'Dryness Disease' Gets Attention

Tuesday, September 19th 2000, 12:00 am
By: News On 6

WASHINGTON (AP) — They call it the dryness disease. Its hallmark symptoms of dry mouth and eyes at first are simply annoying — until they make patients' teeth fall out or damage eyesight.

Sjogren's syndrome is no mere nuisance illness.

Dryness is just the start of this mysterious, painful disease that can gradually attack the joints, organs and nerves, affecting more than 1 million sufferers. But it's so hard to diagnose that patients spend years hunting a medical Sherlock Holmes to solve the puzzling symptoms.

Frustrated by the ignorance, the National Institutes of Health this week holds an unprecedented meeting to teach health workers how to spot Sjogren's (pronounced ``show-grins'') and ease symptoms.

Indeed, the meeting comes as an influx of new research — ranging from eye-lubricating hormones to attempts at artificial salivary glands — has experts hoping improved therapies finally may be on the horizon.

Researchers now realize ``this is not just dry eyes and dry mouth in little old ladies,'' explains Sjogren's expert Dr. Elaine Alexander of San Diego's Arena Pharmaceuticals Inc.

Sjogren's is an autoimmune disease, meaning patients' immune systems create antibodies that attack their own tissue. Salivary glands and eye glands that produce tears and oils that keep eye moisture from evaporating often are damaged first. Tooth loss, fungal infections, corneal abrasions are common; some people can't hold a conversation without constant sips of water.

But dryness isn't always the first symptom. Fatigue and arthritis are common. Sjogren's can attack the thyroid, liver, kidneys or pancreas. It damages nerves, causing numbness in hands and feet. It can inflame blood vessels, called vasculitis. During pregnancy, it can cause fetal heart defects. Patients even have a 44-fold increased risk of the cancer lymphoma.

Part of the diagnosis problem is that symptoms vary so widely, and can range from mild to debilitating, says Dr. Stanley Pillemer of the NIH's National Institute of Dental and Craniofacial Research.

Nobody knows Sjogren's cause. Ninety percent of patients are women. Once thought a problem of old age, it actually can strike quite young.

Take Katherine Hammitt, whose first symptom came at age 15. She wasn't diagnosed until 32, by then suffering tremendous joint pain and numbing nerve damage.

``How can so many people have a disease no one's ever heard of?'' asks Hammitt, past president of the Sjogren's Syndrome Foundation. ``You go to doctor after doctor. ... I went to a neurologist for my numbness and when I could touch my nose, he asked, 'Have you seen a psychiatrist?' After a while, you just quit trying.''

There are a handful of treatments: Prescription pilocarpine and cevimeline stimulate remaining salivary gland tissue to produce more moisture. Moisturizing eye drops are standard. Some doctors prescribe the malaria drug hydroxychloroquine to reduce inflammation, and steroids for severe symptoms like vasculitis.

Now an array of new research attempts to pinpoint Sjogren's attacks and improve therapy:

—Dr. David Sullivan at Harvard Medical School discovered Sjogren's patients have low levels of androgens, male sex hormones like testosterone. Even women need a certain amount of testosterone, and low levels are linked to inflamed and dysfunctional eye glands. His lab created a testosterone eye cream that he said Allergan Corp. should begin testing in clinical trials soon to see if it restores eye-lubricating glands.

—NIH's Sjogren's clinic is hunting patients for clinical trials to see if certain inflammation-reducing drugs — the rheumatoid arthritis treatment Enbrel and thalidomide, that notorious birth defect-causing drug now being cautiously used against some intractable diseases — can help.

—NIH's Dr. Bruce Baum is attempting to use tissue engineering to grow artificial salivary glands, and to insert new genes into damaged salivary glands to make them secrete more saliva and block the immune-system destruction. It's much more preliminary than his colleagues' medication testing — years away from clinical trials — but experts say the research is promising enough to one day work.

—Australian scientists recently discovered antibodies targeting specific cell receptors may cause certain Sjogren's damage. Alexander's Arena Pharmaceuticals is hunting drugs that might protect those receptors and thus prevent dryness and nerve damage.