Sjogren's syndrome is an autoimmune disorder that is characterized by dry eyes and a dry mouth. Autoimmune disorders occur when the immune system, which normally fights against diseases and infections, attacks healthy tissues by mistake. In Sjogren's syndrome, the immune system destroys the glands that secrete tears and saliva first. Sjogren's syndrome is a progressive disorder. If not properly managed, the disorder may eventually damage other tissues in the body, including the nervous system, kidneys, lungs, and skin.
Sjogren's syndrome is divided into two categories: primary and secondary. Both forms occur with similar frequency. Primary Sjogren's syndrome occurs by itself. No other autoimmune disorders are present in patients with primary Sjogren's syndrome. Secondary Sjogren's syndrome occurs when the condition occurs in combination with other autoimmune disorders, such as systemic lupus erythematosus (SLE), scleroderma, or rheumatoid arthritis.
Sjogren's syndrome can develop at any age. However, most people are diagnosed when they are 40 years of age or older. Women are about nine times more likely to develop the condition than men, estimates the National Institute of Neurological Disorders and Stroke (NINDS).
There is currently no known cure for Sjogren's syndrome. Instead, treatment focuses on relieving symptoms of the disorder. Patients with primary Sjogren's syndrome rarely experience any organ involvement and usually have normal life expectancies. However, the prognosis for patients with secondary Sjogren's syndrome depends on the other autoimmune disorders that are present.
The exact cause of Sjogren's syndrome remains unknown. However, researchers know that the condition is an autoimmune disorder. This means that the patient's immune system attacks healthy tissues by mistake. The body's immune cells begin to attack the glands that secrete moisture, particularly the saliva glands and tear ducts. As a result, patients are unable to produce enough tears or saliva. Eventually cells in other parts of the body, such as the lungs, kidneys, nervous system, or skin, may be attacked.
Researchers have not discovered what triggers this abnormal immune response. Most experts believe that a combination of factors, including genetics, hormones, and infections, may contribute to the development of Sjogren's syndrome.
SIGNS AND SYMPTOMS
Common symptoms of Sjogren's syndrome include dry eyes, dry mouth, enlarged salivary glands (called the parotid glands), fatigue, dental cavities, difficulty swallowing or chewing, hoarseness, changes in the sense of taste, blurred vision, sensitivity to light, corneal ulcers, skin rash or dry skin, dry cough, vaginal dryness, oral yeast infections, and joint pain or stiffness.
Birth defects: In rare cases, babies born to mothers with Sjogren's syndrome have been born with heart problems. Women with Sjogren's syndrome who are pregnant or plan to become pregnant should discuss the potential risks of childbirth with their healthcare providers.
Inflamed organs: Another uncommon complication of Sjogren's syndrome is inflamed organs. Sometimes internal organs, such as the kidneys, liver, or lungs, may become enlarged. When this happens, the enlarged organs may become infected or lose some functioning. For instance, if the lungs become enlarged, patients have an increased risk of developing bronchitis or pneumonia.
Lymphoma: In rare cases, patients with Sjogren's syndrome may develop cancer of the lymph nodes called lymphoma. Symptoms of lymphoma typically include enlarged lymph nodes, weight loss, fatigue, and night sweats.
Peripheral nervous system disorders: Although uncommon, Sjogren's syndrome may affect the peripheral nervous system and cause peripheral neuropathies. This part of the nervous system allows individuals to feel sensations, such as pain. Patients with peripheral neuropathies may experience numbness, tingling, and burning sensations in the arms and legs. In other cases, the cranial nerves that are in the face and eyes may become damaged. If cranial damage occurs, patients may experience vision problems or facial pain.
General: There is no single test to diagnose Sjogren's syndrome. A healthcare provider will first take a detailed medical history and perform a physical examination. The doctor will ask if the patient is taking any medications, such as anticholinergics (medicine that opens the airways by relaxing the muscle bands that tighten around the airways), that may cause side effects similar to Sjogren's syndrome symptoms. If the condition is suspected after a physical examination, a healthcare provider first performs a blood test to determine if abnormal immune cells are present. If these cells are present, additional tests, such as eye tests, imaging studies, and/or biopsies, may be performed to confirm a diagnosis and evaluate the patient's condition.
