Sjögren's Syndrome - Primary and/or Secondary

In Sjögren’s syndrome, changes occur in the immune system–the body’s defense against disease. In Sjögren’s, the immune system lacks the usual controls. This causes white blood cells to invade glands in the body that produce moisture, such as the tear and salivary glands, and the Bartholins glands in the vagina. They can destroy the glands and cause them to stop producing moisture.

Sjögren’s Syndrome can occur on its own as a primary condition or as a secondary disease to other autoimmune conditions (lupus, rheumatoid arthritis, scleroderma, fibromyalgia, etc.). In Primary Sjögren’s Syndrome, the disease occurs without the presence of another autoimmune condition. Secondary Sjögren’s, on the other hand, is accompanied by another autoimmune condition and accounts for approximately 50% of those with Sjögren’s Syndrome. 25 % of those patients with Systemic Lupus Erythematosus will also develop Sögren’s Syndrome as an “overlapping” disorder.

Sjögren’s is characterized by dry eyes, dry mouth, swollen lymph glands, dry nose and arthritis (which can be deforming). In addition to affecting the eyes, mouth, glands, nose and joints, Sjögren’s can also involve the sinuses, ears, skin, vagina, kidneys, GI tract, blood vessels, lungs, liver, pancreas, and brain. Generalized symptoms of the condition include fatigue, loss of appetite, low grade fever, weight loss, muscular weakness, and pain. The many symptoms and manifestations of Sjögren’s Syndrome are located below.

Those living with Sjögren’s Syndrome should enlist the services of a knowledgeable rheumatologist, dentist, ophthalmologist, and if necessary an otolaryngologist. There are many treatments available to treat Sjögren’s Syndrome. These include Salagen, NSAIDs, Plaquenil, Prednisone and other corticosteroids, artificial tear and saliva products, moisturizing/lubricating products, and the use of exercise to help keep joints and muscles flexible and strong..

Sjögren’s syndrome can also cause problems in other parts of the body, including the joints, lungs, muscles, kidneys, nerves, thyroid gland, liver, pancreas, stomach, and brain. The causes of this condition are not known. There is some evidence that viral infections, heredity, and hormones may in some way contribute to Sjögren’s syndrome. Sjögren’s syndrome can affect people of any race and any age. It usually affects women.

SYMPTOMS

In people with no other health problems, the most common early symptom is the onset of severe dry mouth and eyes. In people with rheumatoid arthritis or related conditions such as lupus, dry eyes and mouth develop more slowly. In this case, Sjögren’s may be difficult to diagnose.

Sjögren’s syndrome affects everyone differently. You may not have every symptom listed here, and you may have only minor problems with those you do have. The symptoms may seem worse at some times than at others.

Symptoms include:

(1) Dry mouth

The mouth normally contains saliva, which aids chewing and swallowing. In people with Sjögren’s syndrome, the amount of saliva is much less. This makes chewing, swallowing, and speaking difficult. It may also cause a decreased sense of taste.

(2) Dry eyes

Your eyes may feel dry, “gritty,” or “sandy.” They may burn and look red. A thick substance may accumulate in the inner corner of your eyes while you sleep. Your eyes may be more sensitive to sunlight. If not properly treated, Sjögren’s syndrome can lead to ulcers of the cornea (the clear covering of the eyeball}. On rare occasions, this can cause blindness.

(3) Swollen salivary glands

There are three set of glands that produce saliva. They’re located under your tongue, in the cheeks in front of your ears, and in the back of your mouth. They may feel swollen and tender. This may occur along with a fever. This affects about one-half of people with the disorder. Dental cavities This is a common problem that results from a dry mouth. Saliva fights bacteria and defends against cavities. Because you have decreased saliva, your teeth may develop cavities more easily. Dry nose and throat This may make your throat feel dry and tickly. You may have a dry cough and hoarseness. It can also lead to pneumonia, bronchitis, and ear problems

•loss of smell

•loss of taste

•rhinitis

•increased allergic reactions to airborne allergens

•frequent sinusitis

•nosebleeds

•atrophy of the nasal mucosa glands

•itching

•burning

•increased sneezing

(4) Dryness of the vagina

This can cause painful intercourse for women with Sjögren’s syndrome.

(5) Ears and Eustachian Tubes Symptoms

•increased ear pressure

•dulled hearing

•feeling of ear fullness

•dizziness or vertigo

•itching of the external ear canal

•ear dryness

•frequent ear infections

•ear fluid build-up

(6) Fatigue

Fatigue is a common complaint. You may get easily exhausted and feel tired and worn out.

(7) Gastrointestinal Tract (includes liver, pancreas, intestines, stomach, and esophagus)

•difficulty swallowing

•decreased motility of the esophagus

•dry stomach (also known as atrophic gastritis)

•increased gastroenteritis

•chronic hepatitis

•acute pancreatitis

•celiac disease

•biliary cirrhosis

•gastric achlorhydria

(8) Kidney symptoms

•kidney atrophy

•kidney fibrosis

•renal tube defects

•nephrogenic diabetes

(9) Lung symptoms

•scarring of lung tissue

•dry lungs (also known as bronchitis sicca)

•shortness of breath

•pneumonia and pneumonitis

•fibrosis

•infiltrates

•interstitial lung disease

(10) Nervous system symptoms

•peripheral neuropathy

•polymyositis

•confusion

•dizziness or vertigo

•myopathy

•cranial and trigeminal neuropathy

•cerebral vasculitis

•memory loss

•seizures

•multiple sclerosis type symptoms

(11) Vascular system symptoms

•hyperviscosity syndrome (thick blood)

•cryoglobulinemia

•cutaneous vasculitis

•vasculitis of the small vessels

•vasculitis of the medium vessels

•purpura hemorrhages

(12) Raynaud’s phenomenon

(13) Other problems

Sjögren’s syndrome can affect other parts of the body, such as blood vessels, the nervous system, muscles, skin, and other organs. This can lead to muscle weakness, confusion and memory problems, dry skin, and feelings of numbness and tingling.

