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Patient information: Sjögren’s syndrome (Beyond the Basics)

SJÖGREN'S SYNDROME OVERVIEW

Sjögren’s syndrome is a chronic disease in which the body’s immune system mistakenly attacks glands that produce moisture in the eyes, the mouth, and elsewhere in the body. The most common symptoms of Sjögren’s syndrome are dry eyes and dry mouth. The symptoms in Sjögren’s syndrome are commonly divided into “benign” and “systemic” (or extraglandular). Although the symptoms are called “benign,” the complaints of dry or painful eyes/mouth, fatigue, and muscle ache are considered by many patients to be their greatest cause of disability.

This type of disease is called an autoimmune disease, meaning that the body’s immune system attacks its own tissues or organs. Sjögren’s syndrome can affect one or more organ systems including the skin, lung, heart, kidney, and nerves. Patients may have anemia, low white blood cell or platelet count, as well as increased risk of developing of lymphoma. Some people have Sjögren’s syndrome as well as another autoimmune condition, such as systemic lupus erythematosus, rheumatoid arthritis, or scleroderma.

Although there is no cure for Sjögren’s syndrome, a number of treatments are available. This article discusses the possible causes, signs, symptoms, diagnostic process, and treatments of Sjögren's syndrome.

SJÖGREN'S SYNDROME CAUSES

Sjögren’s syndrome is thought to be caused by the body’s own immune system. Lymphocytes are a type of white blood cell in the body’s immune system that normally help to protect the body from infection. In Sjögren’s syndrome, these cells are thought to damage the glands that produce tears and saliva.

A person who develops Sjögren’s probably inherits the risk from one or both parents and is then exposed to some type of environmental trigger (eg, a viral infection), but the exact cause is not known (see "Pathogenesis of Sjögren's syndrome"). Thus, both genetic factors and non-genetic factors play a role. Genome sequencing of Sjögren’s syndrome patients in both the US and Asia has added greatly to our understanding of the genetic factors by identifying at least five different risk-related major gene regions.

SJÖGREN'S SYNDROME SYMPTOMS

The classic symptoms of Sjögren’s syndrome are dry mouth (due to decreased production of saliva) and dry eyes (due to decreased production of tears). Symptoms of Sjögren’s syndrome can develop in otherwise healthy people, especially older adults. (See "Clinical manifestations of Sjögren's syndrome: Exocrine gland disease" and "Clinical manifestations of Sjögren's syndrome: Extraglandular disease".)

As noted in the overview, Sjögren’s syndrome can affect one or more organ systems including the skin, lung, heart, kidney, and nerves. Patients may have anemia, low white blood cell or platelet count, as well as increased risk of developing of lymphoma.

SJÖGREN'S SYNDROME DIAGNOSIS

The most prominent symptoms of Sjögren’s syndrome (eye and mouth dryness) are common and can be caused by conditions other than Sjögren’s syndrome. Therefore, it is important to identify medications or conditions that cause dryness and to determine if alternate non-drying treatments are available.

There are two different diagnostic criteria systems that have been proposed. For the past 20 years, the most commonly used diagnostic system has been the “European-American Consensus Criteria” (AECC). A newer criterion called “SICCA” (related to dryness symptoms) has been proposed to the American College of Rheumatology (ACR). Both sets of criteria are very similar (about 90 percent of patients who fulfill one criteria set also fulfill the other criteria set). However, there are about 10 percent of patients who fill one criteria set but not the other criteria set. Committees are currently trying to resolve the differences in diagnostic criteria, since the presence of multiple criteria systems is causing confusion in the clinical, research, and regulatory environments.

The definition of Sjögren’s syndrome requires that the person have symptoms for a prolonged time (eg, dry mouth for greater than three months) and also requires positive laboratory tests. (See "Diagnosis and classification of Sjögren's syndrome".) The older AECC criteria continue to be used until the new consensus criteria are adopted.

Blood tests — A number of blood tests are typically done in people suspected of having Sjögren’s syndrome. One of the most important is a test for the presence of certain antibodies that are markers for autoimmune disorders. These include the anti-nuclear antibody (ANA) and the Sjögren’s-associated SS-A and SS-B antibodies. Rheumatoid factor may also be present. (See "Patient information: Antinuclear antibodies (ANA) (Beyond the Basics)".)

