Sjogren’s syndrome –An often Overlooked Disease
She didn’t have the energy she used to have. She chalked it up to getting older, although she noted that her 72-year-old mother seemed to have more energy than she did.
Betty B. is a 48–year-old female who noticed some dryness of her eyes several years ago. This wasn’t really a big problem, and she bought some artificial tears at Wal-Mart to keep in her purse. When she saw her eye doctor, he did tell her that her eyes were a little dry. Lately, she started carrying a water bottle around with her because she was thirsty all the time. She felt she was in good health but was having to see the dentist for extensive dental work. In the past six months, she developed fatigue. She didn’t have the energy she used to have. She chalked it up to getting older, although she noted that her 72-year-old mother seemed to have more energy than she did. Her hands started to hurt and she finally decided to see her internist. Blood was drawn and she was found to have a positive antinuclear antibody (ANA). The nurse told her that an ANA was a test for Lupus. Thinking that she remembered that lupus was a fatal disease (which it usually isn’t), she became anxious. Her internist referred her to a rheumatologist, an internal medicine doctor that receives additional training in arthritis, rheumatism, osteoporosis, and connective tissue diseases. At the visit, the rheumatologist noted that she had dry eyes and a dry mouth. He questioned her about fatigue, joint pain, and made note of the dental work. Further blood tests were taken and Sjogren’s antibodies came back positive. She also had positive thyroid antibodies. She was sent to an oral surgeon and a lip biopsy was taken; it was also positive for Sjogren’s syndrome.
Sjogren’s syndrome (pronounced Sho-gren’s) is named for a Swedish ophthalmologist who first recognized it as a syndrome. Sjogren’s is a chronic, autoimmune, and systemic disease. Autoimmune means that the immune system turns against the body and damages normal tissues. In the case of Sjogren’s Syndrome, the glands that make tears and saliva. Systemic means that it effects the entire body. In Sjogren’s, a type of white blood cell called a T cell invades the glands of the eye and mouth destroying them, which leads to the lack of tears and saliva. Nine out of ten cases are reported in women who are usually in their 40’s when it starts. Although Sjogren’s syndrome causes sicca symptoms —that is dryness of the mouth and eyes, it can also affect other parts of the body. In my practice, I have patients with Sjogren’s syndrome who have lung disease (interstitial lung disease), bladder disease (chronic interstitial cystitis), nerve damage (neuropathy), kidney disease (interstitial nephritis), gastrointestinal tract disease, and central nervous system disease (cerebritis). Sjogren’s is a “cousin” of lupus and the ANA test is often positive in Sjogren’s, just as it is in Lupus.
Other diseases are associated with Sjogren’s including Rheumatoid Arthritis, Lupus, Scleroderma, and Polymyositis. These diseases can coexist with Sjogren’s in the same patient. Patients with Sjogren’s often have autoimmune thyroid disease as manifested by antithyroid antibodies (as Betty B. did). This disease is called Hashimoto’s Thyroiditis. Many of these autoimmune diseases are in the Th-1 family of autoimmune diseases. Th-1 refers to a certain pattern of inflammatory cytokines. Cytokines are substances that the white cells produce in order to be able to communicate with each other. They are necessary for everyday good health. But with autoimmune diseases, certain cytokines are produced in excessive amounts, which lead to a number of problems. Cytokines are produced with the inflammation that is part of Sjogren and Hashimoto’s. This excess production leads to a many symptoms that we see including joint pain, low-grade fever, myalgias (muscle pain), cognitive problems (trouble thinking, concentrating, remembering), depression, and fatigue (excessive tiredness). Sjogren’s is also associated with Fibromyalgia, as is Hashimoto’s.
The dryness of the eyes can lead to corneal damage. Treatment with artificial tears is a must. Sometimes, the eye doctor will plug the tear ducts when the artificial tears are not enough or even cauterize them to permanently close them. A new medication called Restasis (cyclosporine ophthalmic emulsion) is a topical eye drop that helps reverse the dryness. Restasis contains cyclosporine which is an immunosuppressant drug also used to prevent transplant rejection. Used topically in the eye, it controls the inflammation of Sjogren’s and can lead to a reversal of the dryness. Restasis itself is not an eye lubricant, and patients using it need to continue to use their artificial tears.
A major problem in Sjogren’s is the dry mouth. While many patients feel that water is a good substitute for saliva, saliva contains many substances not found in water. For example, saliva contains anti-bacterial, anti-fungal, and anti-viral substances as well as minerals to re-mineralize our teeth. Without saliva, dental decay is rampant. Many Sjogren’s patients have terrible teeth and have multiple fillings and crowns as well as bridges, root canals, and dentures. Preventing the loss of teeth is a major goal in treating Sjogren’s. Better oral health is accomplished by using drugs that stimulate saliva. Two are on the market—cevimeline (Evoxac) and pilocarpine (Salagen). Both stimulate the salivary glands to produce saliva that contains all the substances that are needed for good dental health. They also help Sjogren’s patients eat. It is difficult to eat with a dry mouth. Caphosol is artificial saliva that contains calcium and phosphorus that helps re-mineralize the teeth. Patients with Sjogren’s often break teeth because they do not have enough calcium in them. Prevident toothpaste, which is made by Colgate and available by prescription only, contains an increased amount of sodium fluoride and is recommended to help prevent tooth decay. Dental check-ups and cleanings are recommended every six months. Fluoride treatments at the dentist are also recommended.
Because it is a systemic disease, Sjogren’s is often treated with drugs used for other systemic autoimmune diseases such as Lupus and Rheumatoid Arthritis. Plaquenil or hydroxychloroquine, a drug discovered 50 years ago for malaria and related to quinine, is still used today. It is very useful in Sjogren’s, Lupus, and Rheumatoid Arthritis. Recently it was discovered that it decreases the cytokine IL-6 and decreases autoimmunity at the toll-like receptors. It often helps lessen the joint pain and fatigue that is associated with Sjogren’s. Other stronger immunosuppressant drugs , such as leflunomide (Arava) or methotrexate, may be needed depending upon the situation. Occasionally, Sjogren’s may cause an inflammation in the blood vessels known as vasculitis that can be life threatening. In this case, stronger immunosuppressant drugs are needed (e.g., cyclophosphamide or Cytoxan). There are biologic drugs, such as rituximab (Rituxan), that are showing promise in Sjogren’s as well as in rheumatoid arthritis and lupus.
Sjogren’s has been associated with an increased risk of lymphoma, a type of cancer of the lymph glands. Lymphoma has been rare in my experience of over 600 Sjogren’s patients in 22 years of practice, but it has occurred.
There are an estimated four million people with Sjogren’s syndrome in the United States. It is a common disease that can significantly affect a patient’s life. It is frequently overlooked because many patients initially ignore the symptoms associated with it. Physicians fail to ask about dry eyes and dry mouth or they mistakenly diagnose lupus based on a positive ANA. Sometimes they tell the patient that their symptoms reflect aging. Patients may even be diagnosed as having fibromyalgia alone when the underlying problem is Sjogren’s syndrome causing chronic inflammation, fatigue, and pain. Further research into the cause and treatment of Sjogren’s syndrome will lessen the impact of this slowly progressive and often debilitating disease.
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"Sjogren’s syndrome –An often Overlooked Disease"
authored by:
Dr. McLain is board certified in internal medicine and rheumatology and is Chief of Rheumatology at Brookwood Medical Center, Birmingham, AL....
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