Sjögren’s syndrome can also affect the liver and pancreas. When it does, there is a greater chance for developing cancer of the lymph tissue. Although this is unusual, it is one reason why medical exams and continued follow-up are important.

DIAGNOSIS

Your doctor may do several things to find out if you have Sjören’s syndrome. These include:

(1) Physical examination

Your doctor will ask you to describe your symptoms, and will look for other symptoms, such as red, itchy eyes; swollen salivary glands; a dry, cracked tongue; and enlarged lymph glands in your neck.

(2) Blood tests

Tests for specific blood markers can determine if you have Sjögren’s syndrome. However, not everyone with Sjögren’s has these markers.

(3) Schirmer test

This helps determine how dry your eyes may be. It involves placing a small piece of filter paper under the lower eyelid to measure the amount of tears your eyes produce.

(4) Slit-lamp examination

This is a more accurate way to find out if your eyes are dry. In this test, the doctor puts a drop of dye into your eye and examines the eye with a special instrument called a slit lamp. The dye will stain dry or eroded areas of the eye. This test is often done by an ophthalmologist (eye doctor}.

(5) Lip biopsy

In this test, the doctor removes a few salivary glands from inside your lip. The tissue is examined under a microscope. The appearance of the tissue helps determine if you have Sjögren’s syndrome.

(6) Salivary function tests

These measure the actual amount of saliva you produce, to help determine if you have Sjögren’s.

(7) Urine tests

These may be done to test your kidney function. Chest X-ray This can help detect changes in your lungs.

SJÖGREN’S SYNDROME LABORATORY TESTS

Laboratory and test result findings in Sjögren’s Syndrome include:

•elevated sed rate

•positive HLAB8DR3

•anemia

•positive HLADR4

•leukopenia

•positive Schirmer test

•hypergammaglobulin

•positive Slit-Lamp examination

•positive RA factor

•positive salivary gland biopsy (also called a lip biopsy)

•anti-salivary duct antibodies (SSA (Ro) and/or SSB (La))

•positive salivary gland function test results

•positive urine tests results

•anti-nuclear antibodies (positive ANA)

TREATMENTS

As yet, there is no cure for Sjögren’s syndrome. But proper treatment can help relieve symptoms so you can live a comfortable and productive life.

The main goal of treatment is to relieve discomfort and lessen the effects of the dryness. Since Sjögren’s syndrome affects everyone differently, your treatment plan will be based on your specific needs.

Your treatment may include different ways to relieve your symptoms, such as those listed below. If you have arthritis or another condition, you will also want to follow specific treatment for that condition.

See your family doctor and your dentist regularly. Since Sjögren’s syndrome can affect many parts of the body, regular checkups can help detect and prevent future problems. You may also need regular check ups with an arthritis specialist) and an eye specialist.

(1) For dry mouth

•Sip fluids throughout the day.

•Use sugar-free gum or candies to stimulate saliva production.

•Try saliva substitutes or mouth coating products. They may be useful in some people, and are available without a prescription.

(2) To prevent dental cavities:

•Have frequent dental checkups.

•Use mouth rinses that contain fluoride.

•Brush and floss your teeth regularly.

•Use sugar-free products.

(3) For dry eyes

•Use artificial tears or eye drops to help relieve the discomfort of dry eyes. Use preservative-free products, if you apply the drops more than four times per day.

•Try lubricating ointments or small, long-acting pellets for overnight or long-lasting relief.

•Your ophthalmologist may recommend a simple operation that blocks tear drainage from your eye.

(4) For dry skin

•Use moisturizing lotions for sensitive skin.

•Avoid drafts from air conditioners, heaters, and radiators, when possible.

•Use a humidifier in your house and at work.

(5) For vaginal dryness

•Use lubricants made specifically to help vaginal dryness. Do not use petroleum jelly.

MEDICATIONS

Aspirin and nonsteroidal anti-inflammatory drugs (NSAIDs) help reduce joint swelling and stiffness, as well as muscle aches. If you have serious complications, your doctor may recommend stronger medicines.

EXERCISE

Mild exercise, such as walking or swimming, can help keep joints and muscles flexible. Exercise may also protect against further joint damage.

A NOTE ABOUT PREGNANCY

A certain blood marker often found in women with Sjögren’s syndrome can, very rarely, be associated with heart problems in newborn babies. If you’re a woman with Sjögren’s syndrome who is planning to become pregnant, see your doctor about testing for this marker. If it is present, ask your doctor whether pregnancy is advisable. If you do become pregnant, you and your doctor can work out the best plan to manage the situation.

IMPORTANT NOTES

It must be noted no two sufferers will have the same symptoms and most sufferers will never have many of the symptoms listed above.

Sjögren’s syndrome is generally not life-threatening. The outlook for people with this condition is usually good. Dryness, however, may last for the rest of your life. By using artificial moisture and practicing good oral hygiene, you can help prevent serious problems.

If you have Sjögren’s syndrome and a rheumatic disease, make sure you follow your doctor’s complete treatment program.

Support and more information – please contact your local Lupus Association for more information about Sjögren’s Syndrome.