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
RSS