Lacrisert

EYES TOO DRY TO CRY:

How Sjogrens Syndrome Makes the Body a `Desert'

Good advice from singer Johnny Ray in his old hit song "Cry." But what if you can't cry--physically can't produce tears? This is what happens to some people who have severe Sjogren's syndrome.

Sjogren's syndrome, a surprisingly common, though often undiagnosed or misdiagnosed, disorder, robs the body of moisture essential to the smooth functioning of most organs. Named for Henrick Sjogren (pronounced show-gren), a Swedish ophthalmologist, who first described the condition in 1933, the syndrome is marked by dry eyes, dry mouth, and often a connective tissue disease, such as rheumatoid arthritis or systemic lupus erythematosus.

Primary Sjogren's, or "sicca complex," as it is also called, affects about half of all Sjogren's patients and is characterized by dry eyes or dry mouth alone. When associated with a connective tissue disease, the syndrome is called secondary Sjogren's.

As many as 2 million people in this country may suffer from Sjogren's syndrome. Although men and women of any age can develop Sjogren's, women, mostly middle aged, outnumber men 9 to 1.

Sjogren's syndrome is an autoimmune disease. That is, the body's immune system has turned traitor, attacking instead of protecting the body. Lymphocytes, white blood cells that normally fight infection, infiltrate the tissues and attack normal structures.

In Sjogren's syndrome, the moisture-producing glands, such as the lacrimal (tear) and salivary glands and glands in the vaginal area, are destroyed by the invading lymphocytes. The skin, respiratory and gastrointestinal tracts, sweat glands, liver, kidneys, lungs, and thyroid glands may also be affected.

What triggers these events is unknown, but a widely held theory is that the culprit is a virus. Heredity may play a role, but some other factors are necessary for a person to develop Sjogren's.

The onset of Sjogren's syndrome is gradual, beginning when the patient is between 40 and 60. In its early stages it is difficult to diagnose, and many patients go from doctor to doctor before the problem is recognized. A constant and depressing symptom that plagues most patients is an overwhelming fatigue and feeling of malaise. As the disease progresses, the symptoms may come and go, or they may remain stable or get worse.

"I look healthy, but I don't feel it as I pull myself out of bed in the morning, stiff and sore, eyes burning and feeling gritty, my mouth so dry I can't swallow, and my nose dry and crusty," says one patient.

Primary Sjogren's is usually the more severe form of the syndrome. The chance that a patient with primary Sjogren's will later develop a connective tissue disease is slim. The reverse is more likely: About 30 percent of people with rheumatoid arthritis develop secondary Sjogren's.

Sjogren's syndrome is "life altering" rather than " life threatening." However, lack of treatment or improper treatment of dry eyes can lead to blindness. Some Sjogren's patients develop lymphoma--cancer of the lymph glands--although this is unusual.

While there is no cure for Sjogren's syndrome, relief for its major symptoms is available.

Dry Eyes

The symptoms of dry eye--or keratoconjunctivitis sicca--include dryness, itching, and a gritty feeling, as if there were something in the eye. There may be eye pain, fatigue, redness, and light sensitivity. The lids may become inflamed. In severe cases, cells from the eye surface mix with mucus to form painful sticky threads, of filaments, on the eye. Eventually, most patients lose the ability to produce tears. They can still feel emotions, but they can't cry. Low humidity in air-conditioned building or a windy or dry climate can aggravate symptoms. (Where the humidity is high, patients may not even be aware they have the disorder.)

Diagnosing Sjogren's syndrome is sometimes difficult because dry eyes can be associated with other medical conditions, such as an eye infection or vitamin A deficiency, and from use of some common medications, including decongestants, antihistamines, some tranquilizers, antidepressants, and heart and blood pressure medicines.

The mainstay of treatment for most dry eyes is artificial tears, which help lubricate the eye and prevent infection. Artificial tears must be used regularly, as directed by the doctor, not just when there is a feeling of dryness or grittiness. Available without prescription under a variety of trade names (Clerz, Lyteers, Lacril, Tearisol, Tears Plus, Hypotears), these products contain such ingredients as cellulose derivatives, dextran, polyethylene glycol, and polyvinyl alcohol.

