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Sjögren’s Syndrome: Tests, Causes & Treatments

Sjögren’s syndrome (SS) is an autoimmune condition, characterized by an inability to produce sufficient tears or saliva. As a result, people with Sjögren’s often experience dry eyes and mouths.

The condition commonly co-occurs with other autoimmune conditions, such as lupus or rheumatic disease. It can be treated with eye drops, prescription medications, and surgery.

What Is Sjögren’s Syndrome? 

Sjögren’s syndrome is a systemic autoimmune responsible for excessive dryness of the eyes and mouth. 

It comes in both primary and secondary forms. The primary form is mainly an autoimmune disorder, while the secondary form is triggered by a preexisting condition like rheumatoid arthritis or lupus. 

Though dryness of the eyes and mouth are the disorder’s main symptoms, severe cases can result in more debilitating symptoms. Secondary Sjögren’s occurs most often in patients with rheumatoid arthritis at a rate of about 20 percent.

Though symptoms of the syndrome appeared in earlier literature, the condition was first formally described by Henrik Sjögren in the 1930s.

Symptoms

Various signs and symptoms comprise Sjögren’s syndrome. The disorder is mainly characterized by issues with the secretory glands

Lymphocytes, a type of white blood cell, enter the glands and are mistakenly attacked by the immune system. Since these cells are attacked and destroyed over time, the body houses fewer of them, and the immune system is hampered. As a result, Sjögren’s causes an increased risk of developing lymph tumors like lymphoma. 

Causes of Sjögren’s Syndrome

The exact causes of Sjögren’s syndrome are not completely understood. It is believed genetics, hormones, and environment all play a role. It is possible to develop Sjögren’s syndrome independently or as a result of other connective tissue disorders. 

Risk Factors

Several factors influence the likelihood of developing Sjögren’s syndrome:

  • Age: Sjögren’s syndrome typically affects people over 40, but it can also affect children.
  • Sex: Compared to men, women have a 10 times greater chance of developing Sjögren’s syndrome. It’s thought estrogen levels may play a role in the development of the disease.
  • Rheumatic disease: People with rheumatic diseases, such as lupus or rheumatoid arthritis, are more likely to develop Sjögren’s syndrome. 

Complications

While dryness of the eyes and mouth are the most common symptoms of Sjogren’s syndrome, they are not the only areas of the body affected. Known as sicca syndrome, this phenomenon of dryness can extend further into the oral cavity, larynx, pharynx as well as the vaginal cavity. 

Further debilitating symptoms can develop in severe cases of Sjögren’s syndrome. These include the following:

  • Neurological problems: Irregular psychiatric issues, dementia, trouble with mobility, and dementia are possible.
  • Muscle issues: These can include muscular weakness and musculoskeletal problems. 
  • Organ problems: These may include kidney issues like nephrogenic diabetes, or lung problems, which cause respiratory issues. 
  • Cardiovascular issues: Women with primary Sjögren’s syndrome are also twice as likely to develop hypertension, which places them at increased risk for cardiovascular problems. 

Life expectancy normally remains unchanged for patients without additional autoimmune disorders. It might fall if additional health problems co-occur with Sjögren’s syndrome. 

How Is Sjögren’s Syndrome Diagnosed?

Since there is no single progression of Sjögren’s syndrome, it can be difficult for both patients and physicians to identify. It is usually undiagnosed until later in life because the symptoms don’t show at all or are attributed to something else, like worsening vision or skin issues. 

Biopsies from moisture-producing glands, and blood tests are used for prognosis. 

Tests for Sjögren’s Syndrome

A blood test can be given to determine the presence of specific antibodies that are common in Sjögren’s syndrome. Doctors may also use blood tests to rule out other autoimmune conditions.

Some practitioners use a lip biopsy to diagnose the disorder. If inflammatory cells around the salivary gland are found, it can be a sign of Sjögren’s.

