For four million Americans, battling dryness has become a part of everyday life. Dry eyes, dry mouth, dry skin…and that’s just on the outside. The common thread for these Americans is a Sjögren’s syndrome diagnosis. Naming the beast, however, does not necessarily mean that relief is on the way.
For those afflicted with Sjögren’s, a confirmed diagnosis is usually the result of a lengthy process. Sjögren’s symptoms are often associated with other conditions, as well as being common side effects associated with popular medications. Diagnosis, therefore, is a twisted process of elimination that rules out other possibilities without decisively pointing to Sjögren's; when all the other options have been ruled out, Sjögren’s is what remains.
According to the Sjögren's Syndrome Foundation, seven years typically pass before a diagnosis is confirmed and, even then, treatment is limited to management rather than clearing up the condition. Although onset can begin at any age, Sjögren’s patients are often older than 40, and nine out of ten patients are women.
What is Sjögren’s syndrome?
Sjögren’s syndrome is a chronic autoimmune disease in which people’s white blood cells attack their moisture-producing glands. Although the hallmark symptoms are dry eyes and dry mouth, Sjögren’s may also cause dysfunction of other organs such as the kidneys, gastrointestinal system, blood vessels, lungs, liver, pancreas, and the central nervous system. Patients may also experience extreme fatigue and joint pain and have a higher risk of developing lymphoma.
About half of the time Sjögren’s syndrome occurs alone (primary), and the other half it occurs in the presence of another autoimmune connective tissue disease (secondary) such as rheumatoid arthritis, lupus, or scleroderma. Since the disease was first identified in 1933 by Dr. Henrik Sjögren, it has been proven to affect virtually every racial and ethnic group. General awareness about Sjögren’s syndrome is still lacking and increased professional awareness is needed to help expedite new diagnoses and treatment options.
Both primary and secondary Sjögren’s syndrome are systemic, affecting the entire body. Symptoms may remain steady, worsen, or in rare cases, go into remission. While some people experience mild discomfort, others suffer debilitating symptoms that greatly impair their functioning. Early diagnosis and proper treatment are important in order to prevent serious complications and greatly improve a patient’s quality of life.
Screening for Sjögren’s
There is no single test that confirms diagnosis. Rheumatologists have primary responsibility for diagnosing and managing Sjögren’s syndrome and can conduct a series of tests and ask about symptoms. An international group of experts formulated classification criteria for Sjögren’s syndrome, which help doctors arrive at a diagnosis. These criteria consider dryness symptoms, changes in salivary (mouth) and lacrimal (eye) gland function, and systemic (whole body) findings.
Blood tests your physician may perform include:
ANA (Anti-Nuclear Antibody) ANAs are a group of antibodies that react against normal components of a cell nucleus. About 70 percent of Sjögren’s patients have a positive ANA test result.
RF (Rheumatoid Factor) This antibody test is most often performed for the diagnosis of rheumatoid arthritis (RA) but is positive in many rheumatic diseases. In Sjögren’s patients, 60-70 percent have a positive RF.
SS-A (or Ro) and SS-B (or La) These are the marker antibodies for Sjögren’s. Seventy percent of Sjögren’s patients are positive for SS-A and 40 percent are positive for SS-B (these may also found in lupus patients).
ESR (Erythrocyte Sedimentation Rate) This test measures inflammation. An elevated ESR indicates the presence of an inflammatory disorder, including Sjögren’s syndrome.
IGs (Imunoglobulins) These are normal blood proteins that participate in immune reactions and are usually elevated in Sjögren’s patients.
The ophthalmologic (eye) tests include:
Schirmer Test Measures tear production.
Rose Bengal and Lissamine Green
Eyedrops containing dyes that an eye-care specialist uses to examine the surface of the eye for dry spots.
The dental tests include:
Salivary Flow Measures the amount of saliva produced over a certain period of time.
Salivary scintigraphy A nuclear medicine test that measures salivary gland function.
Salivary gland biopsy (usually in the lower lip) Confirms inflammatory cell (lymphocytic) infiltration of the minor salivary glands.
Your physician will consider the results of these tests along with your physical examination to arrive at a final diagnosis. Further research is being conducted to refine the diagnostic criteria for Sjögren’s syndrome and to help make diagnosis easier and more accurate.
What Treatments are available?
Early diagnosis and high-quality professional care are extremely important for Sjögren’s patients. Currently, there is no cure for Sjögren’s syndrome. However, treatments may improve various symptoms and prevent complications.
In addition to over-the-counter eye drops and mouth preparations, prescription products for dry eyes and clude Evoxac® (cevimeline), Salagen® (pilocarpine hydrochloride), and Numoisyn™ for dry mouth and Restasis® (cyclosporine ophthalmic emulsion) and Lacrisert® (hydroxypropyl cellulose ophthalmic insert) for dry eyes.
Some patients are prescribed immunosuppressive medications to treat their internal organ manifestations. Physicians may also prescribe other medications for systemic manifestations or severe flares. Since Sjögren’s syndrome affects each patient differently, a personalized plan should be developed by you and your physician, dentist, and eye-care specialist about how to treat your various symptoms.
In addition, many symptoms and problems of Sjögren’s syndrome can be treated with over-the-counter medications. These medications can help to alleviate different types of dryness and pain, but you should check with your physician when adding these medications to your regimen. He/she may have suggestions for what products you should use and may also give you some tips on how and when to use them.
Will I die from Sjögren’s?
Sjögren’s is serious but generally not fatal if complications are diagnosed and monitored carefully for development of internal organ involvement, related autoimmune diseases, and other serious complications. In particular, patients should be aware that the incidence of lymphomas (cancer of the lymph glands) is significantly higher in people with Sjögren’s compared to the general population.