Unravelling the Mystery

by Carol Milano

Are your eyes often dry, irritated, red or painful? Do you have trouble swallowing dry food, like crackers? Is your mouth frequently parched?

If any of these sound familiar, you might have Sjogren's Syndrome, an immune system disorder. Though little known to the public, "People who work in this area say Sjogren's is the second most common rheumatic disease, after rheumatoid arthritis," observes Reza Dana, MD, MPH, an ophthalmologist at Brigham & Women's Hospital in Boston.


Nearly a century ago, a Swedish ophthalmologist reported on a group of arthritic women with very dry eyes and mouths. The syndrome Henrik Sjogren identified can bring a gritty or burning sensation in the eyes, problems with chewing or swallowing food, and eyelids that stick together. The condition may also lead to dryness of the nose, skin, lungs, kidneys, liver, blood vessels, or vagina, as well as fatigue or joint pain.

Abnormal proteins in the blood of Sjogren's patients mean their immune systems are reacting against their own tissues. As glands that make tears and saliva are damaged by immune cells called lymphocytes, their production decreases.


Ten times more common in women than men, Sjogren's has genetic, hormonal and immunologic causes. The disease can emerge at any age; onset is most common between 45 and 55.

Over a million Americans have Sjogren's, according to the American College of Rheumatology. The National Sjogren's Syndrome Foundation (SSF) puts the population at four million


It isn't rare, but Sjogren's is tricky to identify. A survey of SSF members found that 25% had spent over five years seeking an accurate diagnosis of their problems.

Why is Sjogren's difficult to detect? "Like many auto- immune diseases, its symptoms mimic other conditions. Often misattributed to menopause, Sjogren's symptoms don't raise a red flag in a doctor's mind. It may take longer to recognize or treat it," explains Stuart Kassan, MD, a rheumatologist, Clinical Professor of Medicine, University of Colorade Health Sciences College, and SSF Board member.

Sjogren's can overlap with or resemble scleroderma, lupus, rheumatoid arthritis, and other auto-immune illnesses. Specializing in cornea-external eye disease, Dr. Dana is often the first to recognize Sjogren's in patients referred to him. He finds that rheumatologists may not ask patients about dry eye (or mouth) problems; ophthalmologists can easily mistake serious dry eye for infectious conjunctivitis or another disease. Dentists who are aware of Sjogren's are sometimes the first to identify it, when they find healthy teeth starting to break due to reduced saliva production.

Alexis Stegemann, executive director of SSF, estimates that 50% of patients have Primary Sjogren's, which involves no other auto-immune disease. Those with Secondary Sjogren's also have another connective tissue disorder, such as lupus.


Two new products, created to alleviate the dry mouth of Sjogren's syndrome, are already available. Both Salagen (MGI Pharma, Inc.) and Evoxac (Daiichi Pharmaceutical Corp.) help patients chew, swallow, speak and even sleep more easily.

While over-the-counter eyedrops are too weak to soothe Sjogren's symptoms, several specific new remedies are edging closer to market, including:

  • androgen eyedrops to reduce lacrimal gland inflammation (Allergan)
  • A topical eyedrop to activate tear secretion (Inspire Pharmaceuticals)
  • Restasis, aimed at the underlying inflammatory process of dry eye (Allergan)
  • Evoxac, now being tested for its effectiveness on dry eyes
  • a lozenge to stimulate saliva function and improve oral comfort (Amarillo Biosciences, Inc.)
  • At the National Institutes of Health (NIH), researchers are exploring possible gene transfer to the salivary gland, or even an artificial salivary gland. NIH is also studying the effect of DHEA or thalidomide on Sjogren's patients. Both substances have helped treat other auto-immune conditions.


    Especially for a less-known disease, support groups can be significant. "Most Sjogren's patients don't look ill. The opportunity to talk to other people with the same syndrome is very validatng. Members share product information, everyday tips on dealing with dryness or fatigue, and other coping strategies," explains Stegemann. SSF sponsors 90 support groups in the USA, with thousands of members.


    The National Sjogren's Syndrome Foundation, an excellent source of information, is at or 800-475-6473 Their newsletter, The Moisture Seekers, is an outstanding way to keep up to date. The American College of Rheumatology at provides fact sheets (under Patient Information) and can help you find a specialist.

    Many of these articles appear on the publication's website, which are often password-protected or members-only. For your convenience, I've gathered them on my own website.