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You don’t look sick! What’s the problem? All About Sjogren’s Syndrome

Because Sjogren’s Syndrome is so often missed, and because so few people have heard of it, we don’t actually know how many Native Americans have it.
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Let’s start with some questions:

1) What disease was first observed in 1888 by British physician W. B. Hadden and later reported by a Swedish ophthalmologist in 1933?

2) What disease affects an estimated 4 million people in the U.S., mostly women and has as its well-known personality tennis star Venus Williams?

3) What autoimmune disease is the second most common disease to rheumatoid arthritis?

4) What is the incidence and effect of rheumatic disease in American Indians?

The answer to the first three questions is Sjogren’s Syndrome (pronounced Show-grins). The answer to the last question, according to rheumatologist Jasmine Gaddy of the Indian Health Service is that rheumatic diseases (e.g., Rheumatoid Arthritis and Lupus) are highly prevalent in American Indians and Native Alaskans, are often not typical in the ways they present themselves, and tend to be more aggressive resulting in higher morbidity and mortality.

Because Sjogren’s Syndrome (SS) is so often missed in the larger population, and because so few people have heard of it, we don’t actually know how many American Indians/Native Alaskans have the disease. What we do know is that Sjogren’s patients are 30-35 percent more likely to have close relatives with other autoimmune diseases, according to Frederick Vivino, director of the Sjogren’s Center at the University of Pennsylvania. Therefore, it’s important to learn how many of our people have this rheumatic disease as well.

You would think that a disease first observed 127 years ago now affecting four million people in the U.S. would have received some attention. This is especially true since treating its symptoms can prevent further damage to body organs. Sjogren’s is described by rheumatologists as a “burdensome” disease, yet its symptoms are too often seen as unimportant by family and physicians who say that the patients don’t look sick or that the symptoms are just part of aging. So what is this disease and what are its symptoms?

Like Type 1 Diabetes, Multiple Sclerosis, Rheumatoid Arthritis, and Lupus, Sjogren’s is an autoimmune disease. White blood cells (lymphocytes) attack the glands that produce moisture throughout the body. The mucous membranes and moisture producing glands of the eyes and mouth are often affected first, resulting in dry eyes and dry mouth—called Sicca symptoms. However, since the disease is systemic, other symptoms may appear first. It can affect the gastrointestinal tract, lungs, liver, pancreas, kidneys, blood vessels, and central/peripheral nervous systems. The dryness affects not only the eyes and mouth but can cause dry skin, brittle nails and hair. Vivino says that 70 percent of patients have only dry eyes and mouth, fatigue and muscular-skeletal pain; 25 percent may also have internal organ and nervous system involvement; five percent will also develop non-Hodgkin’s B-cell Lymphoma.

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Other symptoms include dental cavities, poor denture retention, burning mouth, speech difficulties, salivary gland swelling, problems swallowing, hoarseness, muscle pain and weakness, and neuropathy. It can occur alone (Primary) where there is no underlying autoimmune condition. Or it can be associated with an underlying connective tissue disease such as rheumatoid arthritis, lupus, or scleroderma (Secondary). Women are nine times more likely to have the disease but it is seen in all ages, racial and ethnic groups.

The cause is not known. At this point there is no cure, however treatments can ease symptoms and prevent further damage. Recent research has focused on more accurate diagnosis and decreasing the time it takes to achieve diagnosis. Previously it was said to take up to 10 years for diagnosis. Now, the estimate is closer to 5 years. Many of the symptoms can be related to other diseases, can be subtle and slow in development, and can vary from patient to patient. For example, according to Julius Birnbaum, associate director of Johns Hopkins’ Jerome L. Greene Sjogren’s Syndrome Center, neurological syndromes can precede dry eyes and mouth in 40-60 percent of patients and they can have Central/Peripheral Nervous System Disease up to a decade or more before Sicca symptoms.

Symptoms are treated by different specialists depending on the organs or systems affected. The patient may go from physician to physician with symptoms that appear unrelated. Patients may be thought to be depressed or neurotic. While most patients are in their 40s or older, children, teenagers and young adults can also suffer from the disease. Referral to a rheumatologist too often occurs only after numerous attempts to find solutions. As with health care in general, there are limits in insurance and office visit time to address this complex disease.

Over the decades there has been great variation in accepted definitions of the disease and which diagnostic studies produce the most accurate diagnosis. Symptoms may not correlate well with laboratory testing. Common testing can include dry eye and mouth, autoantibody, lip biopsy, and neurological testing. Many patients are antibody positive, however, absence of antibodies does not exclude diagnosis of SS. Birnbaum states that 50 percent of patients with neurological disease and Sjogren’s Syndrome will not have positive antibody tests. Patients may develop extra-glandular complications whether tests are positive or not. Even though test results can be confusing, it is critical that accurate diagnosis is made so that appropriate treatment may be provided and incorrect treatment for a disease with similar symptoms be avoided.

While there is no cure treatments can relieve symptoms, prevent complications and further damage. For dry eyes, there are artificial tears, gels, ointments, and warm compresses; for dry mouth there are sugar free lozenges and gums, artificial saliva and mouth sprays, and medications to stimulate saliva. Dental hygiene is critical. These are mostly over-the-counter aids, which can be obtained without a prescription. However, since non-prescription medications are typically not covered by health insurance they can become expensive. Prescription drugs include an anti-malarial drug (hydroxychloroquine) which alleviates symptoms in some people. Steroids such as prednisone may be used as well. Other drugs are used depending on the organ or system involved.

What should you do if you suspect you may have Sjogren’s? When people find themselves with a number of symptoms typical of the disease, it is important for them to tell their health care providers and not assume they will be discovered in office visits. It is helpful for patients to prepare for medical appointments by keeping notes or journals about their various symptoms, how long they have existed, how and when they occur, and what worsens or relieves the symptoms. It is also important for patients to provide information about other family members who have the disease or who have other autoimmune diseases. Documentation of symptoms will help the various specialists (e.g., dentists, optometrists, allergists, dermatologists, gastroenterologists, neurologists, etc.) see the connections and the critical need to diagnose and treat this disease syndrome.

Sjogren’s is a serious disease with serious consequences. Because so few people know about it, education of the general public and the numerous health care professionals likely to see patients with its many symptoms is critical. Because of the prevalence, morbidity and mortality of rheumatic diseases in the American Indian/Native Alaskan populations it is important that we diagnose and identify Sjogren’s in our communities. The Sjogren’s Syndrome Foundation is actively addressing needs for research, diagnosis, and education of the general population and health professionals. If you are a health provider, it is important to educate yourself regarding this condition. If you or a family member has a combination of symptoms that seem related to Sjogren’s, ask your health provider to consider the possibility of this disease. People with Sjogren’s are often told they don’t look sick. They are.

For more information, education and support, contact the Sjogren’s Syndrome Foundation. You may also contact our American Indian Non-Profit organization for assistance in finding a Sjogren’s specialist near you for diagnosis and treatment.

Dr. Ann Dapice (Lenape/Cherokee) received a Ph.D. in psychology, sociology and philosophy from the University of Pennsylvania. She is Director of Education and Research for T.K. Wolf, Inc., a 501(c)(3) American Indian organization and Founder/Executive Director, Institute of Values Inquiry.