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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.
  15. Singh N, Cohen PL. The T cell in Sjögren’s Syndrome: Force Majeure, Not Spectateur. J Autoimmun. 2012;39:229–233.
  16. Cornec D, Devauchelle-Pensec V, Tobón GJ, et al. B cells in Sjögren’s Syndrome: From Pathophysiology to Diagnosis and Treatment. J Autoimmun. 2012;39:161–167.
  17. Mitsias DI, Kapsogeorgou EK, Moutsopoulos HM. Sjögren’s Syndrome: Why Autoimmune Epithelitis?  2006;Nov;12(6):523-32.
  18. Tzioufas AG, Kapsogeorgou EK, Moutsopoulos HM. Pathogenesis of Sjögren’s Syndrome: What We Know and What We Should Learn. J Autoimmun. 2012;39:4–8.
  19. Routsias JG, Tzioufas AG. Autoimmune Response and Target Autoantigens in Sjögren’s Syndrome. Eur. J Clin Investig. 2010;40:1026–38.
  20. Humphreys-Beher MG, Yamachika S, Yamamoto H, Maeda N, et al. Salivary Gland Changes in the NOD Mouse Model for Sjögren’s Syndrome: Is There a Non-Immune Genetic Trigger? Eur.J. Morphol. 1998;36(Suppl.):247–51.
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  22. Castro I, Sepúlveda D, Cortés J, et al. Oral Dryness in Sjögren’s Syndrome Patients. Not Just a Question of Water. Autoimmun Rev. 2013;12:567–74.
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  24. Lendrem D, Mitchell S, McMeekin P, et al. Health-Related Utility Values of Patients With Primary Sjögren’s Syndrome and Its Predictors. Ann Rheum Dis. 2014;73:1362–1368.
  25. Stevenson W, Chauhan SK, Dana R. Dry Eye Disease: An Immune-Mediated Ocular Surface Disorder. Arch Ophthalmol. 2012;130:90–100.
  26. Segal BM, Pogatchnik B, Henn L, et al. Pain Severity and Neuropathic Pain Symptoms in Primary Sjögren's Syndrome: A Comparison Study of Seropositive and Seronegative Sjögren's Syndrome Patients. Arthritis Care & Research. Vol. 65, No. 8, August 2013, pp 1291–1298.
  27. Mengshoel AM, Norheim KB, Omdal R. Primary Sjögren’s Syndrome: Fatigue Is an Ever-Present, Fluctuating, and Uncontrollable Lack of Energy.  2014;Aug;66(8):1227-32.
  28. Ng WF, Bowman SJ. Primary Sjögren’s Syndrome: Too Dry and Too Tired. Rheumatology. 2012;49:844–853.
  29. Tincani A, Andreoli L, Cavazzana I, et al. Novel Aspects of Sjögren’s Syndrome in 2012.  2013;Apr 4;11:93.
  30. Kittridge A, Routhouska SB, Korman NJ. Dermatologic Manifestations of Sjögren Syndrome. J Cutan Med Surg. 2011;15:8–14. 
  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.
  32. Palm O, Garen T, Berge Enger T, et al. Clinical Pulmonary Involvement in Primary Sjögren’s Syndrome: Prevalence, Quality of Life, and Mortality: A Retrospective Study Based on Registry Data. Rheumatology (Oxford). 2013;52:173–179.
  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.
  34. Fauchais AL, Ouattara B, Gondran G, et al. Articular Manifestations in Primary Sjögren’s Syndrome: Clinical Significance and Prognosis of 188 Patients. Rheumatology (Oxford). 2010;49:1164–1172.
  35. Ebert EC. Gastrointestinal and Hepatic Manifestations of Sjögren Syndrome. J Clin Gastroenterol. 2012;46:25–30.
  36. Garcia-Carrasco M, Ramos-Casals M, Rosas J, et al. Primary Sjögren’s Syndrome: Clinical and Immunological Disease Patterns in a Cohort of 400 Patients. Medicine. 2002;81:270–280.
  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.
  42. Hernández-Molina G, Leal-Alegre G, Michel-Peregrina M. The Meaning of Anti-Ro and Anti-La Antibodies in Primary Sjögren’s Syndrome. Autoimmun Rev. 2011;10:123–125.
  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.
  51. Sjögren H. Zur Kenntnis der Keratoconjunctivitis Sicca. Acta Ophthalmol. 1933;Suppl 2:1-151.
  52. Vivino FB1, Gala I, Hermann GA. Change in Final Diagnosis on Second Evaluation of Labial Minor Salivary Gland Biopsies. J Rheumatol. 2002 May;29(5):938-44.
  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.