Salivary gland testing — A salivary gland biopsy may be recommended to aid in the diagnosis of Sjögren’s syndrome. The biopsy is done by removing a small piece of tissue from the inner portion of the lip. Other salivary gland tests may also be recommended. Salivary gland flow rates also may be tested by several different methods. The most common is measurement of the volume of expectoration of saliva into a pre-weighed cup. Nuclear medicine methods that quantitate the uptake and excretion of a specific substance (technetium) are more accurate but more expensive. Methods using ultrasound appear very promising and may provide a noninvasive method to follow clinical status.

Eye tests — Tests are usually recommended to determine if you produce a normal amount of tears and to determine if there are areas of the eye that have been damaged as a result of dryness. An ophthalmologist (eye specialist) or a rheumatologist (autoimmune disease specialist) may perform these tests.

Schirmer test – In the Schirmer test, a small piece of sterile filter paper is inserted gently between the eye and eyelid in the inner corner of the eye. It is removed after several minutes, and the wetness on the paper is then measured. A decreased amount of wetting is characteristic of Sjögren’s syndrome, although decreased tear production can also occur with other conditions.

Lissamine green test – The dry eye of Sjögren’s syndrome can show damage to the membranes surrounding the eye and eyelids. A test called the Lissamine green test can detect scratches on the surface of the eye.

SJÖGREN'S SYNDROME COMPLICATIONS

The decreased fluid production in the eyes and mouth can lead to additional problems.

Damage to the surface of the eye can occur when the eyes lack the natural lubricating layer.

Injury to the normally transparent cornea can interfere with vision and can cause eye pain.

People with decreased saliva production are at risk of developing cavities in the teeth and infections in the mouth, including painful fungal infections (a yeast infection or thrush).

People with Sjögren’s syndrome have a higher risk of developing diseases of the chest (called interstitial pneumonitis), diseases of the kidneys (interstitial nephritis), and thyroid gland abnormalities.

Some people with Sjögren’s syndrome may develop inflammation of blood vessels (vasculitis). Vasculitis can cause bleeding and pain and can lead to skin, nerve, and/or internal organ damage. (See "Patient information: Vasculitis (Beyond the Basics)".)

Sjögren’s also increases the risk of a cancer of the lymphatic system (such as non-Hodgkin lymphoma). The lymphatic system includes the tissues and organs that produce and store cells that fight infection, including the bone marrow, spleen, thymus, and lymph nodes. (See "Clinical manifestations of Sjögren's syndrome: Extraglandular disease" and "Patient information: Diffuse large B cell lymphoma in adults (Beyond the Basics)" and "Patient information: Follicular lymphoma in adults (Beyond the Basics)".)

EXTRAGLANDULAR MANIFESTATIONS

The symptoms above are due to damage to the tear and saliva-producing glands, extraglandular manifestations of Sjögren’s syndrome include:

Skin changes most commonly include dryness (xerosis), vasculitis (particularly small vessel involvement), hyperglobulinemic purpura, or a rash called “erythema annulare.” These rashes may have similarity to those found in patients with systemic lupus or mixed cryoglobulinemia.

Lung and kidney involvement may have features of interstitial lymphocytic infiltrates or systemic lupus erythematosus (SLE)-like features.

Neurologic and gastrointestinal involvement.

Lymphadenopathy and increased frequency of lymphoma. This frequency of “aggressive” lymphocytes is more common in Sjögren’s syndrome than in SLE.

Hematopoietic – including anemia, low white cell and low platelet counts.

A series of 16 domains of disease activity are measured in an index called the ESSDAI.

Relationship between SS and SLE — Both disorders have similar genetic, laboratory profile, and clinical features. The antibodies to SS-A/B are found in Sjögren’s syndrome but are not criteria for SLE. However, the types of rashes, lung involvement, renal involvement, and frequency of lymphoma are also different in Sjögren’s syndrome and SLE. Genome-wide screens have shown a difference between the two disorders. Of importance, the Sjögren’s syndrome patients have an association with “homing receptors” for lymphocytes that may help to explain the tissue preferential (organ specific) involvement, as well as the increased frequency of lymphomas.

