Sjogren syndrome

Last updated: August 25, 2023

Summarytoggle arrow icon

Sjogren syndrome is a chronic inflammatory autoimmune disease that most commonly occurs in middle-aged women. Primary Sjogren syndrome is idiopathic; Sjogren syndrome that occurs concomitantly with another autoimmune disease (e.g., rheumatoid arthritis, systemic sclerosis) is classified as secondary Sjogren syndrome. Sjogren syndrome most commonly manifests with sicca syndrome but can also manifest with systemic symptoms such as arthritis, Raynaud phenomenon, and GI involvement as a result of lymphocytic infiltration of glandular and nonglandular organs. The diagnosis is confirmed by the detection of autoantibodies (anti-Ro/SSA or anti-La/SSB antibodies) in patients with sicca syndrome. Salivary gland biopsy is the gold standard test but is often only performed in patients with atypical presentations. Management focuses on supportive measures and, in patients with severe systemic disease, immunosuppressive therapy.

Epidemiologytoggle arrow icon

  • Sex: : > (∼ 9:1) [1]
  • Age of onset: : typically 40–60 years [1]

Epidemiological data refers to the US, unless otherwise specified.

Etiologytoggle arrow icon

Viral infections, e.g., HCV, HIV, EBV, and HTLV, are thought to be triggering factors for Sjogren syndrome; however, a causative relationship has not been established. [3]

Clinical featurestoggle arrow icon

Clinical presentation varies widely, from isolated sicca syndrome to systemic involvement. See “Complications” for other clinical presentations.

Sicca syndrome [3][4]

Xerostomia and xerophthalmia are the classic symptoms of sicca syndrome and are present in ∼ 95% of patients with Sjogren syndrome. [3][5]

Systemic disease [3][4]

Systemic symptoms are present in 50–60% of patients with Sjogren syndrome. [3]

Diagnosticstoggle arrow icon

General principles [4][6]

  • Consider Sjogren syndrome in patients with features of sicca syndrome without a known cause.
  • The following are needed to confirm the diagnosis:
  • Classification criteria can help support the diagnosis.
  • Further studies may be obtained depending on suspected organ involvement.

Laboratory studies [3][4]

Obtain routine studies and autoantibodies for all patients. Prognostic markers may be ordered by a specialist.

Assessment of xerostomia

Salivary gland function studies [5][6][7]

  • Unstimulated salivary flow (preferred)
    • Method: Saliva is collected for 5 minutes.
    • Supportive finding: salivary flow ≤ 0.1 mL/minute [6]
  • Alternative studies

Biopsy of minor labial salivary gland [3][4]

Assessment of xerophthalmia [3]

Salivary gland imaging studies [3]

May help determine salivary gland involvement and assess for complications (e.g., infections, lymphoma)

Differential diagnosestoggle arrow icon

The differential diagnoses listed here are not exhaustive.

Treatmenttoggle arrow icon

General principles [4][5][9]

  • Management should be guided by a rheumatologist and other specialists as required.
  • Symptomatic management of sicca syndrome is the mainstay of treatment.
  • For patients with active systemic disease, immunosuppressants may be considered.
  • Monitor patients for the development of complications, e.g., lymphoma, and associated autoimmune diseases.

Patients with Sjogren syndrome have an increased risk of developing MALT lymphoma.

Management of sicca syndrome [5][9]


  • All patients
    • Frequent water intake
    • Caries prophylaxis, e.g., regular dental hygienist visits, topical fluoride or chlorhexidine
    • Avoidance of irritants (e.g., coffee, alcohol) and acidic beverages (e.g., herbal tea, cola) [10]
    • Artificial saliva
      • Preferred treatment for patients without residual glandular function
      • May be considered for all patients
  • Patient with residual glandular function: stimulation of salivary flow
    • First line: nonpharmacological stimulants, e.g., sugar-free lozenges or gum, acidic candy, xylitol
    • Second line: oral muscarinic agonists
      • Usually reserved for moderate or severe salivary dysfunction because of their potential adverse effects and the lack of strong evidence supporting their benefit
      • Options: pilocarpine, cevimeline

Muscarinic agonists are contraindicated in patients with asthma, multiple sclerosis, and/or glaucoma. [11]

Xerophthalmia [5][9]

Advise all patients to maintain adequate eye lubrication and avoid dry environments (e.g., shield eyes from wind, increase indoor humidity).