Fluorescent antinuclear antibody (FANA) test: A blood test, called a fluorescent antinuclear antibody (FANA) test, may be performed if Sjogren's syndrome is suspected. This test detects abnormal antibodies called autoantibodies. These autoantibodies bind to components of an individual's cells and cause the immune system to attack the body. If autoantibodies are present, this suggests that the patient has an autoimmune disorder. However, this test does not determine the specific type of autoimmune disorder that the patient has.
C-reactive protein (CRP) test: The C-reactive protein (CRP) test can be used to monitor inflammation, which is associated with autoimmune disorders. A high or increasing amount of CRP in the blood suggests that the patient has an acute infection or inflammation. In a healthy person, CRP is usually less than 10 milligrams per liter of blood. Most infections and inflammations result in CRP levels higher than 100 milligrams per liter of blood.
While the test is not specific enough to diagnose a particular disease, it can suggest an autoimmune disorder and it can be used to help physicians monitor inflammation and determine if current treatments are effective.
Erythrocyte sedimentation rate (ESR): An erythrocyte sedimentation rate (ESR) test may be conducted to measure and monitor inflammation associated with autoimmune disorders. This blood test measures the rate in which red blood cells settle in unclotted blood.
During an inflammatory response, the high proportion of fibrinogen in the blood causes red blood cells to stick together. The red blood cells form stacks called rouleaux, which settle faster than normal. Elevated levels usually occur in patients who have autoimmune disorders.
Normal values for men younger than 50 years old are 15 millimeters per hour, and normal values for men older than 50 are less than 20 millimeters per hour. The normal values for women who are less than 50 years old are less than 20 millimeters per hour, and the normal values for women older than 50 are less than 30 millimeters per hour.
Like the C-reactive protein test, an ESR test does not suggest a specific diagnosis. However, it is useful in detecting and monitoring tissue death and inflammation.
Schirmer tear test: An eye test, called a Schirmer tear test, may be performed to measure the dryness of the eyes. During the procedure, a small piece of filter paper is placed under the lower eyelid to measure the amount of tears that are produced.
Slit-lamp exam: An eye doctor may use a slit lamp to determine how dry the eye is and whether or not the eye is inflamed. The eye doctor places a drop of colored dye into each eye. The doctor then looks at the patient's eyes using a piece of magnifying equipment, called a slit lamp. The dye stains parts of the eye that have been damaged as a result of dryness.
Imaging studies: A special type of X-ray, called a saliogram, may be performed to determine the condition of the patient's salivary glands. Before the procedure, a colored dye is injected into the patient's parotid glands, which are behind the jaw and in front of the ears. The saliogram produces detailed images of the salivary glands and allows healthcare professionals to see the flow of saliva into the mouth.
Biopsy: A lip biopsy may also be performed to detect the presence of clusters of inflammatory cells, which suggest Sjogren's syndrome. During the procedure a small piece of tissue is removed from salivary glands in the lip and examined under a microscope.
General: There is currently no known cure for Sjogren's syndrome. However, symptoms of the disorder can be managed with treatment. In fact, patients with primary Sjogren's syndrome who receive treatment rarely experience any organ involvement and usually have normal life expectancies. However, the prognosis for patients with secondary Sjogren's syndrome depends on the other autoimmune disorders that are present.
Artificial tears: Artificial tears made with hydroxymethylcellulose or dextran may be applied to the eyes to relieve symptoms of dry eyes. Hydroxypropyl methylcellulose (Lacrisert©) is often used to treat dry eyes caused by Sjogren's syndrome.
Artificial saliva: Artificial saliva products, such as Salivart©, may be used as often as needed to reduce symptoms of dry mouth. These products are available as solutions, mouthwashes, sprays, and swabs. They are also available in many flavors, such as mint. Some of these products are made with calcium or fluoride to help protect the teeth.
Toothpaste and gum: Specially designed toothpastes (such as Biotene Antibacterial Dry Mouth Toothpaste©) and chewing gums (such as Biotene Dry Mouth Gum©) are available for patients with dry mouths. These toothpastes may help limit the amount of bacteria in the mouth and reduce gum irritations caused by dry mouth. Specially designed chewing gums are made with enzymes similar to those found in saliva.
Humidifier: Keeping the air in the home moist helps reduce symptoms of dry eyes. Patients can use a humidifier to prevent the air from becoming dry.
Vaginal lubricants: Vaginal lubricants, such as Replens©, may help treat vaginal dryness.
