Sjogren’s syndrome is also called Sicca syndrome or keratoconjunctivitis sicca. It is a chronic autoimmune condition that targets the glands that produce moisture in the eyes, mouth, and other body parts, resulting in decreased secretions of saliva and tears. Sjogren’s syndrome occurs when the body’s immune system attacks its healthy tissues and damages them mistakenly. Sjogren’s syndrome can occur at any age but is more common in women over the age of 40–50.
Types of Sjogren’s syndrome
Sjogren’s syndrome is a triad of dry eyes, dry mouth, and rheumatoid arthritis. There are two forms of Sjogren’s syndrome–primary and secondary forms.
• Primary form: It occurs on its own and is not associated with any other autoimmune disorder.
• Secondary form: It doesn’t occur on its own and is associated with other autoimmune disorders such as systemic lupus erythematosus, rheumatoid arthritis, scleroderma, or polymyositis.
Causes of Sjogren’s syndrome
Sjogren’s syndrome is autoimmune in nature. The exact cause of its occurrence is unknown. Various factors, such as environmental, genetic, viral infections, and sex hormones, may trigger the condition.
Symptoms of Sjogren’s syndrome
Sjogren’s syndrome may affect different parts of the body, and symptoms may vary from one person to another.
The classic symptoms include:
• Dry eyes: A person may feel itchy, gritty, or burning sensations in their eyes (like sand). It may cause blurry vision or sensitivity to bright light, and irritation due to inflammation.
• Dry mouth: A person may feel a chalky taste in the mouth and experience difficulty swallowing, tasting, and speaking.
Additional symptoms include:
• Muscle and joint pain
• Fatigue
• Dry skin and skin rashes
• Vaginal dryness
• Dry throat and nasal passages
• Dry cough
• Acid reflux
• Difficulty sleeping
• Swelling around the face and neck (glands)
• Poor concentration and memory
• Shortness of breath
• Tooth decay or early tooth loss
• Numbness and weakness in the limbs
Diagnosis of Sjogren’s syndrome
As symptoms of Sjogren’s syndrome vary from one person to another, it is difficult to diagnose. However, the following are the various tests that can help to rule out Sjogren’s syndrome from other conditions:
Blood tests
Blood tests detect specific antibodies such as anti-Sjogren’s syndrome antibodies (anti-Ro or anti-SSA) and anti-Sjogren’s syndrome type-B antibodies (anti-La or anti-SSB), anti-nuclear antibodies (ANA), and rheumatoid factor. It also measures the number of different types of blood cells and any inflammatory conditions.
Antinuclear antibody (ANA) blot test
• The antinuclear antibody (ANA) test detects the presence of antinuclear antibodies that bind to the nucleus of a cell in a given sample of blood. Anti-nuclear antibodies are auto-antibodies (proteins) that react with components of the body’s normal tissues and organs and cause inflammation and infection in healthy tissues.
• A series of tests may be conducted to identify particular Sjogren’s syndrome antibodies if the test is positive.
Interpretation of ANA test results
• Positive result: If the test results are positive, it indicates the presence of antinuclear antibodies in the blood. It means that there is a greater likelihood of having Sjogren’s syndrome. If the test is positive, a healthcare provider could recommend more imaging and eye tests to confirm the diagnosis.
• Negative result: If the test results are negative, it indicates the absence of antinuclear antibodies in the blood. It means that one is less likely to have Sjogren’s syndrome. A negative ANA test does not exclude the diagnosis of Sjögren's syndrome; further tests are done to rule out and clarify the absence of ANA in the person’s blood.
Eye tests
An eye doctor (ophthalmologist) may check the dryness of your eyes using a “Schirmer tear test”. During this test, a piece of filter paper will be placed under the lower eyelid to measure the rate of tear production.
Imaging tests
Imaging tests measure the functioning of salivary glands. It includes:
• Sialogram: It is a special X-ray that measures the amount of saliva produced when a dye is injected into the salivary gland.
• Salivary scintigraphy: It is a nuclear medicine test that tracks how long it will take for a radioisotope to travel from the vein to your salivary glands.
Biopsy
A lip biopsy is performed to detect the presence of clusters of inflammatory cells, which is a histological marker of Sjogren’s syndrome. A lower lip biopsy is most commonly performed.
Treatment of Sjogren’s syndrome
Treatment of Sjogren’s syndrome depends on the symptoms and parts of the body affected. It includes medications that reduce inflammation of the eye and increase the production of tears. Using over-the-counter eye drops and sipping water more frequently may also help to manage it. In severe cases, punctal occlusion (minor surgical procedure) may be done to seal the tear ducts and provide relief from dry eyes.
Sjogren’s syndrome is a long-term condition. The prognosis of Sjogren’s syndrome depends on the symptoms and severity of the condition. There is no permanent cure. However, early diagnosis and symptomatic treatment are important to reduce the complications of Sjogren’s syndrome.