“We have nearly 4 million people in the country with the disease, 90 percent of whom are women,” says Dr. Frederick B. Vivino, head of the Sjögren's Center at the University of Pennsylvania School of Medicine. It affects women more than diabetes or heart attacks, but gets no press.

I first heard about Sjögren’s during a class discussion at the USC Annenberg School of Communication where I am earning a master’s degree in specialized journalism. Before Williams’ well-publicized pullout, little was written about this systemic disease characterized by severely compromised moisture-producing glands, dry mouth, dry eyes, arthritis-like joint pain, and severe fatigue. The parchedness in the mouth and eyes can be so extreme that it causes difficulty in eating, speaking, and even crying.

Despite the efforts of organizations like the Sjögren’s Syndrome Foundation, very little progress has been made in increasing awareness. “Like PMS and migraines, this is another disease that is in the danger of being swept aside as a woman’s disease,” says Dr. Amrita. P, Guru Tegh Bahadur Medical College in New Delhi, India.

Dr. Vivino is grateful for Williams’ disclosure. “In the University of Pennsylvania Medical School, we started giving first-year medical students lectures about Sjögren's only last year. Many doctors say they have never heard of it. This is serious, considering that it is a very major women’s disease.”

Most autoimmune diseases affect women more than men or transgender people. Hormones play a role, explains Vivino. “Some studies in humans also show that a lack of DHA, a testosterone derivative, and changes in the usual androgen-to-female hormone ratio are causes.”

Sjögren’s is not a new discovery. Cases were reported in European medical literature as early as the late 19th century. Though Dr. Sjögren, a Swedish ophthalmologist, was not the first to describe the comprehensive symptoms, in 1933 he was the first to emphasize the systemic nature and the interrelatedness of the three cardinal symptoms of dry eyes, dry mouth, and arthritis.

Judy Davis was 46 when she was diagnosed. “I was an elementary school teacher and would be in such pain that I could barely finish my work.” She went to a rheumatologist who came up with the diagnosis. “I have both lupus and Sjögren’s. People thought I was having emotional difficulties. Most don’t understand how sick you are,” she says.

Many women tend to ignore symptoms until they become debilitating. “Try explaining to your three-year-old why you need to lie down so often and you will get your answer,” says one young woman from Connecticut.

This, according to Dr. Vivino, causes greater complications. “A very common theme compounding the lack of awareness is the fact that many patients look a lot better than they feel.”

The average time between when symptoms begin and when a diagnosis is made usually takes up to seven years. “Venus Williams was playing until recently and she’s had symptoms for a very long time. It affects so many organ systems that it’s difficult to put together all the symptoms and come up with the big picture and give a unifying diagnosis,” says Vivino.

Most patients also experience a ‘brain fog.’ Dr. Susan McDermott explains this as “an experience of feeling not quite ‘all there’ mentally.” In her talk “Brain Fog and Other Central Nervous System Symptoms: Is it Sjögren’s?” Dr. McDermott warns “it is important not to dismiss these things as being ‘just part of my Sjögren’s syndrome.’”

Overlapping clinical manifestations make diagnosing Sjögren's difficult. “So you have a combination of fatigue, joint pains, and symptoms of lupus. Patients often develop fibromyalgia – characterized by body pain, tenderness in the joints, sleep issues, and headaches – as well as rheumatoid arthritis. Tests show positive for RH factors applicable to arthritis as well as Sjögren's. I call it a cousin of lupus,” explains Dr. Vivino.

Often doctors think patients are hypochondriacs, demanding attention. “My doctor thought I was one,” says Ross. “But how could anybody fake the fatigue I was going through?”

Doctors are not being deliberately unsympathetic believes Dr. Vivino. “When I trained in rheumatology in Pennsylvania Medical School, we regarded it as a trivial disease.” He cites three studies done in the last three years that show that Sjögren's patients suffer a far poorer quality of life when compared to lupus sufferers and have increased annual healthcare and dental costs.

Oral symptoms offer key clues to the diagnosis of Sjögren's. Dr. Mahvash Navazesh says, “Unlike other diseases which are specific to one system or organs, Sjögren's has a multisite involvement… One is the primary disease that affects the mouth and eyes, and the other is the secondary disease that involves eye, mouth, and connective tissue disorders.”

Sjögren's patients develop more dental caries and are at a higher risk for gum and fungal infection. They do not have the protective effect of saliva because the saliva is reduced or gone.

The Professor of Diagnostic Sciences and Associate Dean of Academic Affairs and Student Life at Ostrow School of Dentistry of USC, Dr. Navazesh describes herself as someone who fills the gap between medicine and dentistry: “Patients may be blamed for not brushing or flossing properly, but the reality is that the compromised immune system and salivary gland condition alter the oral ecology in favor of microorganisms that can cause infection.”

Extreme cases of Sjögren's can cause death. “Diseases that are complications of Sjögren’s Syndrome are a major cause of mortality, with rates as high as 33 percent,” explains Dr. Vivino.

For years, typical patients were considered peri-menopausal white women, but now research has shown that Sjögren’s can affect women all of ages, races, and ethnic groups. “I believe many African American women are probably being misdiagnosed as having a mild case of lupus,” says Dr. Vivino.

For young people, Sjögren's can be particularly brutal. Paula, 22, was diagnosed with autoimmune illnesses the semester before she began college. “Within a month, I had lost about 20 pounds. I had already been tested for diabetes so I knew that was not the cause. I’m an art student and found it very frustrating when I physically could not do what I am passionate about.”

Paula is now working in New York. “After experiencing the achiness for so long, I am not really as sensitive as I used to be,” she tells me. “I try to keep myself occupied. If you are constantly negative and thinking about the pain, then you won’t get anywhere. It’s keeping a positive outlook on life and doing what you’re passionate about that will make you go far in life.”

For patients with children, Sjögren’s brings additional pressures. “I won’t say that my illness stole my children’s childhood, but yes, it did make them learn to do things and become independent faster than most,” says Ross.

There is no known vaccine or single medicine to treat all the symptoms. As Dr. Vivino says, “This is a field begging for new research.” Yet, Susan Ross may have an answer. “Exercise is what keeps me going. Even when I don’t like doing it I say to myself, ‘Bring on the endorphins’ and I am ready to move!”

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About the Author:
Paromita Pain has been a senior reporter and writer for The Hindu and has worked with several other media projects, focusing on health, development and social journalism. A graduate student in the Annenberg School of Journalism at the University of Southern California, Paromita is specializing in health, human rights and prison systems.