Sjögren's Syndrome

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Sjögren's Syndrome

Topic Overview

Sjögren's syndrome (pronounced " show-grins " ) is a disorder in which

the immune system attacks the body's moisture-producing glands (such

as tear and saliva glands). Sjögren's may cause exceptional dryness

in the eyes and mouth and pain in the joints. Sometimes it may also

cause problems with the function of vital organs. Sjögren's affects

over one million people throughout the United States and is most

common in white women who are in their 40s and 50s. However,

Sjögren's is diagnosed in women and men of all races and even in

children.

The cause of Sjögren's is unknown. It is possible Sjögren's may be

linked to a virus or it may run in families. Sjögren's may also be

connected to hormone levels, since it often affects women who are at

or near the age of menopause.

There are two types of Sjögren's syndrome: primary and secondary.

Primary Sjögren's is characterized by:

Sicca syndrome that has been present for at least three months.

Sometimes specific whole-body (systemic) problems that might involve

vital organ function (such as lungs, kidneys, and liver),

gastrointestinal (digestive) system, and the nervous system.

The absence of another autoimmune disease (connective tissue

disorder).

Secondary Sjögren's is characterized by:

Sicca syndrome.

The presence of another autoimmune disease (connective tissue

disorder), such as rheumatoid arthritis, lupus, or scleroderma.

To diagnose Sjögren's syndrome, doctors look for evidence of sicca

syndrome and positive results from certain laboratory tests. Together

these two criteria distinguish Sjögren's from other disorders with

similar symptoms.

Although there is currently no known cure for Sjögren's syndrome,

treatment for symptoms can offer relief and comfort for many people.

You and your doctor(s) can work together to alleviate the symptoms

you experience as each symptom arises.

Symptoms

The most significant progression of Sjögren's syndrome usually occurs

within the first few years of onset. This means the symptoms you

experience initially may increase and then remain the same. Your

symptoms probably will not get worse after the first few years but

may remain with you throughout your lifetime. While not common, some

people may develop serious complications involving vital organs (such

as the lungs, kidneys, and liver) as Sjögren's syndrome progresses.

It is also possible that Sjögren's can slightly increase the chance

of developing malignant (cancerous) tumors involving the lymphatic

system, such as non-Hodgkin's lymphoma.

Symptoms of Sjögren's syndrome can be divided into those that are

common and less common.

Common symptoms (SICCA syndrome)

Eyes

Dry, gritty, sandy, or itchy feeling in your eyes.

Thick, ropelike strands of dried mucus in your eyes when you wake up

in the morning

Decreased tearing, redness, and eye fatigue

A " filmy " sensation in your eyes that interferes with vision

Mouth

Decreased amount of saliva

Difficulty swallowing food without also drinking a liquid

Abnormal sense of taste

Sores (fissures) on the tongue and lips

Numerous cavities caused by rapid tooth decay

Decreased sense of taste and smell

Glands

Enlarged saliva glands under the chin (submandibular glands) and in

front of the ears (parotid glands) that can be sore and tender

Frequent changes in saliva gland size between normal and swollen

Less common symptoms

Skin

Exceptionally dry skin

Skin rash that can be sensitive to light

Vaginal dryness causing discomfort, itching, and painful intercourse

Stomach

Burning sensation (heartburn) in the chest or throat caused by

abnormal backflow of acid and other digestive juices

Respiratory

Dry nasal and breathing (bronchial) passages

Sinus infections that linger and a chronic cough

Whole body (systemic) symptoms

Increased fatigue

Joint and muscle pain

It is also possible to develop complications of Sjögren's syndrome.

Some examples of associated complications can include:

A yeast infection in the mouth (candidiasis).

Bronchitis that occurs more often and persists longer than normal due

to dry breathing (bronchial) passages.

Pneumonia that develops from persistent or frequent bronchitis.

Hypothyroidism.

Nervous system problems (such as peripheral neuropathies).

