Sjögren's Syndrome
Sjögren's (pronounced "show grins") syndrome is a chronic (life-long) disorder in which the body's immune or disease fighting system makes mistakes. It is generally described as a condition causing dry mouth and eyes but all of the body's glands that excrete sweat, saliva or oil can be affected. Named after the Swedish eye doctor, Dr. Henrik Sjögren, Sjögren's syndrome can be found in people of all ages and races, affecting women 90% of the time, usually showing up past the child-bearing years (45 to 50).
Sjögren's syndrome can occur in two ways. "Primary" Sjögren's syndrome is dry eyes and dry mouth and is not associated with a rheumatic condition. For about half the people diagnosed, a dry mouth and/or dry eyes occurs together with rheumatic conditions or connective tissue diseases like rheumatoid arthritis, systemic lupus erythematosus (lupus), scleroderma or polymyositis. In this case it is called "secondary" Sjögren's syndrome.
As yet, there is no cure for Sjögren's syndrome, but proper treatment can help relieve symptoms so that a person can live a comfortable and productive life.
DIAGNOSIS
Establishing the correct diagnosis is very important, because something can be done to manage most forms of arthritis, and most therapies work best when started early in the disease.
There are no universally recognized criteria for the diagnosis of Sjögren's syndrome. Generally a diagnosis is based on the presence of two of three features: dry eyes, dry mouth and a connective tissue disease.
Your doctor or other health care professional may do several things to determine if you have Sjögren's syndrome. A review of your medical history and a physical examination will be carried out. Your doctor will be looking for symptoms such as red, itchy eyes, swollen salivary glands, a dry cracked tongue and enlarged lymph glands in your neck. For dry eyes, a "Schirmer test" can determine the amount of tears produced by using a small strip of filter paper placed under the lower eyelid. A slit-lamp examination involves placing a drop of dye into the eye and examining the eye with a special instrument to examine areas of dryness or erosion.
A salivary function test can measure the amount of dryness in the mouth. Tests for specific blood markers can be used to determine if you have Sjögren's, although not everyone with Sjögren's will have these markers. Other tests may include lip biopsy, urine tests and chest x-rays. Although Sjögren's syndrome is not especially difficult to diagnose, it is not widely known and not widely recognized.
Signs and Symptoms
Symptoms of Sjögren's syndrome vary widely among individuals. In people who do not have any other health problems, the most common early symptom is the onset of severe dry mouth and eyes. For people who have rheumatoid arthritis or another connective tissue disease, the onset of dry eyes and mouth develops more slowly. Symptoms will vary from person to person and may seem worse at some times than at others. They can include dry mouth with a reduced amount of saliva making it difficult to chew or swallow, dry eyes, which feel "gritty" or "sandy," swollen salivary glands, dental cavities because of decreased saliva, dry nose, dry throat and lungs, dryness of the vagina, fatigue, joint pain (most often the smaller joints and usually symmetrical or affecting both sides of the body) and other problems in other parts of the body such as the blood vessels, nervous system, muscles, skin and other organs. Since Sjögren's syndrome can affect the liver and pancreas, there is a greater chance of developing cancer of the lymph tissue (although this is unusual).
It is important to note that dryness of the eyes and mouth can be a result of conditions other than Sjögren's syndrome, such as the use of certain medications, aging, and hormonal disorders.
WHAT HAPPENS
Sjögren's syndrome is called an auto immune disease. The immune system lacks the usual controls. This causes white blood cells (lymphocytes) to invade the glands of the body that produce moisture, such as the tear and salivary glands. They may eventually destroy the glands altogether. Sjögren's syndrome can also affect other parts of the body including joints, lungs, muscles, kidneys, nerves, thyroid gland, liver, pancreas, stomach and brain.
RISK FACTORS
No one knows for sure what causes Sjögren's syndrome. It is suspected that several factors are involved including infectious (viral), hormonal, genetic and stress.
WHAT TO DO ABOUT SJÖGREN'S SYNDROME
As there is yet no cure for Sjögren's syndrome, treatment is aimed at preventing damage to the eyes, mouth and other structures and reducing the effects of dryness by putting as much fluid back into your system as possible. Consistent maintenance and hygiene are important. Since Sjögren's syndrome affects people individually, your treatment plan will be based on your individual needs. If you have arthritis or another condition, you will also be following your specific treatment plan for that condition.
As there are different symptoms affecting different parts of the body, your health care team may include a rheumatologist (an arthritis specialist), an ophthalmologist (eye specialist), and your dentist in addition to your family doctor. Pharmacists can assist you in choosing products to relieve dryness.
For dry mouth:
- sip fluids throughout the day
- use sugarless gum or candies to stimulate saliva production
- try saliva substitutes or mouth coating products, which are useful for some people.
- avoid drying or irritating foods such as nuts, chocolate, strong cheeses, high acid foods, shellfish, caffeine and alcoholic beverages
To prevent cavities:
- get regular checkups
- brush immediately after eating
- floss a couple of times a day
Avoid smoking as it can be extremely harmful for the mouth, nasal tissue, eyes and lungs.
Consult your pharmacist about medications that can cause dryness, such as antihistamines and antidepressants.
DRYNESS
For dry eyes: Consult your doctor or ophthalmologist for a good quality artificial tear preparation or lubricating ointments for overnight or long-lasting relief. For more severe cases, your ophthalmologist can perform a simple technique that blocks tear drainage from the eye. This allows tears that are produced to accumulate and moisten the eyes. Moisture chamber eyeglasses, which preserve existing tears and protect the eyes from wind and draft, can also be useful.
For dry skin: A good moisturizer can alleviate the tightness of dry skin. Humidifiers are useful for increasing the moisture in the home or office. Avoid drafts from air conditioners, heaters and radiators when possible.
For vaginal dryness: Use lubricants specifically designed for vaginal dryness.
For dry nose: A number of different types of nasal preparations are available.
MEDICATIONS
Aspirin® and nonsteroidal anti-inflammatory medications can be prescribed for reducing joint swelling and stiffness, as well as muscle aches. For more serious complications, stronger medications may be recommended, such as prednisone, or the stronger immunosuppressive drugs.
EXERCISE
Exercise to keep joints and muscles flexible, such as walking or swimming, are helpful. For "secondary" Sjögren's syndrome, the type and intensity of the exercise program should be determined by the nature of the person's associated connective tissue disease.
PREGNANCY & SJOGREN'S SYNDROME
In rare instances, a certain blood marker often found in women with Sjögren's syndrome can be associated with heart problems in newborn babies. Therefore, it is important to discuss the issue of pregnancy with your doctor.
