"Ultimately, a diagnosis is dependent upon a combination of physical findings, blood tests, the symptoms the person presents, and potential additional studies."
Defining Sjogren's Syndrome
Sjogren's syndrome is a chronic disorder first described by Swedish eye doctor Henrick Sjogren which causes a lack of sufficient moisture production in specific glands in a person's body. Sjogren's syndrome is an autoimmune disease that occurs when a person's immune system attacks and destroys their moisture-producing glands. The glands affected include the person's tear and salivary glands, although their bowel, lungs and additional organs may sometimes also be affected.
Autoimmune diseases such as Sjogren's syndrome involve production of antibodies that work against tissues in the person's body. In Sjogren's syndrome, the disease presents inflammation of the person's glands which produce tears, leading to a decrease in production of water for tears and a resulting dryness of the person's eyes. An inflammation of the person's glands which produce saliva leads to both dry mouth and lips. When gland inflammation resulting in both dry eyes and mouth that is not associated with another connective tissue disease occurs, it is referred to as, 'Primary Sjogren's Syndrome.' When these symptoms are associated with a connective tissue disease such as systemic lupus reythematosus, scleroderma, or rheumatoid arthritis, it is referred to as, 'Secondary Sjogren's Syndrome.'
Sjogren's syndrome has the potential to affect persons of any age group, although the symptoms of the disorder commonly appear in persons between the ages of forty-five and fifty-five. Greater than ninety-percent of those affected are women. There are approximately four-million persons in America who are affected by Sjogren's syndrome; half of them also have an associated connective tissue disease such as rheumatoid arthritis or lupus.
Causes of Sjogren's Syndrome
Medical science does not know the exact causes of Sjogren's syndrome, although there is support behind the theory that it is genetically caused. Sjogren's syndrome is found more commonly in families whose members also have additional autoimmune diseases. Usually, a person's immune system protects them from invading bacteria, viruses and additional foreign agents that enter their body. Persons with Sjogren's disease have abnormal proteins in their bloodstream which suggests that their immune system is reacting against their own tissues, producing the symptoms of Sjogren's syndrome. In Sjogren's syndrome, the person's immune system initiates an inflammatory response when there is a lack of foreign substances to fight, causing their white blood cells to both attack and destroy particular moisture-producing glands in their body. Research suggests that along with genetic factors, potential viral infections that are as yet unidentified could predispose some people to development of this syndrome.
Symptoms of Sjogren's Syndrome
The symptoms of Sjogren's syndrome are varied and may involve different parts of the person's body. Symptoms of the disease can affect the person's eyes, mouth, muscles, and vaginal function. Many of the symptoms are associated with a lack of moisture and the resulting dryness caused by the effects Sjogren's syndrome has on the person's glands. The syndrome can also affect additional organs in the person's body.
Tear glands that are inflamed due to Sjogren's and become dry may lead to eye irritation, a decrease in tear production, infection, abrasion of the dome of the person's eye, and a, 'gritty,' sensation. Should the person's salivary glands become inflamed, they can experience swallowing difficulties, dry mouth, gum disease, dental decay, mouth sores or swelling, infection or stones of their Parotid gland inside of their cheeks, as well as dry lips.
Less commonly, other glands in a person's body can be affected by Sjogren's syndrome. One potential involves the lining of the person's breathing passage, which may lead to lung infections. Another involves vaginal dryness, which can lead to recurrent vaginal infections, as well as painful intercourse.
Symptoms not involving a person's glands associated with Sjogren's disease can include Raynaud's phenomenon, lymph-node enlargement, joint pain or inflammation, as well as nerve, kidney, and muscle disease. Rarely, a person may experience a complication involving inflammation of their blood vessels referred to as, 'Vasculitis,' that may damage tissues in their body which are supplied by the blood vessels affected. There is also a common disease that is associated with Sjogren's syndrome on occasion; an autoimmune disease known as, 'Hashimoto's Thyroiditis,' that can lead to abnormal thyroid hormone levels in person's who are affected by it.