SJÖGREN'S SYNDROME TREATMENT

Treatment of Sjögren’s syndrome can be divided into three basic areas (see "Treatment of dry eyes in Sjögren's syndrome" and "Treatment of dry mouth and other non-ocular sicca symptoms in Sjögren's syndrome" and "Treatment of systemic and extraglandular manifestations of Sjögren’s syndrome"):

Treatment of dry eyes and mouth.

Treatment of problems such as oral yeast infections, eyelid irritation (blepharitis), and acid reflux. These problems can complicate Sjögren’s syndrome and can make the condition less responsive to other therapies.

Treatment of fatigue and/or vague symptoms of poor concentration and of impaired memory (such as fibromyalgia). (See "Patient information: Fibromyalgia (Beyond the Basics)".)

Treatment of systemic manifestations has been the subject of multiple trials, but none the investigational therapies have yet been approved by United States Food and Drug Administration (FDA). Fortunately, trials are continuing to improve both the “benign” and systemic manifestations.

Moisturizing interventions — Most people use artificial tears (eye drops) to treat dry eyes. Many different solutions are available; a clinician can recommend an appropriate choice based upon your pattern of dryness and of fluid production in the eye.

Some people are sensitive to the preservatives found in artificial tears. If burning or itching occurs, a brand with a non-irritating preservative may be tried. Alternately, a preservative-free variety can be used. Eye drops without preservatives come in small, single-dose containers that may be hard for some people with joint and/or vision problems. A prescription eye drop containing cyclosporine, which suppresses part of the local immune reaction, is also available.

At night, an eye ointment may be used to provide moisture. It is important to use only a small amount (about 1/8 inches or 3 mm) of the ointment, because overuse can block the ducts and can lead to a condition called blepharitis. (See 'Blepharitis (eyelid inflammation)' below.)

Preserving natural tears — Various measures can be used to preserve your own tears. Shields can be fitted on the sides of glasses, helping to protect the eye from air and wind, reducing evaporation of tears. Goggles or wraparound sunglasses serve a similar function.

Another approach is a simple procedure called punctal occlusion. In this procedure, an ophthalmologist inserts small plugs into the tear ducts in the corner of the lower eyelid, nearest the nose, where the tears normally collect and drain into the nose. By blocking this duct, your tears stay on the eye longer. There are several types of plugs, one of which does not touch the surface of the eyeball; these plugs are generally preferred.

Stimulating saliva — Simply sucking on sugarless candy or dried fruit slices (eg, peaches or nectarines) can stimulate the flow of saliva in many people. Citrus-flavored sugarless tablets and sugar-free chewing gum may also be helpful. In some people, medications (eg, pilocarpine or cevimeline) can be given to increase saliva production.

Replacing secretions in the mouth — Sipping on water throughout the day is an easy and effective treatment of dry mouth for many people. The water does not have to be swallowed. It can be rinsed around the mouth and can then be spit out.

If this is not effective, an artificial saliva product (spray or lozenge) may be helpful. If painful gums are a problem, a gel that relieves dry mouth can be helpful.

Avoiding cavities — People with Sjögren’s syndrome are at increased risk for dental cavities. You should brush and floss after eating meals and snacks. You should visit your dentist at least every six months for a cleaning and evaluation by a dentist.

Toothpastes designed specifically for patients with dry mouth are available. These lack the detergents that are present in many types of toothpaste, which can irritate a dry mouth. Toothbrushes with special features that help clean between the teeth (including electric toothbrushes) may also help to keep the teeth clean.

Toothpaste with fluoride (or a special fluoride rinse or varnish) may help to prevent cavities. A fluoride treatment after each dental cleaning may also be helpful.

Dryness in other areas — People with Sjögren’s syndrome may have dryness in other areas, including the lips, the skin, and the vagina. Dry lips may require petroleum jelly or lip salves. Dry skin usually improves with frequent and liberal use of a moisturizing cream or ointment. Application of lotions and lip balms: “extra dry skin” lotion: morning and bedtime (and after baths or showers). Also, purchase multiple lip balms when on sale to avoid last-minute, costly prices at the market check-out line, and keep a lip balm in your purse, desk, and car.