Skin creams and lotions: Skin creams, such as Eucerin©, or lotions, such as Lubriderm©, may help treat dry skin. Creams and lotions should be applied regularly, especially after bathing or showering.
Immunosuppressants: Autoimmunity is controlled through balanced suppression of the immune system. Immunosuppressants are used to minimize the immune response against body cells without completely eliminating the immune response toward harmful invaders. Immunosuppressants are only available by prescription. Patients with Sjogren's syndrome may take immunosuppressant eye drops called cyclosporine (Restasis©). This helps reduce symptoms of dry eyes.
If the internal organs are affected by Sjogren's syndrome, patients may need to take immunosuppressant by mouth. Commonly prescribed immunosuppressants include cyclosporine (Neoral©, Sandimmune©, or Gengraf©), azathioprine (Azasan© or Imuran©), basiliximab (Simulect©), aclizumab (Zenapax©), muromonab CD3 (Orthoclone OKT3©), tacrolimus (Prograf©), glatiramer acetate (Copaxone©), mycopehnolate (CellCept©), and sirolimus (Rapamune©). Immunosuppressants are available in tablet, capsule, liquid, and injectable forms. The recommended dosage depends on the specific drug, as well as the individual's age and overall health. Side effects of immunosuppressants may lead to severe infections because these drugs weaken the immune system.
Corticosteroids: Corticosteroids, such as prednisone (e.g. Deltasone©) and methylprednisolone (Medrol©), have been used to reduce inflammation associated with Sjogren's syndrome. Corticosteroids reduce the body's immune response, which subsequently alleviates symptoms. Patients with Sjogren's syndrome may take these medications by mouth, apply them to the skin, or use them as eye drops. These drugs are generally very effective when used short-term. However, if corticosteroids are used for many months to years, they may become less effective and serious side effects may develop. Side effects may include easy bruising, thinning of bones, cataracts, weight gain, a round face, and diabetes. Corticosteroids are usually prescribed for a certain amount of time, and then the patient is gradually tapered off the medication. Patients should not stop taking corticosteroids suddenly or change their dosages without first consulting their healthcare providers.
Nonsteroidal anti-inflammatory drugs (NSAIDs): Nonsteroidal anti-inflammatory drugs (NSAIDs) have been used to relieve pain and inflammation caused by Sjogren's syndrome. These medications are especially helpful in patients who have painful or swollen joints. Commonly used over-the-counter NSAIDs include ibuprofen (Advil© or Motrin©) and naproxen sodium (Aleve©). Higher doses of these drugs are also available by prescription. Commonly prescribed NSAIDs include diclofenac (Cataflam© or Voltaren©), nabumetone (Relafen©), and ketoprofen (Orudis©). The frequency and severity of side effects vary depending on the specific NSAID used. The most common side effects include nausea, vomiting, diarrhea, constipation, decreased appetite, rash, dizziness, headache, and drowsiness. The most serious side effects include kidney failure, liver failure, ulcers, heart-related problems, and prolonged bleeding after an injury or surgery. About 15% of patients who receive long-term NSAID treatment develop ulcers in the stomach or duodenum.
Some patients may also benefit from NSAIDs in eye drop form. However, NSAID eye drops should be used cautiously because they may damage the cornea. Patients taking NSAID eye drops should visit their eye doctors regularly.
Pilocarpine (Salagen©): A prescription medication called pilocarpine (Salagen©) may be taken to treat symptoms of dry mouth. However, this drug has been shown to be unsafe for patients with poorly controlled asthma, heart disease, or if pregnant or breastfeeding.
Cevimeline (Evoxac©): A prescription medication called cevimeline (Evoxac©) may be taken to treat symptoms of dry mouth. This medication stimulates glands inside the mouth to produce more saliva.
A minor surgical procedure may be performed to relieve symptoms of dry eyes. During the procedure,
the tear ducts, which drain tears, are partially or completely plugged. This allows the tears to lubricate the eyes longer. The tear ducts may be temporarily or permanently sealed.
Unclear or conflicting scientific evidence:
DHEA (dehydroepiandrosterone): Preliminary evidence suggests that DHEA (dehydroepiandrosterone) may not offer benefit to individuals with Sj©gren's syndrome. Further research is needed in this area.
Avoid if allergic to DHEA. Avoid with a history of seizures. Use cautiously with adrenal or thyroid disorders. Use cautiously if taking anticoagulants or drugs, herbs, or supplements that treat diabetes, heart disease, seizures, or stroke. Stop use two weeks before and immediately after surgery/dental/diagnostic procedures with bleeding risks. Avoid if pregnant or breastfeeding.
