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:
- Ramos-Casals M, Brito-Zerón P,
Sisó-Almirall A, et al. Primary Sjögen Syndrome.BMJ.
2012;344:e3821.
- Mavragani CP, Moutsopoulos HM.
The Geoepidemiology of Sjögren’s Syndrome. Autoimmun
Rev. 2010;9:A305–A310.
- 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.
- 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.
- 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.
- 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.
- Amador-Patarroyo
MJ, Arbelaez JG, Mantilla RD, et al. Sjögren’s Syndrome at the Crossroad of
Polyautoimmunity. J Autoimmun. 2012;39:199–205.
- Roblin X, Helluwaert F, Bonaz B. Celiac
Disease Must Be Evaluated in Patients With Sjögren Syndrome. Arch
Int Med. 2004;vol.
164, Nov 22.
- Triantafyllopoulou A, Moutsopoulos HM.
Persistent Viral Infection in Primary Sjögren’s Syndrome: Review and
Perspectives. Clin Rev Allergy Immunol. 2007;32:210–14.
- 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.
- Kivity
S, Agmon-Levin N, et al. Autoimmunity and Environment: Infections and
Autoimmunity — Friends or Foes? Trends Immunol 2009;30:409–414.
- Mavragani CP, Moutsopoulos
HM. Sjögren’s
Syndrome. Annual Review of Pathology: Mechanisms of Disease. 2014;9:273-85.
- 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.
- 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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N, Cohen PL. The T cell in Sjögren’s Syndrome: Force Majeure, Not Spectateur. J Autoimmun. 2012;39:229–233.
- Cornec
D, Devauchelle-Pensec V, Tobón GJ, et al. B cells in Sjögren’s Syndrome: From
Pathophysiology to Diagnosis and Treatment. J
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- Mitsias DI, Kapsogeorgou
EK, Moutsopoulos HM. Sjögren’s Syndrome:
Why Autoimmune Epithelitis? 2006;Nov;12(6):523-32.
- 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.
- Routsias JG, Tzioufas AG. Autoimmune
Response and Target Autoantigens in Sjögren’s Syndrome. Eur. J Clin Investig. 2010;40:1026–38.
- 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.
- Hayashi T. Dysfunction of Lacrimal and
Salivary Glands in Sjögren’s Syndrome: Nonimmunologic Injury in Preinflammatory
Phase and Mouse Model. J. Biomed. Biotechnol. 2011;407031.
- 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.
- Lee BH, Gauna AE, Perez G, et al. Autoantibodies
Against Muscarinic Type 3 Receptor in Sjögren’s Syndrome
Inhibit Aquaporin 5 Trafficking. PLoS One. 2013;8:e53113.
- 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.
- Stevenson W, Chauhan SK, Dana R. Dry
Eye Disease: An Immune-Mediated Ocular Surface Disorder. Arch Ophthalmol. 2012;130:90–100.
- 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.
- 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.
- Ng
WF, Bowman SJ. Primary Sjögren’s Syndrome: Too Dry and Too Tired. Rheumatology. 2012;49:844–853.
- Tincani A, Andreoli
L, Cavazzana I, et al. Novel Aspects
of Sjögren’s Syndrome in 2012. 2013;Apr 4;11:93.
- Kittridge
A, Routhouska SB, Korman NJ. Dermatologic Manifestations of Sjögren Syndrome. J Cutan Med Surg. 2011;15:8–14.
- 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.
- 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.
- 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.
- 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.
- Ebert
EC. Gastrointestinal and Hepatic Manifestations of Sjögren Syndrome. J Clin Gastroenterol. 2012;46:25–30.
- 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.
- Chai
J, Logigian EL. Neurological Manifestations of Primary Sjögren’s Syndrome. Curr Opin Neurol. 2010;23:509–513.
- 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.
- Bournia
VK, Vlachoyiannopoulos PG. Subgroups of Sjögren Syndrome Patients According to
Serological Profiles. J Autoimmun. 2012;39:15e26.
- Jonsson
R, Theander E, Sjostrom B, et al. Autoantibodies Present Before Symptom Onset
in Primary Sjogen’s Syndrome. JAMA. 2013;310:1854–1855.
- Cornec D, Jamin
C, Pers JO. Sjögren's Syndrome: Where Do We Stand,
and Where Shall We Go? 2014;Jun;51:109-14.
- 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.
- 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.
- 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.
- Zintzaras E, Voulgarelis M, Moutsopoulos HM. The
Risk of Lymphoma Development in Autoimmune Diseases: A Meta-Analysis. Arch
Intern Med. 2005;165:2337–44.
- Kassan SS, Thomas TL, Moutsopoulos HM, et al.
Increased Risk of Lymphoma in Sicca Syndrome. Ann Intern Med. 1978;89:888–92.
- 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.
- 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.
- 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.
- Nezos
A, Papageorgiou A, Fragoulis G, et al. B-Cell Activating Factor Genetic
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- Sjögren H. Zur
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- Vivino
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- Brito-Zerón P, Ramos-Casals
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- 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.
- Bowman
SJ, Fox RI. Classification Criteria for Sjögren’s Syndrome: Nothing Ever Stands
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Vita S, Lorenzon G, Rossi G, et al. Salivary Gland Echography in Primary and
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- Vitali
C, Carotti M, Salaffi F. Is it the Time to Adopt Salivary Gland Ultrasonography
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(ESSPRI): Development of a Consensus Patient Index for Primary Sjögren’s
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- Ramos-Casals
M, Tzioufas AG, Stone JH, et al. Treatment of Primary Sjögren Syndrome: A
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- Sall K, Stevenson
OD, Mundorf TK, Reis BL. Two Multicenter, Randomized
Studies of The Efficacy and Safety of Cyclosporine Ophthalmic
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EK, Lindsley KB, Adyanthaya RS, et al. Treatment of Sjögren’s
Syndrome-Associated Dry Eye an Evidence-Based Review. Ophthalmology. 2011;118:1242–1252.
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S, Worthington HV, Bryan G, et al. Interventions for the Management of Dry
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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.
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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.
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S, Asfuro?lu E, Bicakcigil M, et al.
Hydroxychloroquine Improves Dry Eye Symptoms of Patients With Primary Sjögren’s
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of Hydroxychloroquine on Symptomatic Improvement in Primary Sjögren Syndrome:
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D, Saraux A, Devauchelle-Pensec V, et al. The Future of B Cell Targeted
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F, Cipriani P, Marrelli A, et al. Efficacy and Safety of Rituximab Treatment in
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JM, Meiners PM, Vissink A, et al. Effectiveness of Rituximab Treatment in
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X, Seror R, Quartuccio L, et al. Efficacy and Safety of Belimumab in Primary
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- 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.
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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.
For your credit transcript, please access our website 4
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Release Date: 12/12/2014 | Last Modified On: 12/12/2014 |
Expires: 12/14/2015