Sjögren Syndrome

Also Known As

Keratoconjuntivitis Sicca

Sicca Syndrome

This article was last reviewed on

April 16, 2020.

This article waslast modified on October 28, 2020.

What is Sjögren syndrome?

Sjögren syndrome (shō′grənz sĭn′drōm) is an autoimmune disorder in which the body's immune system mistakenly reacts to the tissues in glands that produce moisture, such as tear (lacrimal) and salivary glands.

It is a long-term (chronic) disorder that causes inflammation. It often progresses to a more complex disorder that can affect many other tissues and organs in your body such as joints, skin, mouth, liver, pancreas, gallbladder, thyroid, cardiovascular, kidneys, lungs, nervous system, sweat glands, and the intestinal tract.
Sjögren syndrome is marked by an abnormal build-up of lymphocytes, a type of white blood cell, in the glands that can produce abnormal autoantibodies.
Sjögren syndrome causes these glands to reduce the amount and quality of saliva and tears, leading to distinctive features of a dry mouth and dry eyes, known as sicca. Other mucous membranes may also become abnormally dry.
People with this condition often have a feeling of sand or grit in the eyes, sensitivity to bright or fluorescent lights, swollen salivary glands, difficulty swallowing, and a decreased sense of taste.
Sjögren syndrome can affect anyone at any age but most often affects people older than age 40. Women are nine times more likely than men to have the disorder, with a predominance in Caucasian women.
According to the National Institute of Neurological Disorders and Stroke (NINDS), between 1 and 4 million people in the United States have Sjögren syndrome.

The cause of Sjögren syndrome is not known, but researchers think it may be a combination of genetic and environmental factors. It is thought that a bacterial or viral infection may trigger the development of Sjögren syndrome in some people who are genetically predisposed to the disorder. In some families, there is a predisposition to develop autoimmune diseases; relatives of those who have Sjögren syndrome may be at an increased risk of developing an autoimmune disease.

The disorder may develop as a primary or secondary disease, with all cases divided roughly in half between the two types:

Primary Sjögren syndrome—develops gradually when your salivary and tear gland functions worsen over time and without any other underlying disorder
Secondary Sjögren syndrome—occurs when you already have another autoimmune disorder, such as systemic lupus, polymyositis, scleroderma, primary biliary cirrhosis, or rheumatoid arthritis

Complications

Complications of Sjögren syndrome may include:

Salivary gland infections and tumors
Dental cavities, periodontal disease
Yeast infection inside the mouth (thrush)
Damage to the eyes
Kidney disease
Lung infections
Higher risk of developing lymphoma
Higher risk of miscarriage in pregnant women who have autoantibodies related to Sjögren syndrome

About Sjögren Syndrome

Signs and Symptoms
The signs and symptoms of Sjögren syndrome can vary from person to person and change in type and severity over time. They may include non-specific symptoms such as chronic coughing, fatigue, and/or fever. Because various parts of your body can be involved, recognizing and diagnosing Sjögren syndrome can sometimes be challenging.

Examples of some of the signs and symptoms include:

Decreased sense of taste and smell

Dry cough

Dry, gritty eyes

Dry or chalky mouth with difficulty swallowing, eating, or talking

Dry skin and rashes

Fatigue

Joint pain and swelling

Muscle pain

Numbness or tingling in the hands or feet (neuropathy)

Raynaud phenomenon

Sore tongue or throat

Swollen salivary (parotid) glands under the tongue, inside the cheeks and jaws

Vaginal dryness

Stomach upset, irritable bowel

Recurrent bronchitis or pneumonia
Testing and Diagnosis
There is no single test that can definitively diagnose Sjögren syndrome.

Because Sjögren syndrome can mimic menopause, certain drug effects, or other autoimmune diseases, establishing a diagnosis can be difficult, often taking several years after the first symptoms appear. According to the Sjögren's Foundation, advancements in recent years in understanding this disease have reduced the time to obtain a diagnosis from 6 years (in 2012) to 2.8 years.

Usually, a healthcare practitioner will order laboratory tests and other types of tests, and the results are considered with other factors such as signs and symptoms and results of a physical exam to help make a diagnosis.

