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Primary Sjögren Syndrome in Primary Health Care

  • Antoni Sisó-Almirall
  • Jaume Benavent
  • Xavier Bosch
  • Albert Bové
  • Manuel Ramos-Casals
Chapter

Abstract

Sjogren’s syndrome (SjS) is a slowly progressive autoimmune disease that predominantly affects middle-aged women, with a female to male ratio on the order of 9:1. SS is characterized by lymphocytic infiltration of the exocrine glands, mainly the lacrimal and salivary glands, resulting in reduced secretory function and oral and ocular dryness. The syndrome can present alone as primary SjS (pSjS) or in the context of underlying connective tissue disease as secondary SjS (sSjS). Although the pathogenesis of the disease remains elusive, environmental, genetic and hormonal factors all appear to contribute [1].

Keywords

Rose Bengal Salivary Flow Rate Primary Care Practitioner Keratoconjunctivitis Sicca Sicca Symptom 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.

References

  1. 1.
    Mavragani CP, Moutsopoulos HM. The geoepidemiology of Sjögren’s syndrome. Autoimmunity Reviews 2010;9:A305–A310.PubMedCrossRefGoogle Scholar
  2. 2.
    Ramos-Casals M, Solans R, Rosas J, et al. Primary Sjögren syndrome in Spain. Clinical and immunologic expression in 1010 patients. Medicine. 2008;87:210–9.PubMedCrossRefGoogle Scholar
  3. 3.
    Lawn JE, Rohde J, Rifkin S, et al. Alma-Ata 30 years on: revolutionary, relevant, and time to revitalise. Lancet. 2008;372(9642):917–27.PubMedCrossRefGoogle Scholar
  4. 4.
    Sisó A, Aymamí A, Campoy A, et al. Síndrome de Sjögren primario: ?Una entidad infradiagnosticada por el Médico de Familia? 18º Congreso Nacional de la Sociedad Española de Medicina Familiar y Comunitaria. Zaragoza 1998. Aten Primaria. 1998;22 Suppl 1:390.Google Scholar
  5. 5.
    Allen J, Gay B, Crebolder H et al. The European definitions of general practice/family medicine. The key features of the discipline of general practice. EURACT 2005 (2005). Available in http://www.euract.org/index.php?folder_id=25 Accessed Date: [11-October-2010].
  6. 6.
    Kassan SjS, Moutsopoulos HM. Clinical manifestations and early diagnosis of Sjögren syndrome. Arch Intern Med. 2004;164:1275–84.PubMedCrossRefGoogle Scholar
  7. 7.
    Sorlí JV, Doménech IE, Zurián FJ, et al. Sjögren syndrome. Aten Primaria. 2009;41(7):417–9.PubMedCrossRefGoogle Scholar
  8. 8.
    Fox RI. Sjogren’s syndrome. Lancet. 2005;366:321–31.PubMedCrossRefGoogle Scholar
  9. 9.
    Al-Hashimi I. The management of Sjogren’s syndrome in dental practice. J Am Dent Assoc. 2001;132:1409–17.PubMedGoogle Scholar
  10. 10.
    Caffery B, Simpson TL, Wang S, et al. Rose bengal staining of the temporal conjunctiva differentiates Sjögren’s syndrome from keratoconjunctivitis sicca. Invest Ophthalmol Vis Sci. 2010;51(5):2381–7.PubMedCrossRefGoogle Scholar
  11. 11.
    García-Carrasco M, Ramos-Casals M, Rosas J, et al. Primary Sjögren syndrome: clinical and immunologic disease patterns in a cohort of 400 patients. Medicine. 2002;81:270–80.PubMedCrossRefGoogle Scholar
  12. 12.
    Skopouli FN, Dafni U, Ioannidis JP, Moutsopoulos HM. Clinical evolution, and morbidity and mortality of primary Sjogren’s syndrome. Semin Arthritis Rheum. 2000;29:296–304.PubMedCrossRefGoogle Scholar
  13. 13.
    Fox PC, Bowman SJ, Segal B, et al. Oral involvement in primary Sjögren syndrome. J Am Dent Assoc. 2008;139:1592–601.PubMedGoogle Scholar
  14. 14.
    Belenguer R, Ramos-Casals M, Brito-Zeron P, et al. Influence of clinical and immunological parameters on the health-related quality of life of patients with primary Sjögren’s syndrome. Clin Exp Rheumatol. 2005;23:351–6.PubMedGoogle Scholar
  15. 15.