Some women with Sjögren’s syndrome have difficulty with vaginal dryness, especially after menopause. There are several products specifically designed for vaginal dryness, including vaginal moisturizers, estrogen cream, vitamin E oil, and vaginal lubricants. Adequate artificial lubrication applied to BOTH partners can prevent painful intercourse. Talk to your healthcare provider for specific recommendations. (See "Patient information: Vaginal dryness (Beyond the Basics)".)

Treating other Sjögren’s-related problems

Fungal infections in the mouth — Prescription medications are available to treat painful mouth lesions due to oral candidiasis (yeast infection). Sugar-free products (certain vaginal troches or lozenges are safe for oral use) are often used. If you wear dentures and develop an infection, you should disinfect the dentures overnight during your treatment.

Dry nose — It is important to treat dry nose or stuffiness because blocked nasal passages can increase mouth breathing and can worsen dry mouth. Saline nasal sprays are available in most drugstores.

Other causes of nasal blockage, including allergy or sinus infection, should be treated promptly. (See "Patient information: Allergic rhinitis (seasonal allergies) (Beyond the Basics)" and "Patient information: Nonallergic rhinitis (runny or stuffy nose) (Beyond the Basics)".)

Blepharitis (eyelid inflammation) — Eyelid inflammation, also called blepharitis, causes symptoms that are similar to those of dry eye (swollen lids and redness of the inside of the lids). Gently washing the skin of the eyelids can relieve blepharitis. This can be done with a warm, wet washcloth and with a small amount of “no tears” shampoo or non-soap face cleanser. With the eyes closed, the excess debris should be rubbed from the inner eye outward to the outer eye area. (See "Blepharitis".)

Reflux (heartburn) — Acid reflux is more common in people with Sjögren’s syndrome. This is probably due to the decreased production of saliva, which normally helps to reduce the acidity of stomach acid. Treatment of reflux in people with Sjögren’s syndrome is similar to treatment in other people. (See "Patient information: Acid reflux (gastroesophageal reflux disease) in adults (Beyond the Basics)".)

Joint and muscle pain — Nonsteroidal antiinflammatory drugs (NSAIDs) such as ibuprofen are recommended as treatment for the joint pains that may accompany Sjögren’s syndrome. (See "Patient information: Nonsteroidal antiinflammatory drugs (NSAIDs) (Beyond the Basics)".)

Low-dose glucocorticoids (also called steroids) such as prednisone may improve joint pain. However, glucocorticoids are generally recommended for short-term treatment because of serious side effects with long-term use (eg, weight gain, high blood pressure, diabetes, bone thinning).

A class of medications called disease-modifying drugs (DMARDs) is commonly used in people with lupus and rheumatoid arthritis to slow the immune system’s destructive effects. Similar treatments have been used in patients with Sjögren’s syndrome. (See "Patient information: Disease-modifying antirheumatic drugs (DMARDs) (Beyond the Basics)" and "Treatment of systemic and extraglandular manifestations of Sjögren’s syndrome".)

Fatigue — Fatigue is common in Sjögren’s syndrome. Fatigue may be due to the active inflammation associated with the disease itself, fibromyalgia which occurs with high frequency in Sjögren’s syndrome, and sleep disturbances, which can occur if you drink a lot of water to treat dry mouth and then need to get up to urinate frequently at night.

General treatment for fatigue includes the attention to diet and exercise that has been helpful in fibromyalgia patients. In addition in Sjögren’s syndrome patients, it is important to adequately control symptoms of dry mouth and dry eye that interfere with sleep (table 1). (See "Patient information: Insomnia (Beyond the Basics)".)

Fibromyalgia — Some people with Sjögren’s syndrome also have a condition called fibromyalgia. Fibromyalgia causes muscle aching and fatigue. The treatment of fibromyalgia is discussed separately. (See "Patient information: Fibromyalgia (Beyond the Basics)".)