Two groups of experts, the American-European Consensus Group and the Sjögren's International Collaborative Clinical Alliance (SICCA), each developed criteria to help healthcare practitioners make a diagnosis of Sjögren syndrome. The American College of Rheumatology (ACR) also adopted the SICCA criteria. In addition to having typical signs and symptoms, a diagnosis generally requires positive blood tests for one or more autoantibodies (see below) as well as a salivary gland biopsy showing inflammation (clumps of lymphocytes, see below).

Laboratory Tests

A few blood tests may be performed to detect autoantibodies. Autoantibodies are antibodies (immune proteins) that mistakenly target and react with your own tissues or organs. One or more autoantibodies may be produced by your immune system and may be detected in your blood when there is an autoimmune process.

Autoantibody testing for Sjögren syndrome may include:

Antinuclear antibodies (ANA) — a primary test for autoimmune disorders; this test is also positive in most cases of Sjögren syndrome.

Antibodies specific to Sjögren syndrome — Anti-SS-A (also called Ro) and Anti-SS-B (also called La); the tests for these antibodies are often performed as part of an ENA panel and are frequently positive.

Rheumatoid factor (RF) — may be positive

Anti-dsDNA — also used to evaluate people for autoimmune diseases; this test is usually specific for lupus, but low levels may sometimes be seen with Sjögren syndrome.

Other general laboratory tests may include:

C-reactive protein (CRP) — this is the preferred test to detect inflammation. It is usually elevated in autoimmune diseases like Sjögren syndrome.

Alternatively, an erythrocyte sedimentation rate (ESR) may be done to detect inflammation.

Comprehensive metabolic panel (CMP) and Liver panel — evaluate the health of various organs, including the kidneys and liver

Complete blood count (CBC) — may be done to detect anemia, a low white blood count, and/or a low platelet count, which are sometimes seen in this disease

Serum protein electrophoresis (SEP) — elevated amounts of immunoglobulins (hypergammaglobulinemia) may be present in some patients and a decreased amount (hypogammaglobulinemia) may be seen in others. Additionally, monoclonal immunoglobulins and cryoglobulins may be seen in up to 20% of patients.

Non-laboratory Tests:

Lip (salivary gland) biopsy — a few small salivary glands may be removed surgically from the inner, lower surface of the lip and then examined by a pathologist under a microscope. This is done to look for inflammation (clumping of white blood cells called lymphocytes) in the gland. This is a feature of Sjögren syndrome. (For more on biopsies, see the article on Anatomic Pathology.)

Schirmer test — to measure tear production

Rose Bengal or Lissamine Green staining of the eye (cornea or conjunctiva) — to evaluate the eye for dry spots

Salivary flow — measures the amount of saliva produced over a specific time period

Salivary scintigraphy — a nuclear medicine test in which a low-level radioactive marker is injected into a vein and tracked for how long it takes to reach salivary glands

Chest X-rays — to look for inflammation in the lungs
Treatment
There is no cure for Sjögren syndrome. Treatment can vary depending on the severity of symptoms. It is usually aimed at relieving symptoms and preventing or minimizing complications and damage to tissues, such as the surface of the eye.

Dry eyes can be treated with over-the-counter or prescription artificial tears, tear stimulants, and/or thicker eye lubricants. Sometimes plugs may be inserted into the drainage ducts in the eyes to help tears remain on the surface of the eyes.

Dry mouth may be helped by frequent small drinks of water or sugarless chewing gum to stimulate saliva production, with mouth lubricants used as necessary. In some cases, medications that increase saliva production may be prescribed.

Regular dental care and checkups are important as you may be prone to cavities and periodontal disease. Frequent use of oral sprays and mouth rinses are generally necessary.

Joint pain and other arthritis symptoms are treated with anti-inflammatory medicines, such as aspirin and other NSAIDs.

In some severe cases, when internal organs of the body are affected, drugs that dampen the immune system (immunosuppressants) and steroids may be prescribed for flare ups.

Disease-Modifying Anti-Rheumatic Drugs (DMARD) such as hydroxychloroquine affect your immune system functions and may be used for long-term therapy.

Sjögren syndrome usually affects people differently, so it is important to discuss options with your healthcare practitioner to decide on the best treatment plan. (For more on treatment, see the Related Content section.)

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