Gamma linolenic acid (GLA): Currently, there is limited evidence showing that GLA is effective in treating Sjogren's syndrome. Additional study is needed before a conclusion can be made.
Reports of allergic reactions to GLA are currently lacking in the available literature. Use cautiously with drugs that increase the risk of bleeding like anticoagulants and anti-platelet drugs. Avoid if pregnant or breastfeeding.
Traditional or theoretical uses lacking sufficient evidence:
Bee pollen: Bee pollen has been suggested as a possible treatment for Sjogren's syndrome. However, research is lacking in this area. Until scientific studies provide evidence of safety and efficacy for this use, bee pollen cannot be recommended for this condition.
Avoid if allergic or hypersensitive to bee pollen or other bee products. Avoid with liver disease or bleeding disorders. Use cautiously because bee pollen may contain mycotoxins, such as ochratoxin A. Avoid if pregnant or breastfeeding due to a lack of scientific data.
Omega-3 fatty acids: Omega-3 fatty acids are found in fish oil and certain plant/nut oils. Fish oil contains both docosahexaenoic acid (DHA) and eicosapentaenoic acid (EPA). Omega-3 fatty acids have been used to treat Sjogren's syndrome. However, scientific studies have not been performed in this area.
Omega-3 fatty acid is generally considered safe if taken in doses that do not exceed the recommended dietary allowance (RDA). Avoid if allergic to fish, omega-3 fatty acid products that come from fish, nuts, linolenic acid, or omega-3 fatty acid products that come from nuts. Avoid during active bleeding. Use cautiously with bleeding disorders, diabetes, low blood pressure, or if taking drugs, herbs or supplements that treat any such conditions. Use cautiously before surgery.
Physical therapy: The goal of physical therapy is to improve mobility, restore function, reduce pain, and prevent further injury. A variety of techniques, including exercises, stretches, traction, electrical stimulation, and massage, are used during physical therapy sessions. Physical therapy has been proposed as a possible treatment for Sjogren's syndrome. However, human studies have not evaluated the safety and efficacy of physical therapy for this condition.
Not all physical therapy programs are suited for everyone, and patients should discuss their medical history with their qualified healthcare professionals before beginning any treatments. Based on the available literature, physical therapy appears generally safe when practiced by a qualified physical therapist. However, physical therapy may aggravate some pre-existing conditions. Persistent pain and fractures of unknown origin have been reported. Physical therapy may increase the duration of pain or cause limitation of motion. Pain and anxiety may occur during the rehabilitation of patients with burns. Both morning stiffness and bone erosion have been reported in the physical therapy literature, although causality is unclear. Erectile dysfunction has also been reported. All therapies during pregnancy and breastfeeding should be discussed with a licensed obstetrician/gynecologist before initiation.
SAMe: SAMe is a natural substance that is found inside the cells of the body. Although SAMe has been used to treat symptoms of Sjogren's syndrome, there is currently no scientific evidence to supports its use. Until human studies are performed in this area, a firm conclusion cannot be reached.
Avoid if allergic or hypersensitive to SAMe. Avoid with bipolar disorder. Avoid during first trimester of pregnancy or if breastfeeding. Use cautiously with diabetes, anxiety disorders, or during the third trimester of pregnancy.
Shark cartilage: Shark cartilage is one of the most popular supplements in the United States, with more than 40 brand-name products sold. Traditionally, shark cartilage has been used to treat Sjogren's syndrome. However, research has not been done to determine if this therapy is safe or effective in humans.
Avoid if allergic to shark cartilage or any of its ingredients (such as chondroitin sulfate or glucosamine). Use cautiously if allergic to sulfur. Use cautiously with coronary artery disease, peripheral vascular disease, liver disorders, diabetes, or kidney disorders. Avoid in children or if pregnant or breastfeeding.
There is currently no known method of prevention for Sjogren's syndrome.
Patients with Sjogren's syndrome should regularly visit their healthcare providers, including their dentists and eye doctors. Patients should also take all medications exactly as prescribed. Properly managing symptoms of Sjogren's syndrome improves the patient's prognosis.
This information has been edited and peer-reviewed by contributors to the Natural Standard Research Collaboration (www.naturalstandard.com).
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