Your doctor might also perform the following tests:

  • Urine tests: These are used to identify antibodies associated with Sjögren’s syndrome.
  • Schirmer’s test: This test measures the number of tears your tear glands produce to keep your eyes moist.
  • Ocular surface staining: With this test, a doctor examines the eye’s surface for damage and dryness.
  • Rose bengal staining: This is a staining test that checks for abnormalities in the lacrimal glands. 

How to Prevent Dry Eye & Other SS Symptoms

Follow these tips to prevent dry eye and other Sjögren’s syndrome symptoms:

  • Drink more water.
  • Use over-the-counter lubricating eye drops, such as artificial tears.
  • Avoid fans and air conditioning that blows air into the face.
  • Wear wraparound sunglasses to protect eyes from the elements.
  • Suck on candies or chew gum to stimulate saliva production.
  • Use petroleum jelly or another lubricating agent on your lips.
  • Apply lotion regularly.
  • Use vaginal moisturizer or lubricants as needed.

Treatment for Sjögren’s Syndrome

There is no one-size-fits-all treatment for Sjögren’s syndrome, as it depends on the extent and severity of your symptoms. 

Medications

Medication may be prescribed to reduce eye inflammation, increase saliva production, or treat specific symptoms, such as fungal growth or inflammation throughout the body. In some cases, immunosuppressants or corticosteroids may be prescribed. Anti-inflammatory or pain medication may be used to address joint pain and other discomfort.

Sjögren’s dry eye is commonly treated with the following prescription products:

  • Restasis: This medication reduces inflammation that disrupts tear production with chronic dry eye.
  • Xiidra: This is an FDA-approved medication for dry eye.
  • CEQUA: This topical treatment stimulates tear production in patients with dry eye.
  • Corticosteroids (cortisone): These drops may be prescribed to treat acute episodes of inflammation in dry eyes. To avoid possible complications such as increased intraocular pressure, glaucoma, cataracts, and infection, corticosteroids should be used in limited amounts and for a limited time.

Surgery

If your dry eye symptoms are severe, your doctor may recommend punctal occlusion surgery. Small silicon or collagen plugs are inserted into the tear ducts to prevent tears from draining from the eyes, thereby reducing dryness.

References

  1. Sjögren’s Syndrome: A Systemic Autoimmune Disease. (June 2021). Clinical and Experimental Medicine.

  2. Evaluation of the Oral Component of Sjögren’s Syndrome: An Overview. (July–August 2016). Journal of International Society of Preventive & Community Dentistry.

  3. Predicting the Risk for Lymphoma Development in Sjogren Syndrome. (June 2016). Medicine.

  4. Reviewing Primary Sjögren’s Syndrome: Beyond the Dryness – From Pathophysiology to Diagnosis and Treatment. (February 2017). International Journal of Medical Sciences.

  5. Cardiovascular Risk Factors in Women With Primary Sjögren’s Syndrome: United Kingdom Primary Sjögren’s Syndrome Registry Results. (May 2014). Arthritis Care & Research.

  6. Pathogenetic Mechanisms in the Initiation and Perpetuation of Sjögren’s Syndrome. (August 2010). Nature Reviews Rheumatology.

  7. Neurological Complications of Sjögren’s Syndrome: Diagnosis and Management. (October 2017). Current Treatment Options in Rheumatology.

  8. Sex Differences in Sjögren’s Syndrome: A Comprehensive Review of Immune Mechanisms. (November 2015). Biology of Sex Differences.

  9. Rate, Risk Factors and Causes of Mortality in Patients With Sjögren’s Syndrome: A Systematic Review and Meta-Analysis of Cohort Studies. (March 2016). Rheumatology.

  10. Quality of Life and Psychological Status in Patients With Primary Sjögren’s Syndrome and Sicca Symptoms Without Autoimmune Features. (2006). Arthritis Care & Research.

  11. Sjögren’s Syndrome: Still Not Fully Understood Disease. (February 2015). Rheumatology International.

  12. Punctal Plugs Versus Artificial Tears for Treating Primary Sjögren’s Syndrome With Keratoconjunctivitis SICCA: A Comparative Observation of Their Effects on Visual Function. (October 2013). Rheumatology International.

Last Updated October 4, 2022

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