Approximately 4 million Americans suffer from Sjögren's syndrome, which was first identified in 1933 by the Swedish ophthalmologist Henrik Sjögren. About 90% of victims are women with a mean age of 50 years, although Sjögren's can strike any age group, including children (Talal 1987).
The main symptoms of Sjögren's are chronic dryness of the eyes and mouth; however, it is also associated with dryness in external genitalia as well as the ear, nose and throat area. There may also be decreased secretions in the gastrointestinal tract.
Sjögren's is an autoimmune disorder. Symptoms associated with Sjögren's are caused by the infiltration of immune-system cells (usually B and T lymphocytes) into glands responsible for secreting fluid. The disease can occur alone (primary Sjögren's) or in conjunction with other autoimmune diseases (secondary Sjögren's). Sjögren's has been associated with rheumatoid arthritis, systemic lupus erythematosus, scleroderma, and other connective tissue diseases (Talal 1987). It has also been associated with autoimmune disorders in the thymus gland; studies have shown that patients with Sjogren's may also suffer from thyroid disorders (Lazarus 2005).
Traditional treatment relies on cholinergics (ie, drugs that stimulate the parasympathetic nervous system); however, they can have significant side effects. Nutritional therapy focuses on essential fatty acids, which have been shown to modulate the immune system. Lifestyle changes are also a valuable therapeutic tool.
Although the disease rarely shortens a patient's life span, Sjögren's can have devastating effects. Ocular (eye) dryness can lead to chronic keratoconjunctivitis and corneal ulcers; oral dryness can result in severe and chronic dental decay, fissures, infections, as well as difficulty speaking and swallowing. The disease is often accompanied by depression, fatigue, and fever. Elevated liver enzymes occur in 25% of patients; liver enlargement in 33%; Raynaud's syndrome in 20%; fibromyalgia in 55%; and lymphoma in less than 5% (Carsons 1998).
Oral symptoms of Sjögren's syndrome include reduced saliva; a dry, sticky mouth; and difficulty chewing and swallowing. Decreased saliva makes the oral cavity more acidic; the lowered pH, along with the reduction in the number of antibacterial enzymes normally present in human saliva, makes tooth decay a significant problem. Dental caries is considered a potential marker for the disease (Lynge Pedersen 2005). Dry mouth can also affect speech, taste, and tolerance to dental prostheses.
Chronic dryness of the eye causes inflammatory reactions (Baudouin 2001), leaving a feeling of grittiness in the eye and intolerance to light. The roughened surface of the eye caused by the dryness causes light to scatter, resulting in blurred vision. Dry eye also renders eyes more sensitive to irritants and susceptible to infections, which can result in corneal ulcerations if left untreated. Eyelid dermatitis may be another manifestation of primary Sjögren's syndrome.
Other symptoms associated with Sjögren's include the following:
- Nasal dryness, nosebleeds, congestion, impaired taste and smell, as well as more serious conditions (eg, bronchitis and pneumonia) caused by damage to mucous glands in the nose
- Dryness in eustachian tubes, which can lead to a clogged feeling in the ear and impaired hearing
- Itchy, dry skin
- Vaginal dryness
- Nutritional malabsorption, caused by affected mucous lining of the stomach
Individuals with Sjögren's may develop neurological problems, such as impaired memory and reduced concentration (Sjögren's Syndrome Foundation). A French study linked the onset of facial palsy involving cranial nerves to Sjögren's (Rousso 2005).
Because it progresses slowly, Sjögren's syndrome is frequently misdiagnosed and left untreated (Derk 2004). Symptoms of primary Sjögren's syndrome—dryness, fatigue, pain, head and neck complaints, hoarseness, or hearing loss—can also occur as a result of medication use, anxiety and depression, or normal aging (Mahoney 2003; Mariette 2002). In a Chinese study, the average time between onset of symptoms and establishment of diagnosis was 7.8 years, indicating most cases were either improperly diagnosed or neglected (Zhang 1993).
Sjögren's is diagnosed on the basis of the presence of symptoms and specific autoantibodies. During diagnosis, the physician will ask questions to determine if a patient is suffering from dry eyes or mouth and will test the functioning of salivary and ocular glands. In addition, tests could be conducted to detect antibodies to Ro/SS-A or La/SS-B antigens. Researchers have also discovered increased levels of the antibody interleukin-18, an immunoregulatory and proinflammatory cytokine in patients with Sjögren's (Bombardieri 2004). This cytokine interferes with acetylcholine, the messenger chemical that triggers saliva production.