In March 1988, the Food and Drug Administration published a standard for over-the-counter ophthalmic drug products, identifying 13 ingredients considered safe and effective for relieving dry eyes. The agency's conclusions, based on recommendations by an advisory panel of experts, are part of FDA's ongoing review of all over-the-counter drugs.

Artificial tears may differ in their viscosity (resistance to flow), their ability to stay in the eye, and in the preservatives used to prevent bacterial contamination. The thicker and longer-lasting drops can be applied less often, but may cause some blurring of vision and leave a residue on the eyelashes.

The preservatives in artificial tears may cause allergic reactions and actually aggravate dry eye in some people. Artificial tears without preservatives are also available over the counter. Two products--Relief and Refresh--come in single-dose form. A third, Unisol, does not and must be discarded after 24 hours.

Another form of relief for dry eyes is the prescription drug Lacrisert, a small pellet about the size of a grain of rice, which contains a reservoir of lubricating material. Placed between the lower eyelid and the eyeball, the pellet releases the lubricant for 6 to 12 hours. Vision may blur, and artificial tears may still be needed occasionally.

Lubrication ointments can be used in severe cases of dry eyes, but they cause significant blurring of vision. Bedtime is a good time to use such ointments. Ten ingredients were listed as safe and effective as eye lubricants in FDA's standard.

Certain drugs called mucolytics help get rid of excessive mucus that often troubles dry eye patients.

Although dry eye victims usually cannot tolerate contact lenses, in some cases a low-water-content soft lens is used as a bandage, preventing evaporation of the tear film between the lens and the eye. Artificial tears still must be used, however.

If these measures don't help, the tiny canals at the inner corner of the eyelids, which drain excess moisture from the eyes, can be temporarily or permanently blocked. This procedure, called punctal occlusion, helps keep any natural or artificial tears in the eye for a longer time.

Wearing watertight swimming goggles, wrap-around sun glasses, or special shielding on regular glasses can help reduce moisture loss and protect the eyes from the elements. Room humidifiers are also beneficial. Car vents and fans should be turned away from the patient's face.

Dry Mouth

Dry mouth, or xerostomia, is sometimes the first symptom of Sjogren's. It is caused by a lack of saliva normally produced by three major glands on each side of the face and carried via ducts to the mouth. Any or all of these glands and ducts may be involved in Sjogren's syndrome.

Saliva protects the teeth from cavity-causing bacteria, provides minerals that keep teeth hard and enzymes that aid digestion, and moistens food so that it can be swallowed. Dry mouth victims often find it difficult to chew and swallow and even to talk. Food sticks to the teeth and the inside of the mouth. There also may be a burning sensation in the mouth and throat. The voice may become hoarse and weak. Cracks develop on the tongue and lips, particularly at the corners of the mouth.

There may be persistent swelling of the salivary glands in front of the ears and under the jaw, resembling a case of the mumps. The senses of smell and taste may also diminish.

Lack of saliva can lead to dental problems, including rampant cavities and swollen gums, often resulting in tooth loss. Wearing dentures may become difficult. Candida (yeast) infections are frequent in patients with dry mouths.

Again, Sjogren's is not the only cause of dry mouth. It can be a side effect of well over 300 drugs, including antihistamines, anti-inflammatories and diuretics, and of chemotherapy, bone marrow transplantation, and radiation treatment to the head and neck. It can accompany anemia, diabetes, stress, depression, and other conditions.

Dry mouth patients are advised to drink a lot of water--particularly when eating--and avoid dry foods, spices, alcohol and tobacco. Chewing sugarless gum or sucking on sugarless hard candy helps stimulate the production of saliva. (To further help prevent cavities, Sjogren's patients should avoid highly sugared foods such as candy and gum, breath mints, cough drops, and beverages with sugar.) Foods cut into small pieces and moistened with sauces and gravies are easier to swallow.

Artificial salivas in spray form are available over the counter to help moisten the mouth. They contain ingredients such as potassium phosphate, magnesium, calcium and sodium, or sodium carboxymethyl cellulose.

