Sjogren Syndrome: Facts, Causes, Prevention & Treatments

Picture of Sjögren's syndrome

Sjogren’s (SHOW-grins) syndrome is a disorder of your immune system identified by its two most common symptoms dry eyes and a dry mouth.

Sjogren’s syndrome often accompanies other immune system disorders, such as rheumatoid arthritis and lupus. In Sjogren’s syndrome, the mucous membranes and moisture-secreting glands of your eyes and mouth are usually affected first resulting in decreased production of tears and saliva.

Although you can develop Sjogren’s syndrome at any age, most people are older than 40 at the time of diagnosis. The condition is much more common in women. Treatment focuses on relieving symptoms.

Fast Facts

  • Sjögren’s syndrome is an autoimmune condition that can occur at any age, but is most common in older women. Many patients develop Sjögren’s syndrome as a complication of another autoimmune disease, such as rheumatoid arthritis or lupus
  • Symptoms vary in type and intensity, but many people with Sjögren’s are able to live normal lives.
  • Most of the treatment for Sjögren’s syndrome is aimed at relieving symptoms of dry eyes and mouth and preventing and treating long-term complications such as infection and dental disease. Treatments often do not completely eliminate the symptoms of dryness.
  • Most patients with Sjögren’s syndrome remain healthy, but some rare complications have been described, including an increased risk for cancer of the lymph glands (lymphoma). Thus, regular medical care and follow up is important for all patients.

Causes of Sjogren syndrome

Doctors don’t know the exact cause. You may have genes that put you at risk. An infection with a bacteria or virus may be a trigger that sets the disease in motion.

For example, let’s say you have a broken gene that’s linked to Sjogren’s, and then you get an infection. Your immune system swings into action.

White blood cells normally lead the attack against the germs. But because of your faulty gene, your white blood cells target healthy cells in the glands that make saliva and tears. There’s no let-up in the fight, so your symptoms will keep going unless you get treatment.

What are risk factors for developing Sjögren’s syndrome?

The main risk factor for the development of Sjögren’s syndrome is being a member of a family that is already characterized as having autoimmune illnesses. This does not mean that it is predictable that a member of a family with known autoimmunity will develop the disease, only that is more likely than if there were no family members with known autoimmunity. Accordingly, it is likely that certain genes that are inherited from ancestors can predispose one to the development of Sjögren’s syndrome. It should also be noted that Sjögren’s syndrome can also be sporadic and occur in a person from a family with no known autoimmunity.

Symptoms of Sjögren’s syndrome

The two main symptoms of Sjogren’s syndrome are:

  • Dry eyes. Your eyes may burn, itch or feel gritty as if there’s sand in them.
  • Dry mouth. Your mouth may feel like it’s full of cotton, making it difficult to swallow or speak.

Some people with Sjogren’s syndrome also experience one or more of the following:

  • Joint pain, swelling and stiffness
  • Swollen salivary glands particularly the set located behind your jaw and in front of your ears
  • Skin rashes or dry skin
  • Vaginal dryness
  • Persistent dry cough
  • Prolonged fatigue

Sjogren’s Syndrome Diagnosis

It can be tricky to diagnose Sjogren’s syndrome, because the symptoms sometimes look a lot like some other diseases. To get clues, your doctor will give you a physical exam and may ask you questions such as:

  • Do your eyes itch or burn often?
  • Are you getting a lot of cavities in your teeth?
  • Does your mouth get dry? How about your lips?
  • Do you have stiff or painful joints?

Your doctor may ask you to get some blood tests. He’ll take some blood from your vein and send it to a lab to get checked.

The blood tests can show if you have germ-fighting proteins (antibodies) that many people with Sjogren’s have. They can also measure inflammation in your body, another sign that you have the disease.

Can Sjögren’s syndrome be Prevented?

Because Sjögren’s syndrome is felt to be inherited, there is no particular way to prevent developing the disease. However, preventing complications of Sjögren’s syndrome, such as infection, eye irritation, etc., can be achieved using the methods described above.

How is Sjögren’s syndrome Treated?

Treatment is designed to lessen the most bothersome symptoms. Dry eyes usually respond to artificial tears applied regularly during the day or to gels applied at night. Other measures, such as plugging or blocking tear ducts, can be used in more severe cases. Eye drops that reduce inflammation in the glands around the eyes, such as cyclosporine (Restasis), may be used to increase tear production. Drinking water, chewing gum, or using saliva substitutes may relieve dry mouth. Some patients benefit from using prescription medications that stimulate saliva flow, such as pilocarpine (Salagen) or cevimuline (Evoxac). If patients develop yeast infections, anti-fungal therapies may be used. Humidifiers and nasal saline irrigation may improve nasal dryness. Medications that reduce gastric acid (such as proton-pump inhibitors and H2 blockers) may lessen symptoms of acid reflux. Treatments may help relieve some of the dryness, but usually some dryness persists.

All patients should receive regular dental care to prevent cavities and tooth loss that may occur as a complication of Sjögren’s. Patients with dry eyes should see an ophthalmologist (eye doctor) regularly for signs of damage to the cornea. Patients with excessive redness and pain in the eyes should be evaluated for infections.

Hydroxychloroquine (Plaquenil), an antimalarial drug used in lupus and rheumatoid arthritis, may be helpful in some patients with Sjögren’s syndrome by reducing joint pain and rash experienced by some patients. Patients with rare but serious systemic symptoms, such as fever, rashes, abdominal pain, or lung or kidney problems, may require treatment with corticosteroids such as prednisone (Deltasone and others) and/or immunosuppressive agents like methotrexate (Rheumatrex), azathioprine (Imuran), mycophenolate (Cellcept) or cyclophosphamide(Cytoxan). In addition, researchers are evaluating rituximab (Rituxan) and other biological therapies to treat cases of Sjögren’s that affect the entire body.

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