The Scoop on Sjögren's

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The Scoop on Sjögren's

Friday, April 18, 2008

FMOnline, vol. 8, no. 4 Courtesy of the Sjögren' Syndrome Foundation

Sjögren's (pronounced SHOW-grins) syndrome is a chronic autoimmune

inflammatory disease in which moisture-producing glands are damaged,

significantly decreasing the quantity and quality of saliva and

tears. The disease was first dentified by a Swedish physician,

Henrik Sjögren, in 1933. Although the hallmark symptoms are dry eyes

and dry mouth, Sjögren's also may cause dysfunction of other organs,

affecting the kidneys, gastrointestinal system, blood vessels,

lungs, liver, pancreas, and the nervous system. Patients may

experience extreme fatigue and joint pain and have a higher risk of

lymphoma.

Sjögren's is one of the most prevalent autoimmune disorders,

striking as many as 4 million Americans. Nine out of ten patients

are women. About half of the time Sjögren's syndrome occurs alone,

and the other half it occurs in the presence of another connective

tissue disease such as rheumatoid arthritis, lupus, or scleroderma.

When Sjögren's occurs alone, it is referred to as " Primary

Sjögren's. " When it occurs with another connective tissue disease,

it is referred to as " Secondary Sjögren's. " All instances of

Sjögren's syndrome are systemic, affecting the entire body.

Symptoms may plateau, worsen, or, uncommonly, go into remission.

While some people experience mild discomfort, others suffer

debilitating symptoms that greatly impair their functioning. Early

diagnosis and proper treatment are important—they may prevent

serious complications and greatly improve a patient's quality of

life.

Diagnosis

Sjögren's syndrome often is undiagnosed or misdiagnosed. The

symptoms of Sjögren's syndrome may mimic those of menopause, drug

side effects, or medical conditions such as lupus, rheumatoid

arthritis, fibromyalgia, chronic fatigue syndrome, and multiple

sclerosis. Because all symptoms are not always present at the same

time and because Sjögren's can involve several body systems,

physicians and dentists sometimes treat each symptom individually

and do not recognize that a systemic disease is present. The average

time from the onset of symptoms to diagnosis is over six years.

No single test will confirm the diagnosis and, since it is a

syndrome, Sjögren's syndrome may appear in many different forms in

different patients. Physicians may conduct a series of tests and ask

about symptoms. An international group of experts has formulated

classification criteria for Sjögren's syndrome which will help your

doctor arrive at a diagnosis. These criteria consider dryness

symptoms, changes in salivary (mouth) and lacrimal (eye) gland

function, and systemic (whole body) findings.

Blood tests you may have include:

ANA (Anti-Nuclear Antibody): found in 70 percent of Sjögren's

patients and people with other autoimmune diseases.

RF (Rheumatoid Factor): antibody found in 60-70 percent of Sjögren's

patients and people with rheumatoid arthritis.

SS-A (or Ro) and SS-B (or La): marker antibodies for Sjögren's.

Seventy percent of Sjögren's patients are positive for SS-A and 40

percent are positive for SS-B. Also found in lupus patients.

ESR (Erythrocyte Sedimentation Rate): measures inflammation. An

elevated ESR can indicate an inflammatory disorder, including

Sjögren's syndrome.

IGs (Immunoglobulins): normal blood proteins, usually elevated in

Sjögren's.

The eye tests include:

Schirmer test: measures tear production.

Rose Bengal and Lissamine Green: use dyes to examine the surface of

the eye for dry spots.

The dental tests include:

Salivary flow: measures the amount of saliva produced over a certain

period of time.

Salivary scintigraphy: a nuclear medicine test that measures

salivary gland function.

Salivary gland biopsy (usually in the lower lip): confirms

lymphocytic infiltration of the minor salivary glands.

Your physician will consider the results of these tests and his or

her examination to arrive at a final diagnosis. Further research is

being conducted to refine the diagnostic criteria for Sjögren's

syndrome and to help make diagnosis easier and more accurate.

Treating Sjögren's

Rheumatologists have primary responsibility for managing Sjögren's

syndrome. Patients also are seen and treated by specialists such as

ophthalmologists, optometrists, dentists and others.

Currently, there is no cure for Sjögren's syndrome. However,

treatments may improve various symptoms and prevent complications.

Prescription medicines for dry eyes and dry mouth are available. A

number of over-the-counter products may also be used to alleviate

different types of dryness. Immunosuppressive medications are used

to treat serious internal organ manifestations.

Sjögren's syndrome is serious, but generally not fatal if

complications are diagnosed and treated early. Sjögren's syndrome

patients must be monitored carefully for development of internal

organ involvement, related autoimmune diseases, and other serious

complications. In particular, patients should be aware that the

incidence of lymphomas

(cancer of the lymph glands) is significantly higher in people with

Sjögren's compared to the general population.

For more information about Sjögren's syndrome, contact the Sjögren'

Syndrome Foundation at 1-.