    Ostuni P, Botsios C, Sfriso P, et al. Prevalence and clinical features of fibromyalgia in systemic lupus erythematosus, systemic sclerosis, and Sjögren’s syndrome. Minerva Med. 2002;93:203–9.PubMedGoogle Scholar
  16. 16.
    Vitali C, Bombardieri S, Moutsopoulos HM, et al. Preliminary criteria for the classification of Sjogren’s syndrome. Results of a prospective concerted action supported by the European community. Arthritis Rheum. 1993;36:340–7.PubMedCrossRefGoogle Scholar
  17. 17.
    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
  18. 18.
    Ramos-Casals M, Brito-Zerón P, Pérez-de-Lis M, et al. Sjögren syndrome or Sjögren disease? the histological and immunological bias caused by the 2002 criteria. Clin Rev Allergy Immunol. 2010;38(2–3):178–85.PubMedCrossRefGoogle Scholar
  19. 19.
    Rosas J, Ramos-Casals M, Ena J, et al. Usefulness of basal and pilocarpine-stimulated salivary flow in primary Sjogren’s syndrome. Correlation with clinical, immunological and histological features. Rheumatology. 2002;41:670–5.PubMedCrossRefGoogle Scholar
  20. 20.
    Theander E, Manthorpe R, Jacobsson LT. Mortality and causes of death in primary Sjögren’s syndrome: a prospective cohort study. Arthritis Rheum. 2004;50:1262–9.PubMedCrossRefGoogle Scholar
  21. 21.
    Ioannidis JP, Vassiliou VA, Moutsopoulos HM. Long-term risk of mortality and lymphoproliferative disease and predictive classification of primary Sjogren’s syndrome. Arthritis Rheum. 2002;46:741–7.PubMedCrossRefGoogle Scholar
  22. 22.
    Ramos-Casals M, Brito-Zeron P, Yague J, Akasbi M, Bautista R, Ruano M, et al. Hypocomplementaemia as an immunological marker of morbidity and mortality in patients with primary Sjogren’s syndrome. Rheumatology (Oxford). 2005;44:89–94.CrossRefGoogle Scholar
  23. 23.
    Ramos-Casals M, Font J, Garcia-Carrasco M, et al. Primary Sjogren syndrome: hematologic patterns of disease expression. Medicine. 2002;81:281–92.PubMedCrossRefGoogle Scholar
  24. 24.
    Brito-Zeron P, Ramos-Casals M, Nardi N, et al. Circulating monoclonal immunoglobulins in Sjogren syndrome: prevalence and clinical significance in 237 patients. Medicine. 2005;84:90–7.PubMedCrossRefGoogle Scholar
  25. 25.
    Pérez-de-Lis M, Akasbi M, Sisó A, et al. Cardiovascular risk factors in primary Sjögren’s syndrome: a case-control study in 624 patients. Lupus. 2010;19(8):941–8.PubMedCrossRefGoogle Scholar
  26. 26.
    Kang JH, Lin HC. Comorbidities in patients with primary Sjögren’s Syndrome: a registry-based case-control study. J Rheumatol. 2010;37:1188–94.PubMedCrossRefGoogle Scholar
  27. 27.
    Segal B, Bowman SJ, Fox PC, et al. Primary Sjögren’s Syndrome: health experiences and predictors of health quality among patients in the United States. Health Qual Life Outcomes. 2009;7:46.PubMedCrossRefGoogle Scholar
  28. 28.
    Meijer JM, Meiners PM, Huddleston Slater JJ, et al. Health-related quality of life, employment and disability in patients with Sjogren’s syndrome. Rheumatology. 2009;48(9):1077–82.PubMedCrossRefGoogle Scholar
  29. 29.
    Aragona P, Di Stefano G, Ferreri F, Spinella R, Stilo A. Sodium hyaluronate eye drops of different osmolarity for the treatment of dry eye in Sjögren’s syndrome patients. Br J Ophthalmol. 2002;86(8):879–84.PubMedCrossRefGoogle Scholar
  30. 30.
    Aragona P, Papa V, Micali A, Santocono M, Milazzo G. Long term treatment with sodium hyaluronate-containing artificial tears reduces ocular surface damage in patients with dry eye. Br J Ophthalmol. 2002;86(2):181–4.PubMedCrossRefGoogle Scholar
  31. 31.
    Brignole F, Pisella PJ, Dupas B, Baeyens V, Baudouin C. Efficacy and safety of 0.18% sodium hyaluronate in patients with moderate dry eye syndrome and superficial keratitis. Graefes Arch Clin Exp Ophthalmol. 2005;243(6):531–8.PubMedCrossRefGoogle Scholar
