How can a disease that afflicts some 3 million Americans, 90 percent of them women, be as obscure as Sjogren’s syndrome? Experts say it is one of the three most common autoimmune disorders, but few laypeople know of it, and doctors rarely think of it when patients describe its various symptoms.
Medical students, even those in postgraduate training, learn little or nothing about Sjogren’s (pronounced SHOW-grins), in which the body attacks its own secretory glands and tissues. Diagnosis can be difficult because symptoms vary widely from patient to patient, and many of those symptoms mimic those of a host of other conditions.
As a result, “this major women’s health problem is still largely underdiagnosed and undertreated,” said Frederick Vivino, a rheumatologist at the University of Pennsylvania Medical Center and director of the Penn Sjogren’s Syndrome Center in Philadelphia.
But Vivino said in an interview that there had been “a dramatic change” in the diagnosis and
management of Sjogren’s in the last five to 10 years. Although it was originally listed in the National Organization for Rare Disorders registry, recent population studies in the US and in Britain have documented Sjogren’s as the second-most common autoimmune rheumatic disease, behind rheumatoid arthritis.
The disorder was first described in 1892 in a 42-year-old man and called Mikulicz’s syndrome. But the diagnosis fell into disuse because it seemed to encompass so many conditions. The syndrome was resurrected in 1933 by Henrik Sjogren, a Swedish ophthalmologist, who described 19 women suffering from dry mouth and dry eyes, the most common symptoms of the disorder.
A SPECTRUM OF SYMPTOMS
Lisa Worthington, 43, finally learned she had Sjogren’s last December, after six to eight years of confusing health problems.
“Sjogren’s is like a chameleon — it causes so many disparate symptoms throughout the body,” Worthington said in an interview. “It can seem like multiple sclerosis, stroke, fibromyalgia, Lyme disease, chronic fatigue, reflux and a bunch of other diseases. I’ve often had to explain it to physicians.”
Worthington now runs a marketing and public relations agency out of her home on the Eastern Shore of Maryland, which permits her to take time off when her health demands it. Before receiving a correct diagnosis and treatment for her spectrum of symptoms, Worthington said, she was told she had fibromyalgia, a reasonable explanation for her chronic muscle fatigue, joint pain and weakness.
“If I had to spend all day on my feet, the next day I had to stay in bed,” she said. But not until she awoke one morning with her eyes so dry she could not open them — “the lids were stuck to the eyeballs” — did a rheumatologist suggest Sjogren’s.
Though a blood test for telltale antibodies was negative, which happens in 40 percent of cases, the doctor sent her for a lip biopsy at the Sjogren Clinic at the National Institutes of Health, which confirmed his suspicions.
The all-too-common delay in diagnosis, which Vivino said still averages six years, can result in serious complications, including damage to vital organs.
Prompt diagnosis is also important because people with Sjogren’s face a 44-fold increased risk of developing lymphoma and must be alert to early signs of this cancer.
CHANGES FOR THE BETTER
Vivino said that although Sjogren’s was long thought to be an affliction primarily of white women around the age of menopause, it is increasingly being recognized in nearly all population groups: younger women, women of various racial and ethnic groups, men and even children.
“About 200 pediatric cases have been identified thus far,” he said.
Worthington was 37 when she developed symptoms that prompted her to consult a doctor; knowing what she does now, she believes signs were present years earlier.
Two other important advances are the publication in 2002 of internationally accepted criteria for diagnosing primary Sjogren’s (the condition can also occur secondary to other disorders), and the development of several medications that can stimulate the flow of saliva and relieve dryness and inflammation of the eyes.
Vivino said there was also growing interest among pharmaceutical companies in biologic remedies.
Sjogren’s can affect the basics of daily living and forces its sufferers to adjust life around it.
“I have trouble swallowing,” Worthington said. “I can’t swallow anything without liquid. I always carry a bottle of water with me, long before it was fashionable. Even at night, I have to drink water frequently and, of course, use the bathroom frequently. I often wake up feeling like I never slept.”
My stepmother, Sophie Brody, also had Sjogren’s. She thought at first she had a tumor because food would become stuck in her throat. Eventually she was unable to swallow anything but pureed and liquid foods. Even to swallow those without difficulty, she had to have her esophagus dilated every three months.
Her dentist was the first to suspect Sjogren’s. Her teeth were inexplicably decaying, breaking and falling out, the result of an insufficient flow of saliva to cleanse them. Women who develop Sjogren’s in early adulthood often need complete dentures by their mid-40s, Vivino said.
Depression is another frequent component of Sjogren’s, often preceding the onset of debilitating symptoms that may make anyone depressed. In fact, doctors at Johns Hopkins Medical Center are investigating various neurological manifestations of the syndrome, including spinal inflammation that can result in a misdiagnosis of multiple sclerosis.
Because Sjogren’s affects so many organ systems, other common symptoms and signs include vaginal dryness; enlarged parotid glands, the salivary glands in front of the ears; hoarseness; chronic dry cough; recurrent sinusitis, bronchitis and pneumonia; an extreme sensitivity to cold; dry skin and rashes; digestive problems, including a sensitivity to gluten; thyroid disorder; kidney problems; memory problems; numbness, tingling and burning pain in the feet and hands; frequent oral yeast infections; and an altered sense of taste.
Worthington’s taste buds are now much less sensitive, she said, and when she cooks she has to be careful not to use much garlic, salt or pepper.
She uses a variety of medications to relieve her symptoms, including one that stimulates secretions throughout the body, special eye drops that treat both the dryness and inflammation, and regular eye drops throughout the day.
Now that she and her doctors know what they are dealing with, she is checked every three months by a rheumatologist for signs of lymphoma, every six months by a dentist and every year by an ophthalmologist.
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