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Sjogren syndrome

Last updated: June 17, 2021

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Sjogren syndrome is a chronic inflammatory autoimmune disease that occurs mainly in middle-aged women. The cause of primary Sjogren syndrome is unknown, whereas secondary Sjogren syndrome is associated with underlying autoimmune diseases (e.g., rheumatoid arthritis). As the immune system mainly attacks lacrimal and salivary glands, patients typically present with xerophthalmia (dry eyes) and xerostomia (dry mouth), the combination of which is also known as sicca syndrome. The disease may also involve the skin, joints, internal organs, and nervous system. It is diagnosed via the detection of autoantibodies (anti-SSA/Ro, ANA) and salivary gland biopsy. Management focuses on supportive measures and, in more severe cases, immunosuppressants.

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

Epidemiological data refers to the US, unless otherwise specified.

Glandular symptoms [3]

Extraglandular symptoms [4][5]

The classical presentation is a middle-aged woman with dry eyes and a dry mouth, accompanied by rheumatoid arthritis or systemic lupus erythematosus

Sjogren syndrome is diagnosed based on the typical clinical features and is confirmed via detection of specific antibodies and a pathologically low tear production. Biopsy of the salivary gland is the most accurate test, but is not needed to establish the diagnosis.

Laboratory tests [6]

Eye examination

Biopsy

Ultrasound of the parotid gland

  • Findings
    • Honeycomb or cloud-like structure of the glandular parenchyma
    • Alternating hypoechoic areas and band-like hyperechoic septa and cysts

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

References:[1]

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

  1. Ng WF, Bowman SJ. Primary Sjogren's syndrome: too dry and too tired. Rheumatology (Oxford). 2010; 49 (5): p.844-853. doi: 10.1093/rheumatology/keq009 . | Open in Read by QxMD
  2. Sjögren’s Syndrome Foundation’s Clinical Practice Guidelines - Systemic Manifestations in Sjögren’s Patients. https://www.sjogrens.org/files/research/CPGSystemic.pdf. . Accessed: January 5, 2018.
  3. Segal B, Thomas W, Rogers T, et al. Prevalence, severity, and predictors of fatigue in subjects with primary Sjögren's syndrome. Arthritis Rheum. 2008; 59 (12): p.1780-1787. doi: 10.1002/art.24311 . | Open in Read by QxMD
  4. Kruszka P, O'Brian RJ. Diagnosis and management of Sjögren syndrome. Am Fam Physician. 2009; 79 (6): p.465-470.
  5. 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
  6. 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
  7. 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