Last July, FDA approved the Salitron System, a medical device that can stimulate saliva production from the glands.

Good dental hygiene is essential for the dry mouth victim, who must brush and floss more often than others. Use of a "Water-Pik" also helps keep teeth clean. At least three visits a year to the dentist are a must. Fluoride treatment and oral remineralizing solutions can replace minerals normally provided by saliva.

Connective Tissue Diseases

The major symptoms of the connective tissue diseases associated with Sjogren's include joint pain, primarily in the fingers, wrists and knees; joint swelling and stiffness, especially in the morning; pain and color change in the fingertips or toes in low temperatures; and a distinctive rash on the face.

Secondary Sjogren's usually develops after the patient has had a connective tissue disease for many months or years. Because the symptoms are less severe that those of primary Sjogren's, the disease may even go unnoticed by a patient with severe arthritis. Early diagnosis is important, however, since delaying treatment can result in complications.

Treatment of the underlying arthritic disease is the same, regardless of whether the patient also has dry eye or dry mouth symptoms. In the case of rheumatoid arthritis, drugs such as aspirin, fenoprofen, ibuprofen, indomethacin, sulindac, or gold compounds may be prescribed. Care must be taken, however, since some arthritis medications, such as the gold compounds and penicillamine, can cause dry eyes.

Other Sjogren's Symptoms

Nasal dryness and symptoms of sinusitis, the result of the decreased functioning of the moisture-producing glands in the nose and mouth, can be treated with saline sprays ( such as Ocean, Solinex, Ayr) and humidifiers at night. Nasal irrigation (washing out the nose) with normal saline often proves helpful, as well.

Dry skin and lips can be soothed with creams and lotions, which are best applied after a shower or bath while the skin is still moist.

Antifungal creams are useful in healing cracking at the angles of the cheek due to Candida infection.

Vaginal dryness, which can cause intercourse to be painful, can be reduced with K-Y jelly, Surgilube, or other sterile lubricants.

Antidepressant drugs are sometimes used to help regulate sleep patterns and treat fatigue. Care must be taken to use products that do not have a drying side effect.

In general, a balanced program of rest and mild exercise can help the Sjogren's patient. It is important for patients to pace their daily activities to avoid fatigue. When symptoms flare up, more rest may be needed. Whenever possible, sources of irritation--such as extremely cold or dry climates and exposure to wind, smoke and hot air--should be avoided.

To cover all the bases, Sjogren's patients should be under the medical supervision of several physicians: an ophthalmologist for eye care, a dentist for mouth care, and, for those with a connective tissue disease, a rheumatologist.

Research

Research into Sjogren's syndrome is under way in the United States and abroad on several fronts. Since 1983 the Dry Mouth Clinic of the National Institute of Dental Research in Bethesda, Md., has been screening dry mouth patients to determine how their Sjogren's began. Bromhexine, a cough medicine ingredient, is being tested for its ability to increase salivary and tear gland function. Other experiments include using the patient's own blood serum to create artificial tears, and a recently completed study suggests that pilocarpine, a drug used to treat glaucoma, can stimulate saliva production.

While there is still no cure for Sjogren's syndrome, today's research holds hope that those who suffer from this moisture-robbing condition can at least fine some relief. Annabel Hecht is a free-lance writer in Silver Spring, Md., specializing in health reporting.

PHOTO : Because there is no cure for their condition, victims of Sjogren's syndrome often need to

PHOTO : try a variety of ways to treat their symptoms. Room humidifiers can help relieve dry eyes,

PHOTO : one of the main complaints. Artificial tears, lubricating ointments, and other

PHOTO : medications are also available.

PHOTO : Artificial tears are the mainstay of treatment for most cases of dry eye. a major symptom

PHOTO : of Sjogren's syndrome. Although the drops are available without a prescription, they

PHOTO : should be used regularly, as directed by a doctor, not just when the eyes feel dry or

PHOTO : gritty. Lubricating ointments also are available to help lubricate the eyes and prevent

PHOTO : infection.

COPYRIGHT 1989 U.S. Government Printing Office
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