Sjogren Syndrome
- PMID: 28613703
- Bookshelf ID: NBK431049
Sjogren Syndrome
Excerpt
In the early 1900s, Swedish physician Henrik Sjögren (SHOW-gren) first described a group of women whose chronic arthritis was accompanied by dry eyes and dry mouth. Today, Rheumatologists know more about the syndrome that is named for Sjögren and—most significantly for patients—can offer advice about how to live with it.
Primary Sjogren syndrome is a systemic autoimmune disorder most commonly presenting with sicca symptoms. Sicca refers to dryness most often involving the eyes and mouth due to inflammation and resultant pathology of the lacrimal and salivary glands. Up to one-half of affected individuals also develop extra-glandular involvement implying the occurrence of signs and symptoms in organs distinct from the salivary and lacrimal glands including the joints, skin, lungs, gastrointestinal (GI) tract, nervous system, and kidneys. Sjogren syndrome frequently occurs in conjunction with other autoimmune disorders including rheumatoid arthritis (RA) and systemic lupus erythematosus (SLE). In this setting, authors will refer to it as secondary Sjogren or Sjogren-overlap syndrome. Therapies are directed toward replacing moisture at affected glandular sites and suppressing the autoimmune response locally as well as systemically.
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Conflict of interest statement
 
 
Sections
- Continuing Education Activity
- Introduction
- Etiology
- Epidemiology
- Pathophysiology
- Histopathology
- History and Physical
- Evaluation
- Treatment / Management
- Differential Diagnosis
- Prognosis
- Complications
- Deterrence and Patient Education
- Pearls and Other Issues
- Enhancing Healthcare Team Outcomes
- Review Questions
- References
References
- 
    - Lessard CJ, Li H, Adrianto I, Ice JA, Rasmussen A, Grundahl KM, Kelly JA, Dozmorov MG, Miceli-Richard C, Bowman S, Lester S, Eriksson P, Eloranta ML, Brun JG, Gøransson LG, Harboe E, Guthridge JM, Kaufman KM, Kvarnström M, Jazebi H, Cunninghame Graham DS, Grandits ME, Nazmul-Hossain AN, Patel K, Adler AJ, Maier-Moore JS, Farris AD, Brennan MT, Lessard JA, Chodosh J, Gopalakrishnan R, Hefner KS, Houston GD, Huang AJ, Hughes PJ, Lewis DM, Radfar L, Rohrer MD, Stone DU, Wren JD, Vyse TJ, Gaffney PM, James JA, Omdal R, Wahren-Herlenius M, Illei GG, Witte T, Jonsson R, Rischmueller M, Rönnblom L, Nordmark G, Ng WF, UK Primary Sjögren's Syndrome Registry. Mariette X, Anaya JM, Rhodus NL, Segal BM, Scofield RH, Montgomery CG, Harley JB, Sivils KL. Variants at multiple loci implicated in both innate and adaptive immune responses are associated with Sjögren's syndrome. Nat Genet. 2013 Nov;45(11):1284-92. - PMC - PubMed
 
- 
    - Baer AN, Akpek EK, Alevizos I, 18-21 April 2018, Washington, DC, USA 14th International Symposium on Sjögren's Syndrome. Clin Exp Rheumatol. 2018 May-Jun;36 Suppl 112(3):241-255. - PubMed
 
- 
    - Kroese FGM, Haacke EA, Bombardieri M. The role of salivary gland histopathology in primary Sjögren's syndrome: promises and pitfalls. Clin Exp Rheumatol. 2018 May-Jun;36 Suppl 112(3):222-233. - PubMed
 
- 
    - Björk A, Mofors J, Wahren-Herlenius M. Environmental factors in the pathogenesis of primary Sjögren's syndrome. J Intern Med. 2020 May;287(5):475-492. - PubMed
 
- 
    - Baldini C, Fulvio G, La Rocca G, Ferro F. Update on the pathophysiology and treatment of primary Sjögren syndrome. Nat Rev Rheumatol. 2024 Aug;20(8):473-491. - PubMed
 
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