  32. 32.
    Condon PI, McEwen CG, Wright M, Mackintosh G, Prescott RJ, McDonald C. Double blind, randomised, placebo controlled, crossover, multicentre study to determine the efficacy of a 0.1% (w/v) sodium hyaluronate solution (Fermavisc) in the treatment of dry eye syndrome. Br J Ophthalmol. 1999;83(10):1121–4.PubMedCrossRefGoogle Scholar
  33. 33.
    McDonald CC, Kaye SB, Figueiredo FC, Macintosh G, Lockett C. A randomised, crossover, multicentre study to compare the performance of 0.1% (w/v) sodium hyaluronate with 1.4% (w/v) polyvinyl alcohol in the alleviation of symptoms associated with dry eye syndrome. Eye (Lond). 2002;16(5):601–7.CrossRefGoogle Scholar
  34. 34.
    Toda I, Shinozaki N, Tsubota K. Hydroxypropyl methylcellulose for the treatment of severe dry eye associated with Sjögren’s syndrome. Cornea. 1996;15(2):120–8.PubMedCrossRefGoogle Scholar
  35. 35.
    Papas AS, Sherrer YS, Charney M, et al. Successful treatment of dry mouth and dry eye symptoms in Sjögren’s syndrome patients with oral pilocarpine: a randomized, placebo-controlled, dose-adjustment study. J Clin Rheumatol. 2004;10(4):169–77.PubMedCrossRefGoogle Scholar
  36. 36.
    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 syndrome: a randomized, placebo-controlled, fixed-dose, multicenter trial. P92-01 Study Group. Arch Intern Med. 1999;159(2):174–81.PubMedCrossRefGoogle Scholar
  37. 37.
    Wu CH, Hsieh SC, Lee KL, Li KJ, Lu MC, Yu CL. Pilocarpine hydrochloride for the treatment of xerostomia in patients with Sjögren’s syndrome in Taiwan – a double-blind, placebo-controlled trial. J Formos Med Assoc. 2006;105(10):796–803.PubMedCrossRefGoogle Scholar
  38. 38.
    Fife RS, Chase WF, Dore RK, et al. Cevimeline for the treatment of xerostomia in patients with Sjögren syndrome: a randomized trial. Arch Intern Med. 2002;162(11):1293–300.PubMedCrossRefGoogle Scholar
  39. 39.
    Leung KC, McMillan AS, Wong MC, Leung WK, Mok MY, Lau CS. The efficacy of cevimeline hydrochloride in the treatment of xerostomia in Sjögren’s syndrome in southern Chinese patients: a randomised double-blind, placebo-controlled crossover study. Clin Rheumatol. 2008;27(4):429–36.PubMedCrossRefGoogle Scholar
  40. 40.
    Ono M, Takamura E, Shinozaki K, et al. Therapeutic effect of cevimeline on dry eye in patients with Sjögren’s syndrome: a randomized, double-blind clinical study. Am J Ophthalmol. 2004;138(1):6–17.PubMedCrossRefGoogle Scholar
  41. 41.
    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. Arthritis Rheum. 2002;46(3):748–54.PubMedCrossRefGoogle Scholar
  42. 42.
    Voulgarelis M, Moutsopoulos HM. Malignant lymphoma in primary Sjögren’s syndrome. Isr Med Assoc J. 2001;3:761–6.PubMedGoogle Scholar
  43. 43.
    Kruszka P, O’Brian RJ. Diagnosis and management of Sjögren syndrome. Am Fam Physician. 2009;79(6):465–70.PubMedGoogle Scholar

Copyright information

© Springer-Verlag London Limited 2011

Authors and Affiliations

  • Antoni Sisó-Almirall
    • 1
  • Jaume Benavent
    • 1
  • Xavier Bosch
    • 2
  • Albert Bové
    • 3
  • Manuel Ramos-Casals
    • 3
  1. 1.Research Group on Primary Care, Institut d’Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), CAP Les Corts, GesClínicUniversity of BarcelonaBarcelonaSpain
  2. 2.Department of Internal MedicineICMiD, Hospital ClínicBarcelonaSpain
  3. 3.Sjögren Syndrome Research Group (AGAUR), Laboratory of Autoimmune Diseases Josep Font, Institut d’Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), Department of Autoimmune DiseasesHospital ClínicBarcelonaSpain

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