Vasculitis — Vasculitis is inflammation of blood vessels. Damage to arteries or veins may result in bleeding, pain, and damage to skin, nerves, and internal organs. When vasculitis occurs, it often requires treatment with drugs that suppress the immune system. Medications such as cyclophosphamide, azathioprine, or mycophenolate mofetil may be prescribed by clinicians experienced in their use. Careful monitoring for side effects and for response to treatment is necessary. (See "Patient information: Vasculitis (Beyond the Basics)".)

Anesthesia and Sjögren's syndrome — If you need surgery for any reason, you should be certain that the anesthesiologist is aware of your diagnosis; Sjögren’s syndrome can increase the risks of general anesthesia. There may be an increased risk of developing mucous plugs in your airways during and after surgery, and medications used during the surgery can dry the airways further. If aware of the diagnosis of Sjögren’s syndrome, the anesthesiologist can take special measures to lower the risk of these complications.

WHERE TO GET MORE INFORMATION

Your healthcare provider is the best source of information for questions and concerns related to your medical problem.

This article will be updated as needed on our web site (www.uptodate.com/patients). Related topics for patients, as well as selected articles written for healthcare professionals, are also available. Some of the most relevant are listed below.

Patient level information — UpToDate offers two types of patient education materials.

The Basics — The Basics patient education pieces answer the four or five key questions a patient might have about a given condition. These articles are best for patients who want a general overview and who prefer short, easy-to-read materials.

Patient information: Sjögren’s syndrome (The Basics)
Patient information: Antinuclear antibodies (The Basics)

Beyond the Basics — Beyond the Basics patient education pieces are longer, more sophisticated, and more detailed. These articles are best for patients who want in-depth information and are comfortable with some medical jargon.

Patient information: Antinuclear antibodies (ANA) (Beyond the Basics)
Patient information: Diffuse large B cell lymphoma in adults (Beyond the Basics)
Patient information: Follicular lymphoma in adults (Beyond the Basics)
Patient information: Fibromyalgia (Beyond the Basics)
Patient information: Vaginal dryness (Beyond the Basics)
Patient information: Allergic rhinitis (seasonal allergies) (Beyond the Basics)
Patient information: Nonallergic rhinitis (runny or stuffy nose) (Beyond the Basics)
Patient information: Acid reflux (gastroesophageal reflux disease) in adults (Beyond the Basics)
Patient information: Nonsteroidal antiinflammatory drugs (NSAIDs) (Beyond the Basics)
Patient information: Disease-modifying antirheumatic drugs (DMARDs) (Beyond the Basics)
Patient information: Insomnia (Beyond the Basics)

Professional level information — Professional level articles are designed to keep doctors and other health professionals up-to-date on the latest medical findings. These articles are thorough, long, and complex, and they contain multiple references to the research on which they are based. Professional level articles are best for people who are comfortable with a lot of medical terminology and who want to read the same materials their doctors are reading.

Diagnosis and classification of Sjögren's syndrome
Clinical manifestations of Sjögren's syndrome: Exocrine gland disease
Clinical manifestations of Sjögren's syndrome: Extraglandular disease
Interstitial lung disease associated with Sjögren's syndrome: Clinical manifestations, evaluation, and diagnosis
Pathogenesis of Sjögren's syndrome
Renal disease in Sjögren's syndrome
Treatment of dry eyes in Sjögren's syndrome
Treatment of dry mouth and other non-ocular sicca symptoms in Sjögren's syndrome
Treatment of systemic and extraglandular manifestations of Sjögren’s syndrome

The following organizations also provide reliable health information.

National Library of Medicine
(www.nlm.nih.gov/medlineplus/healthtopics.html)

National Institute of Neurological Disorders and Stroke
(www.ninds.nih.gov/disorders/sjogrens/sjogrens.htm)

Sjögren's Syndrome Foundation, Inc
(www.sjogrens.org/)

The Arthritis Foundation
(http://www.arthritis.org/conditions-treatments/disease-center/sjoumlgrens-syndrome/)

American College of Rheumatology
phone: 404-633-3777
(www.rheumatology.org)

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Literature review current through: Sep 2014. | This topic last updated: Jul 17, 2014.
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