On rare occasion, Primary Biliary Cirrhosis, an immune disease of the liver leading to scarring of the liver, has been associated with Sjogren's syndrome. A small number of person's with Sjogren's syndrome develop lymph gland cancer after several years. More commonly, persons with Sjogren's syndrome develop heartburn, difficulties with swallowing, and GERD. Some persons with the syndrome experience episodes of swelling in their saliva glands around their face; others experience numbness, weakness, or tingling in affected areas. The following is a list of potential symptoms associated with Sjogren's syndrome:
Diagnosing Sjogren's Syndrome
Diagnosing Sjogren's syndrome involves detection of features of dryness in the person's eyes and mouth. Dryness in the person's eyes can be detected in a doctor's office through a test that examines the eye's ability to dampen a test strip of paper which is placed underneath the person's eyelid; this test is referred to as the, 'Shirmer's Test.' Radiologic Nuclear Medicine salivary scans can be used to detect salivary glands that have become enlarged or hardened. The amount of saliva a person produces can be measured through salivary flow testing. A diagnosis is supported through a biopsy of salivary gland tissue. Glands in the person's lower lip are many times used to obtain a biopsy of tissue that will assist in a diagnosis of Sjogren's syndrome.
Persons with Sjogren's syndrome commonly produce a number of antibodies against various tissues in their body, referred to as, 'Auto-antibodies.' These auto-antibodies can be detected through blood tests, and may include auto-antibodies that are typical of Sjorgen's disease such as anti-nuclear antibodies, rheumatoid factor, and anti-SSA and SSB antibodies. Thyroid antibodies may also be present in the person's bloodstream.
Ultimately, a diagnosis is dependent upon a combination of physical findings, blood tests, the symptoms the person presents, and potential additional studies. Dry eyes and mouth alone could be early symptoms of the syndrome, yet require more investigation because these symptoms can be caused by other conditions or medications. Secondary Sjogren's syndrome is commonly diagnosed when a person has an established autoimmune disease as well as extreme dry mouth and eyes. Once a person has a diagnosis of an autoimmune disease other than Sjogren's syndrome and presents symptoms of dry eyes and mouth, a lip biopsy is rarely required.
Treating Sjogren's Syndrome
There is currently no cure for Sjogren's syndrome. Treatment focuses on relief of the symptoms affecting particular areas of the body. Dry eyes are treated with artificial tears during the day, and eye-lubricating ointments at night. An ophthalmologist may plug the person's tear duct to help tears cover their eye longer. The use of Cyclosporine eye drops may reduce the inflammation associated with Sjogren's syndrome and tear glands, improving their function.
Dry mouth associated with Sjogren's syndrome can be treated through drinking plenty of fluids, pursuing proper dental care, and use of a humidifier. Stimulation of the salivary glands by chewing gum, sucking on lemon drops or ice, or using glycerin swabs can help. Using mouthwash, vitamin E oil, or other forms of over-the-counter products may also help. Should an oral infection occur, it is important to deal with the infection as promptly as possible to prevent further complications that could become severe. There are also prescription medications available to assist with dry mouth such as Cevimeline, or Pilocarpine, although persons with asthma, glaucoma, or some heart diseases should not use these medications.
For nasal dryness, there are a number of nasal sprays available on an over-the-counter basis. These nasal sprays are often variations of saltwater sprays (saline) that can help to relieve dryness in the person's nasal passages. Nasal infections should be promptly dealt with through a doctor.
There are vaginal lubricants available on an over-the-counter basis for women who experience vaginal dryness. These lubricants may ease vaginal dryness. Vaginal lubricants may also be considered for sexual intercourse.
Persons with Sjogren's syndrome who experience affects to their internal organs, such as the kidneys, brain, gastrointestinal system, or spinal cord, may be prescribed immunosuppressive medications. The medications prescribed may include Prednisone, or more rarely, Methotrexate - a chemotherapy-type of medication. Every person with Sjogren's syndrome receives treatment specific to their particular needs and symptoms.