What is Sjögren’s Syndrome?

by | Dec 28, 2021 | Eye Conditions, Eye Infections / Disease

Sjögren’s (SHOW-grins) syndrome is an autoimmune disease–that is, a disease in which the immune system turns against the body’s own cells. In Sjögren’s syndrome, the immune system targets moisture-producing glands and causes dryness in the mouth and eyes. Other parts of the body can be affected as well, resulting in a wide range of possible symptoms.  Normally, the immune system works to protect us from disease by destroying harmful invading organisms like viruses and bacteria. In the case of Sjögren’s syndrome, disease-fighting cells attack the glands that produce tears and saliva (the lacrimal and salivary glands). Damage to these glands keeps them from working properly and causes dry eyes and dry mouth. In technical terms, dry eyes are called keratoconjunctivitis sicca, or KCS, and dry mouth is called xerostomia. Your doctor may use these terms when talking to you about Sjögren’s syndrome.  The disease can affect other glands too, such as those in the stomach, pancreas, and intestines, and can cause dryness in other places that need moisture, such as the nose, throat, airways, and skin.

You might hear Sjögren’s syndrome called a rheumatic disease. A rheumatic disease causes inflammation in joints, muscles, skin, or other body tissue, and Sjögren’s can do that. The many forms of arthritis, which often involve inflammation in the joints, among other problems, are examples of rheumatic diseases. Sjögren’s is also considered a disorder of connective tissue, which is the framework of the body that supports organs and tissues (joints, muscles, and skin).

Primary versus Secondary Sjögren’s Syndrome

Sjögren’s syndrome is classified as either primary or secondary disease. Primary Sjögren’s occurs by itself, and secondary Sjögren’s occurs with another disease. Both are systemic disorders, although the symptoms in primary are more restricted.

In primary Sjögren’s syndrome, the doctor can trace the symptoms to problems with the tear and saliva glands. People with primary disease are more likely to have certain antibodies (substances that help fight a particular disease) circulating in their blood than people with secondary disease. These antibodies are called SS-A and SS-B. People with primary Sjögren’s are more likely to have antinuclear antibodies (ANAs) in their blood. ANAs are autoantibodies, which are directed against the body.

In secondary Sjögren’s syndrome, the person had an autoimmune disease like rheumatoid arthritis or lupus before Sjögren’s developed. People with this type tend to have more health problems because they have two diseases, and they are also less likely to have the antibodies associated with primary Sjögren’s.

What Are the Symptoms of Sjögren’s Syndrome?

The main symptoms are:

  • Dry eyes –Your eyes may be red and burn and itch. People say it feels like they have sand in their eyes. Also, your vision may be blurry, and bright light, especially fluorescent lighting, might bother you.
  • Dry mouth –Dry mouth feels like a mouth full of cotton. It’s difficult to swallow, speak, and taste. Your sense of smell can change, and you may develop a dry cough. Also, because you lack the protective effects of saliva, dry mouth increases your chances of developing cavities and mouth infections.
  • Both primary and secondary Sjögren’s syndrome can affect other parts of the body as well, including the skin, joints, lungs, kidneys, blood vessels, and nervous system, and cause symptoms such as
  • Dry skin
  • Skin rashes
  • Thyroid problems
  • Joint and muscle pain
  • Pneumonia
  • Vaginal dryness
  • Numbness and tingling in the extremities

When Sjögren’s affects other parts of the body, the condition is called extraglandular involvement because the problems extend beyond the tear and salivary glands. These problems are described in more detail later.

Finally, Sjögren’s can cause extreme fatigue that can seriously interfere with daily life.

What Causes Dryness in Sjögren’s Syndrome?

In the autoimmune attack that causes Sjögren’s, disease-fighting cells called lymphocytes target the glands that produce moisture–primarily the lacrimal (tear) and salivary (saliva) glands. Although no one knows exactly how damage occurs, damaged glands can no longer produce tears and saliva, and eye and mouth dryness result. When the skin, sinuses, airways, and vaginal tissues are affected, dryness occurs in those places, too.

Who Gets Sjögren’s Syndrome?

