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Educational objectives:

Upon completion of this activity, participants should be able to:

  • Describe the clinical manifestations and laboratory findings included in making a diagnosis of Sjögren’s syndrome (SS)
  • Understand the pathophysiology of SS, including environmental and hormonal factors, and genetic contributors
  • Discuss symptomatic management as treatment of SS
  • Understand the American-European Consensus Group (AECG) and the American College of Rheumatology  (ACR) classification criteria for SS

Reading Resources:

  1. Ramos-Casals M, Brito-Zerón P, Sisó-Almirall A, et al. Primary Sjögen Syndrome.BMJ. 2012;344:e3821.
  2. Mavragani CP, Moutsopoulos HM. The Geoepidemiology of Sjögren’s Syndrome. Autoimmun Rev. 2010;9:A305–A310.
  3. 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;61:554–558.
  4. Maldini C, Seror R, Fain O, et al. Epidemiology of Primary Sjögren’s Syndrome in a French Multiracial/Multiethnic Area. Arthritis Care Res (Hoboken). 2014;66:454–463.
  5. Peri Y, Agmon-Levin N, Theodor E, et al. Sjögren’s Syndrome, The Old and The New.Best Pract Res Clin Rheumatol. 2012;26:105–117.
  6. Shiboski SC, Shiboski CH, Criswell L, et al. American College of Rheumatology Classification Criteria for Sjögren’s syndrome: A Data-Driven, Expert Consensus Approach in the Sjögren’s International Collaborative Clinical Alliance Cohort. Arthritis Care Res (Hoboken). 2012;64:475–487.
  7. Amador-Patarroyo MJ, Arbelaez JG, Mantilla RD, et al. Sjögren’s Syndrome at the Crossroad of Polyautoimmunity. J Autoimmun.  2012;39:199–205.
  8. Roblin X, Helluwaert F, Bonaz B. Celiac Disease Must Be Evaluated in Patients With Sjögren Syndrome. Arch Int Med. 2004;vol. 164, Nov 22.
  9. Triantafyllopoulou A, Moutsopoulos HM. Persistent Viral Infection in Primary Sjögren’s Syndrome: Review and Perspectives. Clin Rev Allergy Immunol. 2007;32:210–14.
  10. Lee SJ, Lee JS, Shin MG, et al. Detection of HTLV-1 in the Labial Salivary Glands of Patients with Sjögren’s Syndrome: A Distinct Clinical Subgroup? J Rheumatol. 2012;39:809–15.
  11. Kivity S, Agmon-Levin N, et al. Autoimmunity and Environment: Infections and Autoimmunity — Friends or Foes? Trends Immunol 2009;30:409–414.
  12. Mavragani CP, Moutsopoulos HM. Sjögren’s Syndrome. Annual Review of Pathology: Mechanisms of Disease.  2014;9:273-85.
  13. Cruz-Tapias P, Rojas-Villarraga A, Maier-Moore S, et al. HLA and Sjögren’s Syndrome Susceptibility. A Meta-Analysis of Worldwide Studies. Autoimmun Rev. 2012;11:281–287.
  14. Karaiskos D, Mavragani CP, Makaroni S, et al. Stress, Coping Strategies, and Social Support in Patients With Primary Sjögren’s Syndrome Prior to Disease Onset: A Retrospective Case-Control Study. Ann Rheum Dis. 2009;68:40–46.
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  31. Brito-Zerón P, Retamozo S, Akasbi M, et al. Annular Erythema in Primary Sjögren’s Syndrome: Description of 43 Non-Asian Cases.  2014;Feb; 23(2):166-75.
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  33. Goules AV, Tatouli IP, Moutsopoulos HM, et al. Clinically Significant Renal Involvement in Primary Sjögren's Syndrome: Clinical Presentation and Outcome. Arthritis Rheum. 2013;Nov;65(11):2945-53.
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  37. Chai J, Logigian EL. Neurological Manifestations of Primary Sjögren’s Syndrome. Curr Opin Neurol. 2010;23:509–513.
  38. Gottenberg JE, Seror R, Miceli-Richard C, et al. Serum Levels of Beta2-Microglobulin and Free Light Chains of Immunoglobulins Are Associated With Systemic Disease Activity in Primary Sjögren’s Syndrome: Data at Enrollment in the Prospective ASSESS Cohort. PLoS One. 2013;8:e59868.
  39. Bournia VK, Vlachoyiannopoulos PG. Subgroups of Sjögren Syndrome Patients According to Serological Profiles. J Autoimmun. 2012;39:15e26.
  40. Jonsson R, Theander E, Sjostrom B, et al. Autoantibodies Present Before Symptom Onset in Primary Sjogen’s Syndrome. JAMA. 2013;310:1854–1855.
