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Primary Sjögren's Syndrome

An autoimmune disease that affects the exocrine glands and causes dry mouth and dry eyes.

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Written by: Saygı Hospital Health Guide Editorial Board
Last updated:

This content has been compiled by the Saygı Hospital Health Guide Editorial Board and is periodically reviewed by a specialist physician.

References (5)

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What is Primary Sjögren's Syndrome?

Primary Sjögren's syndrome is an autoimmune disease characterised by chronic lymphocytic infiltration of the exocrine glands, particularly the salivary and lacrimal glands. The most typical features are dry mouth and dry eyes — the so-called sicca complex. The disease may also affect the joints, lungs, kidneys, nervous system and haematological system.

B-cell activation has a central role in pathogenesis. Anti-Ro/SSA and anti-La/SSB autoantibodies, polyclonal hypergammaglobulinaemia and rheumatoid-factor positivity are typical. A minor salivary-gland biopsy showing focal lymphocytic sialadenitis (focus score of 1 or more) supports the diagnosis.

Over the long term, the risk of non-Hodgkin lymphoma (particularly MALT lymphoma) is substantially higher than in the general population. Persistent salivary-gland swelling, a low C4, cryoglobulinaemia and purpura therefore require close follow-up.

Symptoms

Chronic dry mouth and swallowing difficulty
Dry eyes, burning and a gritty sensation
Recurrent swelling of the parotid gland
A marked increase in dental caries
Fatigue and joint pain
Raynaud phenomenon
Vaginal dryness
Peripheral neuropathy and a purpuric rash

Risk Factors

Female sex (a 9:1 female predominance)
Middle age and the postmenopausal period
HLA-DR3 and HLA-B8 positivity
A family history of autoimmune disease
Viral infections (EBV, HCV, HTLV-1)
Oestrogen deficiency
Exposure to environmental toxins
Coexisting autoimmune disease

When to See a Doctor?

If you experience any of the following symptoms, seek medical attention promptly:

  • Dry mouth and dry eyes lasting months
  • Recurrent swelling of the salivary glands
  • Unexplained joint pain and fatigue
  • Rapidly progressive dental decay
  • A unilateral firm mass in the parotid (suspected lymphoma)
  • A purpuric rash on the legs

Treatment Methods

01
Artificial tears and ocular lubricants
02
Salivary stimulants (pilocarpine, cevimeline)
03
Active oral care and regular dental review
04
Hydroxychloroquine for systemic features
05
Methotrexate for joint involvement
06
Rituximab and immunosuppressants for severe organ involvement
07
Lymphoma surveillance and annual follow-up
08
Lifestyle advice and support for sicca symptoms

Which Department to Visit?

You can visit our Dahiliye (İç Hastalıkları) department for these complaints. Our specialist physicians will create the most suitable treatment plan for you.

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Health Disclaimer: The information on this page is prepared for general informational purposes only. It does not replace medical diagnosis and treatment. Please consult your physician for your complaints. Saygı Hospital does not accept responsibility for actions taken based on the information on this page.