Management of systemic disease [5][9]

Management depends on the specific manifestation and should always be guided by a specialist.

TNF-α inhibitors can cause serious adverse effects (e.g., serious infections, cytopenias) and increase the risk of developing non-Hodgkin lymphoma, and therefore should be avoided in patients with Sjogren syndrome. [9]

Complicationstoggle arrow icon


We list the most important complications. The selection is not exhaustive.

Referencestoggle arrow icon

  1. Brito-Zerón P, Baldini C, Bootsma H, et al. Sjögren syndrome. Nat Rev Dis Primers. 2016; 2 (1).doi: 10.1038/nrdp.2016.47 . | Open in Read by QxMD
  2. Kruszka P, O'Brian RJ. Diagnosis and management of Sjögren syndrome. Am Fam Physician. 2009; 79 (6): p.465-470.
  3. Ramos-Casals M, Brito-Zerón P, Bombardieri S, et al. EULAR recommendations for the management of Sjögren’s syndrome with topical and systemic therapies. Ann Rheum Dis. 2020; 79 (1): p.3-18.doi: 10.1136/annrheumdis-2019-216114 . | Open in Read by QxMD
  4. Shiboski CH, Shiboski SC, Seror R, et al. 2016 American College of Rheumatology/European League Against Rheumatism Classification Criteria for Primary Sjögren's Syndrome: A Consensus and Data-Driven Methodology Involving Three International Patient Cohorts.. Arthritis Rheumatol. 2017; 69 (1): p.35-45.doi: 10.1002/art.39859 . | Open in Read by QxMD
  5. Navazesh M. Methods for Collecting Saliva. Ann N Y Acad Sci. 1993; 694: p.72-77.doi: 10.1111/j.1749-6632.1993.tb18343.x . | Open in Read by QxMD
  6. Baer AN, Walitt B. Update on Sjögren Syndrome and Other Causes of Sicca in Older Adults. Rheumatic Disease Clinics of North America. 2018; 44 (3): p.419-436.doi: 10.1016/j.rdc.2018.03.002 . | Open in Read by QxMD
  7. Vivino FB, Carsons SE, Foulks G, et al. New Treatment Guidelines for Sjögren's Disease.. Rheum Dis Clin North Am. 2016; 42 (3): p.531-51.doi: 10.1016/j.rdc.2016.03.010 . | Open in Read by QxMD
  8. Rugg-Gunn AJ, Maguire A, Gordon PH, McCabe JF, Stephenson G. Comparison of Erosion of Dental Enamel by Four Drinks Using an Intra-Oral Applicance. Caries Res. 1998; 32 (5): p.337-343.doi: 10.1159/000016469 . | Open in Read by QxMD
  9. West SG. Rheumatology Secrets. Elsevier Mosby ; 2014
  10. Jung SW, Park EJ, Kim JS, et al. Renal Tubular Acidosis in Patients with Primary Sjögren's Syndrome.. Electrolyte & blood pressure : E & BP. 2017; 15 (1): p.17-22.doi: 10.5049/EBP.2017.15.1.17 . | Open in Read by QxMD
  11. Frederick B. Vivino. Sjogren's syndrome: Clinical aspects. Clinical Immunology. 2017; 182: p.48-54.doi: 10.1016/j.clim.2017.04.005 . | Open in Read by QxMD
  12. Teos LA, Alevizos I. Genetics of Sjögren's syndrome. Clinical Immunology. 2017; 182: p.41-47.doi: 10.1016/j.clim.2017.04.018 . | Open in Read by QxMD

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