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126

R.R. Walvekar and F. Marchal

Other vocal cord lesions described in association with SjS include a patient with bilateral vocal nodules with different gross appearance: one side presenting with a granuloma and the other with a polypoidal and edematous lesion [25]. Grundy et al. [30] reported a patient with SjS who presented with a rapidly enlarging mass on the left vocal cord that required a temporary tracheostomy and lateral laryngotomy for tumor excision. Pathology could not distinguish between a sarcomatous transformation versus a pseudolymphoma. Another variation includes a patient reported to have presented with progressive hoarseness and dyspnea that was associated with diffuse thickening and narrowing of the glottic and supraglottic structures [31].

Tracheal involvement in SjS includes dryness and thick adherent mucus with erythrema of the mucosa seen in 25% of patients [9]. Crusting is often present and consequently hemoptysis may occur. However, it must be borne in mind that SjS can be associated with actual lymphocytic infiltration and also with pseudolymphoma of the larynx [9, 25, 30]. Consequently, concerning symptoms such as persistent hoarseness, associated dysphagia, laryngeal lesions, and hemoptysis need further evaluation, histopathological confirmation of benign disease, and exclusion of coexistent malignancy of the aerodigestive tract.

Most laryngopharyngeal and tracheal evaluations reported in the literature have been performed by indirect laryngoscopy. In current practice, high-definition tools with recording capabilities have significantly improved quality of head and neck assessments. Flexible laryngoscopy, high-definition imaging, and stroboscopy to delineate vocal fold motion, and transnasal in-office esophagoscopy and bronchoscopy are tools available to the otolaryngologist today.

The diagnosis and treatment of SjS can be challenging. A collaborative effort involving the otolaryngologist, ophthalmologist, internist, rheumatologist, radiologist, pathologist, and dental services to appropriately diagnose, classify, and treat patients effectively and in a comprehensive fashion is needed [2]. There remain many gaps in our understanding of the ear, nose, and throat manifestations of this disorder.

Acknowledgments 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).

References

1.Doig JA et al. Otolaryngological aspects of Sjogren’s syndrome. Br Med J. 1971;4(5785): 460–3.

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.

3. Tucci M, Quatraro C, Silvestris F. Sjogren’s syndrome: an autoimmune disorder with otolaryngological involvement. Acta Otorhinolaryngol Ital. 2005;25(3):139–44.

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

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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.

5. Mahoney EJ, Spiegel JH. Sjogren’s disease. Otolaryngol Clin North Am. 2003;36(4): 733–45.

6. Trune DR. Cochlear immunoglobulin in the C3H/lpr mouse model for autoimmune hearing loss. Otolaryngol Head Neck Surg. 1997;117(5):504–8.

7. Nariuchi H et al. Mechanisms of hearing disturbance in an autoimmune model mouse NZB/kl. Acta Otolaryngol Suppl. 1994;514:127–31.

8. Moysan JF et al. Is the target of anti-cardiolipin antibodies the same in GougerotSjogren syndrome and lupus erythematosus disseminatus? Rev Med Interne. 1987;8(2): 163–8.

9. Campbell SM, Montanaro A, Bardana EJ. Head and neck manifestations of autoimmune disease. Am J Otolaryngol. 1983;4(3):187–216.

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.

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.

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.

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.

14. Henkin RI et al. Abnormalities of taste and smell in Sjogren’s syndrome. Ann Intern Med. 1972;76(3):375–83.

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.

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.

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.

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.

19. Skopouli FN et al. Clinical evolution, and morbidity and mortality of primary Sjogren’s syndrome. Semin Arthritis Rheum. 2000;29(5):296–304.

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.

21. Alamanos Y et al. Epidemiology of primary Sjogren’s syndrome in north-west Greece, 1982–2003. Rheumatology (Oxford). 2006;45(2):187–91.

22. Kjellen G et al. Esophageal function, radiography, and dysphagia in Sjogren’s syndrome. Dig Dis Sci. 1986;31(3):225–9.

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.

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.

25. Prytz S. Vocal nodules in Sjogren’s syndrome. J Laryngol Otol. 1980;94(2):197–203.

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.

27. Murano E et al. Bamboo node: primary vocal fold lesion as evidence of autoimmune disease. J Voice. 2001;15(3):441–50.

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.

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29. Gilliam JN, Cheatum DE. Immunoglobulins in the larynx in systemic lupus erythematosus. Arch Dermatol. 1973;108(5):696–7.

30. Grundy DJ. Sjogren’s disease with widespread lymphoid deposits. Proc R Soc Med. 1972;65(2):167–8.

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.

Chapter 9

Fatigue in Primary Sjögren’s Syndrome

Barbara M. Segal

Contents

 

9.1

Epidemiology of Fatigue.................................................................................................

129

9.2

Assessing Fatigue.............................................................................................................

130

9.3

Prevalence of Fatigue and Impact of Fatigue on Health-Related

 

 

Quality of Life in Primary SS ........................................................................................

133

9.4

Relationship of Fatigue to Cognitive Symptoms and to Depression...........................

135

9.5Fatigue Viewed From the Physiological Perspective: Relationships

 

Between Fatigue, Sleep Quality, and Neuroendocrine Function ................................

137

9.6

Relationship Between Fibromyalgia and SS.................................................................

138

9.7

Management of Pain and Fatigue..................................................................................

141

9.8

Summary..........................................................................................................................

142

References.................................................................................................................................

142

9.1Epidemiology of Fatigue

Recognition of a syndrome characterized by fatigue in patients without an obvious cardiopulmonary illness dates back to the late nineteenth century [1–3]. Early descriptions of patients with “neurasthenia” focused on the substantial weakness and lassitude accompanying the syndrome, which was particularly prominent among women. Following the conclusion of the First World War, physicians were challenged by the large numbers of battle-scarred veterans afflicted by chronic fatigue [4]. The lack of a generally accepted tool for measuring fatigue impeded progress in understanding that symptom.

Barbara M. Segal

Division of Rheumatic and Autoimmune Disorders,

University of Minnesota Medical School, Minneapolis, MN, USA

Department of Medicine, Hennepin County Medical Center,

Minneapolis, MN, USA

M. Ramos-Casals et al. (eds.), Sjögren’s Syndrome,

129

DOI 10.1007/978-0-85729-947-5_9, © Springer-Verlag London Limited 2012

 

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