C. Belafsky
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Correspondence to Peter C. Belafsky .

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Editors and Affiliations

  1. Research Foundation, Rheumatology Clinic, Scripps Memorial Hospital &, Caminito Sinnecock 6565, La Jolla, 92037, California, USA

    Robert I. Fox

  2. Research Foundation, Rheumatology Clinic, Scripps Memorial Hospital &, Caminito Sinnecock 6565, La Jolla, 92037, California, USA

    Carla M. Fox

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Allen, J.E., Belafsky, P.C. (2011). Otolaryngologic Manifestations of Sjögren’s Syndrome. In: Fox, R., Fox, C. (eds) Sjögren’s Syndrome. Springer, New York, NY. https://doi.org/10.1007/978-1-60327-957-4_16

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  • DOI: https://doi.org/10.1007/978-1-60327-957-4_16

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