  41. Cornec D, Jamin C, Pers JO. Sjögren's Syndrome: Where Do We Stand, and Where Shall We Go?  2014;Jun;51:109-14.
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  43. Sumida T, Tsuboi H, Iizuka M, et al. The Role of M3 Muscarinic Acetylcholine Receptor reactive T Cells in Sjögren’s Syndrome: A Critical Review. J Autoimmun. 2014;51:44e50.
  44. Pertovaara M, Bootorabi F, Kuuslahti M, et al. Novel Carbonic Anhydrase Autoantibodies and Renal Manifestations in Patients With Primary Sjögren’s Syndrome. Rheumatol Oxf. 2011;50:1453e7.
  45. Zintzaras E, Voulgarelis M, Moutsopoulos HM. The Risk of Lymphoma Development in Autoimmune Diseases: A Meta-Analysis. Arch Intern Med. 2005;165:2337–44.
  46. Kassan SS, Thomas TL, Moutsopoulos HM, et al. Increased Risk of Lymphoma in Sicca Syndrome. Ann  Intern Med. 1978;89:888–92.
  47. Lazarus MN, Robinson D, Mak V, et al. Incidence of Cancer in a Cohort of Patients With Primary Sjögren’s Syndrome. Rheumatology. 2006;45:1012–15.
  48. Quartuccio L, Isola M, Baldini C, et al. Biomarkers of Lymphoma in Sjögren’s Syndrome and Evaluation of the Lymphoma Risk in Prelymphomatous Conditions: Results of a Multicenter Study. J Autoimmun. 2014;51:75e80.
  49. Nocturne G, Boudaoud S, Miceli-Richard C, et al. Germline and Somatic Genetic Variations of TNFAIP3 in Lymphoma Complicating Primary Sjögren’s Syndrome. Blood. 2013;122: 4068e76. 
  50. Nezos A, Papageorgiou A, Fragoulis G, et al. B-Cell Activating Factor Genetic Variants in Lymphomagenesis Associated with Primary Sjögren’s Syndrome. J Autoimmun. 2014;51: 89e98.
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  53. Brito-Zerón P, Ramos-Casals M. Advances in the Understanding and Treatment of Systemic Complications in Sjögren’s Syndrome. .2014;Sep;26 (5):520-7.
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  56. De Vita S, Lorenzon G, Rossi G, et al. Salivary Gland Echography in Primary and Secondary Sjögren’s Syndrome. Clin Exp Rheumatol. 1992;10:351e6.
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  63. Akpek EK, Lindsley KB, Adyanthaya RS, et al. Treatment of Sjögren’s Syndrome-Associated Dry Eye an Evidence-Based Review. Ophthalmology. 2011;118:1242–1252.
  64. Furness S, Worthington HV, Bryan G, et al. Interventions for the Management of Dry Mouth: Topical Therapies. Cochrane Database Syst Rev. 2011;7:CD008934.
  65. Noaiseh G, Baker JF, Vivino FB. Comparison of the Discontinuation Rates and Side-Effect Profiles of Pilocarpine and Cevimeline for Xerostomia in Primary Sjögren's Syndrome. Clin Exp Rheumatol. 2014;Jul-Aug;32(4):575-7.
  66. Rihl M, Ulbricht K, Schmidt RE, et al. Treatment of Sicca Symptoms With Hydroxychloroquine in Patients with Sjögren’s Syndrome. Rheumatology (Oxford). 2009;48:796–799.
  67. Yavuz S, Asfuro?lu E, Bicakcigil M, et al. Hydroxychloroquine Improves Dry Eye Symptoms of Patients With Primary Sjögren’s Syndrome. Rheumatol Int. 2011;31:1045–1049.
  68. Gottenberg JE, Ravaud P, Puéchal X, et al. Effects of Hydroxychloroquine on Symptomatic Improvement in Primary Sjögren Syndrome: The JOQUER Randomized Clinical Trial. JAMA. 2014;Jul 16;312(3):249-58.
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Dr. Noaiseh has reported no relevant relationships with any entities producing health care goods or services.

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The University of Pittsburgh School of Medicine designates this enduring material for a maximum of .5 AMA PRA Category 1 Credits™. Each physician should only claim credit commensurate with the extent of their participation in the activity. Other health care professionals are awarded (0.05) continuing education units (CEU) which are equivalent to .5 contact hour.

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Release Date: 12/12/2014 | Last Modified On: 12/12/2014 | Expires: 12/14/2015

This course has been expired.