SJÖGREN'S SYNDROME OVERVIEW
Sjögren’s syndrome (SS) 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.
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. 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 in not known. (See "Pathogenesis of Sjögren's syndrome".)
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 SS 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".)
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.
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 "Classification and diagnosis of Sjögren's syndrome".)
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. (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.
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 eye specialist (ophthalmologist) or a rheumatologist 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.
- Rose Bengal test — The dry eye of Sjögren’s syndrome can show damage to the membranes surrounding the eye and eyelids. A test called the Rose Bengal 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.
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)".)
Dry eye treatments — 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 wrap-around 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.
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.
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; talk to your healthcare provider for specific recommendations. (See "Patient information: Vaginal dryness (Beyond the Basics)".)
Treating other problems
Fungal infections in the mouth — Prescription medications are available to treat painful mouth lesions due to oral candidiasis (yeast infection). Sugar-free products (vaginal troches or lozenges) 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 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 disease itself or to difficulties staying asleep, which can occur if you drink a lot of water to treat dry mouth and then need to urinate frequently at night.
General treatment for fatigue includes adequately controlling of dry mouth, using a humidifier in the bedroom, and practicing good sleep hygiene. Sleep hygiene uses methods that increase the chances of sleeping at night, including avoiding daytime napping, avoiding caffeine and other stimulants in the evening, observing a period of peaceful relaxation time before sleep, and ensuring that the bedroom is quiet and comfortable (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.
Classification and diagnosis 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.