  54. Vitali C, Bootsma H, Bowman SJ, et al. Classification Criteria for Sjögren’s Syndrome: We Actually Need to Definitively Resolve the Long Debate on the Issue. Ann Rheum Dis. 2013;72:476–478.
  55. Bowman SJ, Fox RI. Classification Criteria for Sjögren’s Syndrome: Nothing Ever Stands Still! Ann Rheum Dis. 2014;73:1–2.
  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.
  57. Vitali C, Carotti M, Salaffi F. Is it the Time to Adopt Salivary Gland Ultrasonography as an Alternative Diagnostic Tool for the Classification of Patients with Sjögren’s Syndrome? Comment on the article by Cornec et al. Arthritis Rheum. 2013;65:1950.
  58. Cornec D, Jousse-Joulin S, Saraux A, et al. Reply: to PMID 23108632. Arthritis Rheum. 2013;65:1951.
  59. Seror R, Ravaud P, Bowman SJ, et al. EULAR Sjögren’s Syndrome Disease Activity Index: Development of a Consensus Systemic Disease Activity Index for Primary Sjögren’s Syndrome. Ann Rheum Dis. 2010;69:1103-9.
  60. Seror R, Ravaud P, Mariette X, et al. EULAR Sjögren’s Syndrome Patient Reported Index (ESSPRI): Development of a Consensus Patient Index for Primary Sjögren’s Syndrome. Ann Rheum Dis. 2011;70:968-72.
  61. Ramos-Casals M, Tzioufas AG, Stone JH, et al. Treatment of Primary Sjögren Syndrome: A Systematic Review. J Am Med Assoc. 2010;304:452-60.
  62. Sall K, Stevenson OD, Mundorf TK, Reis BL. Two Multicenter, Randomized Studies of The Efficacy and Safety of Cyclosporine Ophthalmic Emulsion in Moderate to Severe Dry Eye Disease. CsA Phase 3 Study Group. Ophthalmology. 2000Apr;107(4):631-9.
  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.
  69. Iaccarino L, Rampudda M, Canova M, et al. Mycophenolate Mofetil: What is its Place in the Treatment of Autoimmune Rheumatic Diseases? Autoimmun Rev. 2007;6:190–195
  70. Atzeni F, Doria A, Carrabba M, et al. Potential Target of Infliximab in Autoimmune and Inflammatory Diseases. Autoimmun Rev. 2007;6:529–536.
  71. Cornec D, Saraux A, Devauchelle-Pensec V, et al. The Future of B Cell Targeted Therapies in Sjögren’s Syndrome. Immunotherapy. 2013;5:639-46.
  72. Gottenberg JE, Guillevin L, Lambotte O, et al. Tolerance and Short Term Efficacy of Rituximab in 43 Patients With Systemic Autoimmune Diseases. Ann Rheum Dis. 2005;64:913-20.
  73. Carubbi F, Cipriani P, Marrelli A, et al. Efficacy and Safety of Rituximab Treatment in Early Primary Sjögren’s Syndrome: A Prospective, Multi-Center, Follow-Up Study. Arthritis Res Ther. 2013;15:R172.
  74. Dass S, Bowman SJ, Vital EM, et al. Reduction of Fatigue in Sjögren Syndrome With Rituximab: Results of a Randomised, Doubleblind, Placebo-controlled Pilot Study. Ann Rheum Dis. 2008;67:1541-4.
  75. Meijer JM, Meiners PM, Vissink A, et al. Effectiveness of Rituximab Treatment in Primary Sjögren’s Syndrome: A Randomized, Double-Blind, Placebo-Controlled Trial. Arthritis Rheum. 2010;62: 960-8.
  76. Brown S, Navarro Coy N, Pitzalis C, et al. The TRACTISS Protocol: A Randomised Double Blind Placebo Controlled Clinical Trial of Anti-B-Cell Therapy in Patients With Primary Sjögren’s Syndrome. BMC Musculoskelet Disord. 2014;15:21.
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  79. Meiners PM, Vissink A, Kroese FG, et al. Abatacept Treatment Reduces Disease Activity in Early Primary Sjögren’s Syndrome (Open-Label Proof of Concept ASAP Study). Ann Rheum Dis. 2014;73:1393–1396.
  80. Adler S, Korner M, Forger F, et al. Evaluation of Histologic, Serologic, and Clinical Changes in Response to Abatacept Treatment of Primary Sjögren’s Syndrome: A Pilot Study. Arthritis Care Res (Hoboken). 2013;65:1862–1868.

Disclosures:

Dr. Noaiseh has reported no relevant relationships with any entities producing health care goods or services.

Accreditation Statement:

The University of Pittsburgh School of Medicine is accredited by the Accreditation Council for Continuing Medical Education (ACCME) to provide continuing medical education for physicians.

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.