Women are nine times more likely than men to develop Sjogren's syndrome, a chronic, incurable disorder in which immune system cells attack and destroy the glands that produce tears and saliva. Sjogren's (pronounced 'show-grins') can occur at any age, but a woman is at higher risk for developing the condition after menopause.

The Sjogren's Syndrome Foundation estimates that four million Americans suffer from this disorder; many go undiagnosed.

The syndrome, named after Swedish ophthalmologist Henrik Sjogren, is an autoimmune disorder-a disorder in which your immune system attacks your body's own healthy tissues. Ordinarily, the immune system produces antibodies and cells that target such destructive material as viruses and bacteria. In the case of Sjogren's and other autoimmune diseases, autoreactive cells and autoantibodies-antibodies that turn against the part of the body they should protect-are produced.

Sjogren's syndrome may occur alone (referred to as primary Sjogren's syndrome), or it may be associated with other autoimmune/rheumatic diseases, such as rheumatoid arthritis, lupus, polymyositis and scleroderma. When Sjogren's occurs with another autoimmune disease it is called secondary Sjogren's syndrome.

The hallmark symptoms of Sjogren's are dry eyes and dry mouth. The disorder may also cause skin, nose and vaginal dryness, and it can affect other organs of the body as well, including your skin, lungs, gastrointestinal tract, liver, pancreas, kidneys, thyroid, joints, muscles, blood, blood vessels, brain, spinal cord and peripheral nerves.

 

Inadequate tear production can result in eye irritation and a feeling of having grit or sand in your eyes. The lack of saliva generally causes dry mouth (xerostomia); this can lead to difficulties with speech and swallowing food, extensive tooth decay, tooth loss, oral sores and fungal infections in the mouth. If dry mouth is troublesome at night, it can also cause sleep problems through a cycle of increased fluid intake and increased urination. The salivary glands may enlarge to produce visible swelling, particularly below the ears at the angle of the jaw; this swelling may be confused with mumps.

Another problem is that the quality of the saliva that is produced is not as good, predisposing you to problems related to oral bacteria. Saliva is the main protective factor for the upper gastrointestinal tract-the mouth, pharynx and esophagus. Salivary proteins help to prevent tooth decay, to repair wounds or ulcers in the soft mucous membrane tissues, to kill bacteria, certain fungi and some viruses, and to permit proper swallowing of food. Saliva also facilitates your ability to taste and to speak. All of these functions can be affected if you develop Sjogren's.

Salivary glands are a prime target of Sjogren's. They are composed of two parts, the acinar region or secretory end piece and the ductal region. Acinar cells are the site of fluid secretion, and they are responsible for making and secreting more than 85 percent of the proteins involved in producing saliva. Ductal cells cannot secrete fluid. In Sjogren's, autoreactive lymphocytes (a type of white blood cell) infiltrate and destroy the glandular tissue.

In severe cases, Sjogren's can damage vital organs. Symptoms vary, as this is a disease of many manifestations. The symptoms may level out, worsen or go into remission (disappear for a period of time). Some people experience only the mild symptoms of dry eyes and mouth, while others experience cycles of good health followed by severe disease. Many patients are able to treat problems symptomatically, but others must cope with blurred vision, constant eye discomfort, recurrent mouth infections, swollen parotid glands (the salivary glands near the ears), hoarseness, difficulty in swallowing and eating, bronchitis and other pulmonary disorders, debilitating fatigue and joint pain. Patients with Sjogren's syndrome also have a 40-fold increased risk of developing lymphoma, cancer of the lymphocytes.

An autoantibody to the protein SS-A/Ro, found in women with Sjogren's syndrome, can very rarely be associated with congenital heart block and neonatal lupus in newborn babies. If you have Sjogren's syndrome and plan to become pregnant, see your health care professional about testing for this marker and deciding what to do if the marker is present.