Sjögren's Syndrome Clinic
What is Sjögren's Syndrome?
Sjögrens
syndrome, named after the Swedish ophthalmologist, Henrik Sjögren, is an autoimmune
disorder. An autoimmune disorder is one in which the immune system attacks a persons
own body. This attack gives rise to inflammation that may cause destruction of the tissues
of the body or impair their function. In Sjögrens syndrome salivary and tear glands
are the major targets of the attack and the result is a decrease in production of saliva
and tears. The lack of saliva may cause symptoms of dry mouth (known as xerostomia),
difficulties with speech and swallowing food, extensive tooth decay, tooth loss, oral
sores and fungal infections in the mouth. The salivary glands may enlarge to produce
visible swelling, particularly below the ears at the angle of the jaw, and this may be
confused with mumps. Inadequate production of tears can produce symptoms of irritation and
a feeling of having grit or sand in the eyes. Many Sjögrens syndrome sufferers
experience debilitating fatigue. Blood tests show the presence of autoantibodies.
Antibodies are substances produced by the immune system of the body to defend against
foreign material, including viruses and bacteria. However, in Sjögrens syndrome,
these antibodies are directed against components of the body, such as cells or large
molecules.
Who gets the disorder?
Sjögrens
syndrome is thought to affect more than one million people in the United States alone. The
disorder can occur at any age, but the average person with the disorder at our clinic is
in his or her late fifties. Women with the disorder outnumber men by 9:1.
Sjögrens syndrome may occur alone or in association with other diseases such as
rheumatoid arthritis or lupus.
Where is the Clinic?
We are located in the
Gene Therapy and Therapeutics Branch (GTTB), National Institute of Dental and Craniofacial
Research (NIDCR), National Institutes of Health (NIH) at the Warren Grant Magnusson
Clinical Center in Bethesda, Maryland. Here we conduct basic and clinical research on
salivary function and Sjögrens syndrome.
The Sjögrens Syndrome Clinic: Past and
Present
Drs. Bruce Baum and Phil Fox established the
first dry mouth clinic in the United States in 1982. They made a conscious decision to
operate out of a bench-to-bedside program. The effort has progressed from fundamental
bench studies of salivary physiology to immunological investigations and most recently
toward gene therapy and the development of artificial salivary glands. In each of these
areas the studies included research that has progressed from cellular studies to animal
model studies and finally toward clinical studies. Sjögrens syndrome studies are
conducted in the Gene Therapy and Therapeutics Branch, NIDCR that is headed by Dr. Bruce
Baum. Excluding patients who visited the clinic purely to arrange for and have laboratory
and other tests, more than 300 patient visits occurred at the Salivary Dysfunction Clinic
in the past year. Currently more than 100 Sjögrens syndrome patients are regularly
followed at the clinic each year. We have collected systematic clinical and laboratory
data on the Sjögrens syndrome (and salivary dysfunction) population for a number of
years. Over 1200 patients with salivary dysfunction have been evaluated since the
inception of our clinic. Dr. Fox left the National Institutes of Health in December 1998,
and Dr. Stanley Pillemer, a rheumatologist, who has wide experience in the area of
clinical trials, currently oversees the Sjögrens Syndrome Clinic.
The
Future
Gene therapy has the potential
for inserting molecules such as cytokines that could modulate inflammation in salivary
glands to slow or prevent their destruction in Sjögrens syndrome. Also, the
capacity to produce molecules that enhance saliva production by residual salivary gland
cells, might be introduced by gene transfer. Eventually, development of artificial
salivary glands may have application in patients, who have lost all functional salivary
glands, because of radiation treatment or Sjögrens syndrome. We are expanding our
recruitment of patients so that we can accelerate the conduct of clinical trials for the
disorder.
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