Very rarely, Sjögren's might increase the incidence of certain

related conditions, such as:

Malignant (cancerous) tumors in the lymphatic system.

Non-Hodgkin's lymphoma.

If you are experiencing symptoms other than or along with those

identified, it is possible you may have another autoimmune disease

along with Sjögren's syndrome. You should let your doctor know all of

your symptoms so you can be accurately assessed.

There are many other conditions with symptoms similar to Sjögren's

syndrome. Your doctor will make a clinical assessment of your

symptoms and perform some laboratory tests to confirm a diagnosis of

Sjögren's syndrome.

Exams and Tests

Your doctor will confirm a diagnosis of Sjögren's syndrome by

assessing your symptoms and conducting certain laboratory tests. Your

doctor will ask you for a list of the medications you have been

taking to determine whether they may be causing or worsening your

symptoms.

Doctors use specific clinical criteria for diagnosing Sjögren's

syndrome. Certain tests may help determine whether decreased

formation of tears or saliva, abnormal antibodies, or inflammation of

saliva gland tissue is present.

Schirmer's tests measure tear flow.

A rose bengal test measures potential damage to eyes.

Salivary function tests measure saliva flow.

Blood tests for Sjögren's syndrome (autoantibodies) measure amounts

of autoantibodies in the blood.

Lip or saliva gland tissue biopsy measures the amount of inflamed or

damaged saliva gland cells.

Additional tests may be performed by your doctor to help check for

evidence of other autoimmune diseases, which may also cause sicca

syndrome and other symptoms not related to Sjögren's. Further tests

that could help your doctor determine whether you suffer from

Sjögren's syndrome or another autoimmune disorder might include:

Erythrocyte sedimentation rate.

Rheumatoid factor.

Antinuclear antibodies.

To accurately diagnose Sjögren's syndrome or any other autoimmune

disorder, positive test results must also be supported by a clinical

evaluation of your symptoms.

Treatment Overview

There is no known cure for Sjögren's syndrome. Home treatment

measures can help you effectively manage most symptoms. However, if

your symptoms get worse in spite of home treatment, your doctor may

want to try medication or other, more aggressive forms of treatment.

Treatment for sicca syndrome (common)

If home treatment measures have not helped to improve your symptoms

of dry eyes or dry mouth, your doctor may use the following

treatments:

Help for dry eyes

If using a tear substitute has not eased your dry eyes, your doctor

may place temporary or permanent plugs in your tear ducts (lacrimal

ducts) to help keep moisture in your eyes.

Help for dry mouth

If a yeast infection develops in your mouth, antifungal drugs may be

used.

A fluoride rinse may be used to help prevent cavities caused by rapid

tooth decay.

A prescription-strength saliva stimulant, such as pilocarpine

(Salagen) tablets, may be prescribed if other methods used to

increase saliva production have not brought relief for a dry mouth.

Research to develop new drugs to treat the symptoms of Sjögren's

syndrome is ongoing.

Treatment for whole-body (systemic) problems

Medications such as nonsteroidal anti-inflammatory drugs (NSAIDs),

hydroxychloroquine sulfate (Plaquenil), or corticosteroids (such as

prednisone) are sometimes used to treat joint pain, chronic

inflammation in saliva and tear glands, or other more serious

symptoms that have not responded to other treatment measures.

If you have additional symptoms associated with another autoimmune

disease (connective tissue disorder), other treatment may also be

needed. See the topics Lupus and Rheumatoid Arthritis for specific

information.

Who to see

Since symptoms from Sjögren's can affect many different body systems,

you may want to work with several different doctors who specialize in

the following areas:

A doctor who specializes in treatment of autoimmune disorders and

inflammation of joints and muscles (rheumatologist)

A doctor who specializes in care of the eyes (ophthalmologist),

particularly for your dry eye symptoms and for monitoring potential

damage to your eyes

A doctor who specializes in treatment of the teeth, gums, and mouth

(dentist)

An internist or family practitioner who will treat upper respiratory

tract infections when they occur and will facilitate all care

provided to you