When tennis superstar Venus Williams recently announced that she had been diagnosed with Sjögren syndrome, the tennis world issued a collective gasp. It was followed by the question: What on Earth is Sjögren syndrome? Venus had been plagued by, as she put it, an “energy-sucking” disease for some time. She suffered from so much fatigue and joint pain that it was sometimes hard for her to even lift her racket. Fans were incredulous. Venus appeared to be in such good shape and, in fact, had won her first match at the 2011 US Open. But then she withdrew from the competition suddenly. Many were bitterly disappointed and suggested that this was just an excuse for her to drop out of the tournament.
Nothing could be farther from the truth. The idea that Sjögren’s patients are “faking it” or are hypochondriacs is nothing new. That is due to two major factors—the disease is difficult to diagnose, and patients, like Venus, often appear to be perfectly healthy. So let’s put this nonsense to rest and finally understand what we can about this mysterious—and serious—autoimmune disease.
FOUR MILLION AMERICANS HAVE THIS DISEASE
Sjögren syndrome is a chronic inflammatory disorder in which disease-fighting white blood cells mistakenly attack the body’s own moisture-producing glands, causing symptoms such as dry eyes and dry mouth. The disease can strike children and older adults, but typically, patients develop it between the ages of 40 and the mid-50s. (Venus, who is only 31 years old, got it very young.) Up to four million Americans have Sjögren’s (90% of them are female, though doctors aren’t sure why), and unfortunately there is no cure. However, it can be managed. Alan Baer, MD, director of the Johns Hopkins Jerome L. Greene Sjögren’s Syndrome Center, explains…
Sjögren’s can present many challenges…
It’s tough to diagnose. On average, it takes six and a half years for doctors to put the puzzle pieces together.
• Symptoms seem unrelated. When the body’s immune system attacks the moisture-producing glands, it causes complications all over the body. The eyes and mouth become dry—without the benefit of cleansing saliva teeth develop cavities… and fatigue and joint pain may develop. In women, there may be a lack of vaginal lubrication, which leads to increased risk for infection and pain during intercourse.
• It’s dangerous. The longer a person goes without getting a diagnosis, the higher the health risks. If the disease advances undetected, it can set off a widespread inflammatory reaction that can harm the lungs, kidney, liver, pancreas and blood vessels as well as the gastrointestinal and central nervous systems. Up to 30% of Sjögren’s patients suffer organ damage, and about 5% develop lymphoma (lymph node cancer).
An accurate diagnosis of the syndrome takes into account multiple factors, including a blood test to check for inflammation, antibodies and elevations in immune-related blood proteins. A biopsy from the inside of the lower lip is taken to assess the inflammatory reaction within the small salivary glands. The production of saliva and tears is also tested.
Patients can choose from a variety of over-the-counter preparations that are generally very helpful for symptom relief, says Dr. Baer. Lubricants help with vaginal dryness. For dry eyes, there are drops and ointments… and for dry mouth there are sprays, gums, gels, lozenges, mouthwashes and toothpastes.
More powerful prescription drugs can also help dry eyes and mouth. The drug hydroxychloroquine (Plaquenil), originally developed to treat malaria, is useful in Sjögren’s to relieve fatigue and joint pain. To quiet an overactive immune system, patients can take methotrexate (Rheumatrex, Trexall)—it is used in high doses as chemotherapy, and now it’s also used in low doses to treat autoimmune disorders. Methotrexate can be well-tolerated but can also cause some nasty side effects, including mouth sores, stomach upset, skin rash, hair loss and liver toxicity.
One bright spot: Research on drugs for autoimmune diseases is extremely active, and as general awareness of Sjögren’s increases, patients are not likely to be left behind.
STEPS FOR QUALITY OF LIFE
Sjögren’s can be mild, moderate or severe. Severe cases can cause renal failure, disability due to peripheral neuropathy, impaired vision, hepatitis or pneumonitis. But Dr. Baer says that most cases are mild. To help maintain quality of life, exercise of any kind is important for physical and psychological reasons. An anti-inflammatory diet built on healthy foods is essential—eat plenty of vegetables, fruits, fish and other proteins, and limit processed foods and refined grains. Dr. Baer says that fish oil supplements and flaxseed oil may help the problem of dry eyes. To maintain a good level of energy, patients need plenty of sleep. They also need to avoid triggers such as extreme heat, fumes, cigarette smoke, dust and winds—all of which can aggravate symptoms.
Quality of life also has much to do with finding doctors who are well-versed in Sjögren’s. Rheumatologists are the primary doctors who handle it, but the nature of the disease also requires the involvement of ophthalmologists, otolaryngologists, dentists, neurologists and for women, gynecologists. Ideally, a patient will be treated at a Sjögren syndrome center, such as the one at Johns Hopkins, but these centers are relatively rare. More typically, doctors treat Sjögren’s within rheumatology centers, where expertise on autoimmune diseases is available. Once a patient has been diagnosed, his/her primary care doctor will locate an appropriate place for treatment. For an impressive amount of information about the disease and tips for living well with it, go to Sjögren’s Syndrome Foundation.
As for Venus, she says that she is very glad to have a diagnosis. She had gone to one doctor after another and left without answers. Now her symptoms all make sense, and she’s finally found the help she needs. There’s no doubt that her courage in coming out to talk about this mysterious illness will help many people down the road.