Sjogren's Syndrome
Dr. Robert Fox
Dr. Robert Fox
Sjogrens Syndrome
Sjogrens Syndrome
Scleritis in RA
Scleritis in RA
Dry Mouth
Dry Mouth

Sjögren’s syndrome (pronounced as SHOW-grins syndrome) is a chronic autoimmune disease described by a Swedish doctor Henrik Sjögren in 1933. This is a disease of glands that secrete fluid and maintain moisture in various organs. This leads to dryness especially in eyes, mouth, skin and vagina. The disease is 9 times more common in females than males and occurs in their mid-fifties. Sjögren’s syndrome is the second most common rheumatologic disease after rheumatoid arthritis and affects about 1-4 adults per 1000. This disease can affect children too. These patients have 16-18 times higher risk of development of lymphoma (a cancer) as compared to general population. Early Sjogren’s syndrome implies disease duration of 4 years. The diagnosis is often delayed (usually more than 6 years) due to lack of awareness in people as well as doctors. Sjogren’s syndrome can be primary or associated with other rheumatologic diseases (secondary) such as rheumatoid arthritis and systemic lupus erythematosus.

Features:
Dryness, pain (body in general and joints in particular) and fatigue are main features of Sjögren’s syndrome. Dryness of mouth is due partial destruction of salivary glands and leads to significant disability. Lack of salivation leads to sticky and dry mouth and the patient
is required to drink water frequently. There is difficulty in swallowing solid food and talking for a long time. The tongue sticks to the palate and appears smooth and red. Lack of saliva also leads to dental caries, infections and loss of teeth. Dryness leads to feeling of sand or gravel in eyes. There is sticky, itchy and burning sensation in eyes which may become red intermittently. This problem worsens in summer. Abrasions and perforation with loss of vision can complicate the situation in later stages. Impaired smell sensation due to dry nose, itching and dry skin, itching and burning vagina leading to painful intercourse and dry cough due to dryness of lungs are other features of this disease. Swelling of parotid glands (mumps-like) is often painless. Joint pain with or without swelling occurs in 60-70% patients. Anxiety and depression are common. Enlargement of lymph nodes, muscle pains and weakness, skin rashes and facial pain due to nerve involvement (trigeminal neuralgia) are other features that can occur in some cases. Other rare complications of Sjögren’s syndrome include involvement of lungs, heart, kidneys, nervous system and gastrointestinal tract. Hypokalemic periodic paralysis, a peculiar dangerous condition, is often associated with Sjögren’s syndrome.
Sjögren’s syndrome is a systemic disease involving multiple organs and varies in severity from patient to patient. It can remain steady or worsen with increasing dryness and involvement internal organs.

Other causes of dryness:

  1. Ageing
  2. Prolonged use of contact lenses
  3. Anxiety and depression
  4. Fibromyalgia and chronic fatigue
  5. Drugs(over 400 drugs implicated) – atropine, morphine, codeine (used as cough-remedy), tobacco, cannabis, cocaine, some drugs used in depression, blood pressure, allergy and anxiety
  6. Viruses – HIV, Hepatitis C
  7. Radiation (for cancer) to head and neck
  8. Diabetes, high levels of lipids (cholesterol and others)
  9. Sarcoidosis, lymphoma
Investigations:
Dryness of eyes can be easily measured by placing a small strip of filter paper under your lower eyelid (Schirmer’s test).Eyes are considered dry if the paper wets less than 5 mm in 5 minutes. There are other tests too which measure dryness as well as damage to the eye. Salivary flow can be measured by sialometry. The function of salivary glands can also be assessed by isotope scan (scintigraphy) or X-ray (sialography) tests.
There are no tests that can confirm diagnosis. Blood tests such as anti-nuclear antibodies by immunofluorescence (50-80% positive), anti-SSA antibodies (50-80% positive), anti-SSB antibodies (30-50% positive) and rheumatoid factor (50-80% positive) can indicate possibility of disease in a given symptomatic patient. ESR, a marker of inflammation, is often raised. Various other tests may be required to be performed for assessing organ involvement in this disease.

Treatment:
Although there is no cure for Sjögren’s syndrome, early diagnosis and expert rheumatology management can improve various symptoms and prevent complications. No increase in mortality is reported in these patients. It is, therefore, imperative that treatment should be continued for lifetime. The disease varies from patient to patient requiring personalized management. Help from a dentist and an eye specialist is also required. Pain can usually be managed with paracetamol. Stronger pain killers may be required in some cases. Regular aerobic exercises help in alleviating fatigue. Anti-depressants are required in selected cases. In cases with organ involvement, immunosuppressant drugs are prescribed. These must be used under expert supervision. Rituximab, a new biologic agent, has shown promising results in cases of Sjögren’s syndrome. More drugs are being studied and are expected to be available in coming years.

Care of mouth:

  1. Drink sips of water regularly and maintain good hydration.
  2. Avoid frequent sugary fruit juices and carbonated drinks as many of them are acidic. Citrus fruits and drinks (acidic) and irritants (tobacco, coffee and alcohol) should also be avoided.
  3. Sugar-free gums and lozenges containing xylitol or lactoferrin increase flow of saliva.
  4. Use non-abrasive fluoride containing toothpaste. Local fluoride gels are also useful.
  5. Brush and floss after every exposure to sweets. Always brush before going to bed.
  6. Pilocarpine and cevimeline are useful in most cases with functional salivary gland. They should be used with caution in patients with asthma, heart disease, glaucoma and liver disease.
  7. Fungus infection (thrush) due to Candida albicans must be promptly treated. This infection manifests as distaste or metallic taste, burning sensation and redness of tongue. Replace old toothbrush by a new one. Consult a dentist and start treatment without delay.
  8. Visit your dentist every 4-6 months.

Care of eyes:

  1. Avoid dry surroundings such as air-conditioned rooms and heat (afternoons and summer season). Humid air helps – use humidifiers in room.
  2. Avoid blowing air into your eyes (fans). Avoid smoke. Do not rub eyes.
  3. Blink more frequently – this spreads your tear film more evenly.
  4. Wear glasses when moving out. Glasses must cover the entire eye and should not have a nasal bridge (wrap-around like swim-goggles, safety glasses or glasses with side shields). Moisture chamber glasses can also help.
  5. Try to avoid drugs that cause dryness.
  6. Artificial tear drops and ointments – Various formulations are available. Choose the one that helps you the most. Use eye drops before irritation develops. It is good to use gel at night.
  7. Punctal occlusion blocks a duct that drains tears into nostril. This can be temporary or permanent.
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