Syndrome Basic Facts

The case presented was a 55 year old woman who complained of dry eyes and dry mouth. Her eyes often felt “itchy,” and “scratchy” when she blinked. Her eating preferences had moved towards moist or juicy foods, as she had trouble chewing and swallowing dry foods. Her lips often felt dry. She also began to have dental cavities.

The student physicians were asked what they were thinking, and what tests they would order. After some wild guesses, they began to home-in on the answer and suggested a number of tests and asked for more information.

They found out the woman’s dry eyes had been daily, and for longer than three months duration. Yes, she said it felt like sand in her eyes. Similarly, her mouth had been dry-feeling daily for longer than three months, and she felt the need to drink water to help her swallow. Yes, she did think her cheeks may be swollen.

No, she did not have any arthritis or kidney disease. No, she did not have HIV, her chest x-ray was normal, and she never had radiation to her head and neck (which might have damaged her tear- and saliva-producing glands).

The students were proud of themselves when they learned blood tests and a biopsy had established the diagnosis, Sjögren’s Syndrome
What is Sjögren’s?

First, how is it pronounced? Dr. Sjögren was Swedish (some publications erroneously say Danish). It is pronounced “Shø-gren” (with the first vowel sound as in French “feu”), although many Americans say “SHOW-gren.”

It is an autoimmune disease with symptoms as described above. The person feels as if she has dry eyes and mouth, and this dryness is called sicca, Latin for dryness.

Autoimmune means for some reason the body seems to be having an immune reaction against itself. In this condition, it seems as if the body’s immune system is attacking the lacrimal and salivary glands, cutting off the supply of tears and saliva.

Types of Sjögren’s

Sicca features are sometimes found in other diseases such as systemic lupus erythematosis, rheumatoid arthritis, and systemic sclerosis; less often, in conditions like sarcoidosis. In all those cases, it is called Associated Sjögren’s

But when sicca occurs without those conditions, and without a history of other conditions that could cause sicca, such as radiation treatment to the face, then it is Primary Sjögren’s Syndrome (SS).

What is Happening?

“Something” (as yet unidentified) activates cells in the involved glands. The “something” might be a virus infection, for example. This leads to changes in the blood vessels, then release of chemokines that draw inflammatory cells to the gland. Lymphocytes flood in, jamming up the gland and impairing the production of tears or saliva. The lymphocytes also react with the altered blood vessels, consider them “foreign,” and establish a permanent immune response within lymphocyte memory. This is the “autoimmune” feature. Lymphocytes with this memory may attack blood vessels elsewhere that seem similar enough.

Who Gets Sjögren’s?

About 90% of cases are female. It occurs in two peaks: after the surge of adolescent hormones, i.e., late 20s, and after menopause. This suggests some relation to drops in estrogen levels. Generally, this is the same population in which systemic lupus occurs, suggesting a possible similar etiology.
How is It Diagnosed?

It’s diagnosed from the symptoms as well as tests. Available tests include blood tests (ANA, SS-A antibodies) and biopsy, especially of the easily accessible lacrimal gland.

Can It Be Cured?

No. But SS by itself can be treated with topical products: artificial tears, substitute saliva. Sometimes anti-inflammatory medications are used. Rarely, the activated lymphocytes may progress to a serious lymphoproliferative disease.