Sjögren’s syndrome (SS)

Sjögren’s syndrome (SS)

Author
Robert Fox, MD, PhD
Section Editor
Peter H Schur, MD
Deputy Editor
Paul L Romain, MD

Disclosures

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 cause, signs and 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 and prognosis”.)

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 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) as well as 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)”.)

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 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.

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 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), the kidneys (interstitial nephritis), and thyroid gland abnormalities.
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 prognosis” and “Patient information: Diffuse large B-cell lymphoma in adults” and “Patient information: Follicular lymphoma in adults”.)
SJÖGREN’S SYNDROME TREATMENT — Treatment of Sjögren’s syndrome can be divided into three basic areas: (see “Treatment 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 make the condition less responsive to other therapies.
Treatment of fatigue and/or vague symptoms of poor concentration and impaired memory (such as fibromyalgia). (See “Patient information: Fibromyalgia”.).
Moisturizing treatments

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 on your pattern of dryness and 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 preservative 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″ or 3 mm) of the ointment because overuse can block the ducts and 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 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 then 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, 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”.)

Treating other problems

Fungal infections in the mouth — Prescription medications are available to treat painful mouth lesions due to oral candidiasis (yeast infection). 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 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)” and “Patient information: Nonallergic rhinitis (runny or stuffy nose)”.)

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 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”.)

Joint and muscle pain — Nonsteroidal anti-inflammatory 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)”.)

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) are 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)” and “Treatment 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 adequate control 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 no 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”.)

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”.)

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.

 

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:

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

Professional Level Information:

Classification and diagnosis of Sjögren’s syndrome
Clinical manifestations of Sjögren’s syndrome: Exocrine gland disease
Clinical manifestations of Sjögren’s syndrome: Extraglandular disease and prognosis
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 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
(www.arthritis.org/conditions/DiseaseCenter/sjogrens.asp)

American College of Rheumatology
(www.rheumatology.org)

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Ramos-Casals M, Tzioufas AG, Font J. Primary Sjögren’s syndrome: new clinical and therapeutic concepts. Ann Rheum Dis 2005; 64:347.
Kassan SS, Moutsopoulos HM. Clinical manifestations and early diagnosis of Sjögren syndrome. Arch Intern Med 2004; 164:1275.
Fox RI, Stern M, Michelson P. Update in Sjögren syndrome. Curr Opin Rheumatol 2000; 12:391.
Soto-Rojas AE, Kraus A. The oral side of Sjögren syndrome. Diagnosis and treatment. A review. Arch Med Res 2002; 33:95.