Experts believe 1 to 4 million people have the disease. Most–90 percent—are women. It can occur at any age, but it usually is diagnosed after age 40 and can affect people of all races and ethnic backgrounds. It’s rare in children, but it can occur.

What Causes Sjögren’s Syndrome?

Researchers think Sjögren’s syndrome is caused by a combination of genetic and environmental factors. Several different genes appear to be involved, but scientists are not certain exactly which ones are linked to the disease since different genes seem to play a role in different people. For example, there is one gene that predisposes Caucasians to the disease. Other genes are linked to Sjögren’s in people of Japanese, Chinese, and African American descent. Simply having one of these genes will not cause a person to develop the disease, however. Some sort of trigger must activate the immune system.  Scientists think that the trigger may be a viral or bacterial infection. It might work like this: A person who has a Sjögren’s-associated gene gets a viral infection. The virus stimulates the immune system to act, but the gene alters the attack, sending fighter cells (lymphocytes) to the eye and mouth glands. Once there, the lymphocytes attack healthy cells, causing the inflammation that damages the glands and keeps them from working properly. These fighter cells are supposed to die after their attack in a natural process called apoptosis, but in people with Sjögren’s syndrome, they continue to attack, causing further damage. Scientists think that resistance to apoptosis may be genetic.  The possibility that the endocrine and nervous systems play a role is also under investigation.

How Is Sjögren’s Syndrome Diagnosed?

The doctor will first take a detailed medical history, which includes asking questions about general health, symptoms, family medical history, alcohol consumption, smoking, or use of drugs or medications. The doctor will also do a complete physical exam to check for other signs of Sjögren’s.  You may have some tests, too. First, the doctor will want to check your eyes and mouth to see whether Sjögren’s is causing your symptoms and how severe the problem is. Then, the doctor may do other tests to see whether the disease is elsewhere in the body as well.  Common eye and mouth tests are:

  • Schirmer test–This test measures tears to see how the lacrimal gland is working. It can be done in two ways: In Schirmer I, the doctor puts thin paper strips under the lower eyelids and measures the amount of wetness on the paper after 5 minutes. People with Sjögren’s usually produce less than 8 millimeters of tears. The Schirmer II test is similar, but the doctor uses a cotton swab to stimulate a tear reflex inside the nose.
  • Staining with vital dyes (rose bengal or lissamine green)–The tests show how much damage dryness has done to the surface of the eye. The doctor puts a drop of a liquid containing a dye into the lower eye lid. These drops stain on the surface of the eye, highlighting any areas of injury.
  • Slit lamp examination–This test shows how severe the dryness is and whether the outside of the eye is inflamed. An ophthalmologist (eye specialist) uses equipment that magnifies to carefully examine the eye.
  • Mouth exam–The doctor will look in the mouth for signs of dryness and to see whether any of the major salivary glands are swollen. Signs of dryness include a dry, sticky mouth; cavities; thick saliva, or none at all; a smooth look to the tongue; redness in the mouth; dry, cracked lips; and sores at the corners of the mouth. The doctor might also try to get a sample of saliva to see how much the glands are producing and to check its quality.
  • Salivary gland biopsy of the lip–This test is the best way to find out whether dry mouth is caused by Sjögren’s syndrome. The doctor removes tiny minor salivary glands from the inside of the lower lip and examines them under the microscope. If the glands contain lymphocytes in a particular pattern, the test is positive for Sjögren’s syndrome.

Because there are many causes of dry eyes and dry mouth, the doctor will take other possible causes into account. Generally, you are considered to have definite Sjögren’s if you have dry eyes, dry mouth, and a positive lip biopsy. But the doctor may decide to do additional tests to see whether other parts of the body are affected. These tests may include:

  • Routine blood tests–The doctor will take blood samples to check blood count and blood sugar level, and to see how the liver and kidneys are working.
  • Immunological tests–These blood tests check for antibodies commonly found in the blood of people with Sjögren’s syndrome.

For example:

  • Antithyroid antibodies are created when antibodies migrate out of the salivary glands into the thyroid gland. Antithyroid antibodies cause thyroiditis (inflammation of the thyroid), a common problem in people with Sjögren’s.
  • Immunoglobulins and gamma globulins are antibodies that everyone has in their blood, but people with Sjögren’s usually have too many of them.
  • Rheumatoid factors (RFs) are found in the blood of people with rheumatoid arthritis, as well as in people with Sjögren’s. Substances known as cryoglobulins may be detected; these indicate risk of lymphoma.
  • Similarly, the presence of antinuclear antibodies (ANAs) can indicate an autoimmune disorder, including Sjögren’s.
  • Sjögren’s antibodies, called SS-A (or SS-Ro) and SS-B (or SS-La), are specific antinuclear antibodies common in people with Sjögren’s. However, you can have Sjögren’s without having these ANAs.
  • Chest x ray–Sjögren’s can cause inflammation in the lungs, so the doctor may want to take an x ray to check them.
  • Urinalysis–The doctor will probably test a sample of your urine to see how well the kidneys are working.

What Type of Doctor Diagnoses and Treats Sjögren’s Syndrome?

Because the symptoms of Sjögren’s are similar to those of many other diseases, getting a diagnosis can take time–in fact, the average time from first symptom to diagnosis ranges from 2 to 8 years. During those years, depending on the symptoms, a person might see a number of doctors, any of whom may diagnose the disease and be involved in treatment. Usually, a rheumatologist (a doctor who specializes in diseases of the joints, muscles, and bones) will coordinate treatment among a number of specialists. Other doctors who may be involved include:

  • Allergist
  • Dentist
  • Dermatologist (skin specialist)
  • Gastroenterologist (digestive disease specialist)
  • Gynecologist (women’s reproductive health specialist)
  • Neurologist (nerve and brain specialist)
  • Ophthalmologist (eye specialist)
  • Otolaryngologist (ear, nose, and throat specialist)
  • Pulmonologist (lung specialist)
  • Urologist

How Is Sjögren’s Syndrome Treated?

Treatment is different for each person, depending on what parts of the body are affected. But in all cases, the doctor will help relieve your symptoms, especially dryness. For example, you can use artificial tears to help with dry eyes and saliva stimulants and mouth lubricants for dry mouth. Treatment for dryness is described in more detail below. If you have extraglandular involvement, your doctor–or the appropriate specialist–will also treat those problems. Treatment may include nonsteroidal anti-inflammatory drugs for joint or muscle pain, saliva- and mucus-stimulating drugs for nose and throat dryness, and corticosteroids or drugs that suppress the immune system for lung, kidney, blood vessel, or nervous system problems. Hydroxychloroquine, methotrexate, and cyclophosphamide are examples of such immunosuppressants (drugs that suppress the immune system).

What Can I Do About Dry Eyes?

Artificial tears can help. They come in different thicknesses, so you may have to experiment to find the right one. Some drops contain preservatives that might irritate your eyes. Drops without preservatives don’t usually bother the eyes. Nonpreserved tears typically come in single-dose packages to prevent contamination with bacteria. At night, an eye ointment might provide more relief. Ointments are thicker than artificial tears and moisturize and protect the eye for several hours. They may blur your vision, which is why some people prefer to use them while they sleep.  Another alternative is surgery to close the tear ducts that drain tears from the eye. The surgery is called punctal occlusion. For a temporary closure, the doctor inserts collagen or silicone plugs into the ducts. Collagen plugs eventually dissolve, and silicone plugs are “permanent” until they are removed or fall out. For a longer lasting effect, the doctor can use a laser or cautery to seal the ducts.

General Tips for Eye Care

  • Don’t use artificial tears that irritate your eyes–try another brand or preparation.
  • Nonpreserved drops may be more comfortable.
  • Blink several times a minute while reading or working on the computer.
  • Protect your eyes from drafts, breezes, and wind.
  • Put a humidifier in the rooms where you spend the most time, including the bedroom, or install a humidifier in your heating and air conditioning unit.
  • Don’t smoke and stay out of smoky rooms.
  • Apply mascara only to the tips of your lashes so it doesn’t get in your eyes. If you use eyeliner or eye shadow, put it only on the skin above your lashes, not on the sensitive skin under your lashes, close to your eyes.
  • Ask your doctor whether any of your medications contribute to dryness and, if so, how to reduce that effect.