Also Known As
Keratoconjuntivitis Sicca
Sicca Syndrome
This article was last reviewed on
This article waslast modified on
October 10, 2017.
What is Sjögren syndrome?

Sjögren syndrome is an autoimmune disorder in which the body's immune system mistakenly reacts to the tissue in glands that produce moisture, such as tear and salivary glands. It is a chronic, inflammatory disease that often progresses to a more complex, systemic disorder that can affect other tissues and organs in the body such as joints, skin, kidneys, lungs, nervous system, and the intestinal tract. Sjögren syndrome is characterized by an unusual accumulation (infiltration) of a particular type of white blood cell, lymphocytes, in the glands that are responsible for fluid production.

Sjögren syndrome affects the amount and quality of saliva and tears produced by glands, leading to distinctive features, including a dry mouth and dry eyes, that are referred to as "sicca syndrome." Other mucous membranes may also become abnormally dry. People with this condition often have a feeling of sand or grit in the eyes, swollen salivary glands, difficulty swallowing, and a decreased sense of taste.

Sjögren syndrome can affect anyone at any age, but the majority of those afflicted are older than 40, and women are nine times more likely than men to have the disorder. It is estimated to be the second most common autoimmune disease, after lupus. 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 trigger such as a bacterial or viral infection may prompt development of Sjögren syndrome in some people who are genetically predisposed to the disorder.

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

  • Primary Sjögren syndrome—develops gradually with salivary and tear gland function worsening over time and without any other underlying disorder
  • Secondary Sjögren syndrome—occurs when a person already has an autoimmune disorder, such as lupus, polymyositis, scleroderma, or rheumatoid arthritis

Complications can develop in relation to Sjögren syndrome and may include salivary gland infections and tumors, dental cavities, damage to the eyes, kidney disease, and lung infections. Pregnant women who have autoantibodies related to Sjögren syndrome may have a higher risk of miscarriage. Sjögren syndrome has also been associated with a higher risk of developing lymphoma.

The Sjögren's Syndrome Foundation says that reaching a diagnosis can be difficult and often takes an average of about 4 years from the onset of symptoms. The American-European Consensus Group and the Sjögren's International Collaborative Clinical Alliance (SICCA) each developed criteria to help health practitioners in making a diagnosis of Sjögren syndrome. The American College of Rheumatology (ACR) adopted the SICCA criteria in 2012. Criteria for diagnosis include, for example, signs and symptoms and positive tests for ANA, anti-SSA and anti-SSB, and/or RF as well as a positive salivary gland biopsy. (See the Tests section for more on these.)

Accordion Title
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 fatigue and fever and may involve various parts of the body such as the skin, joints, lungs, nerves, kidneys, and the digestive tract. Because of this, 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 mouth with difficulty swallowing or talking
    • Dry skin and rashes
    • Fatigue
    • Joint pain and swelling
    • Muscle pain
    • Numbness or tingling in the hands or feet (neuropathy)
    • Sore tongue or throat
    • Swollen salivary (parotid) glands
    • Vaginal dryness
    • Stomach upset, irritable bowel
    • Recurrent bronchitis or pneumonia
  • Tests

    There is no single test that can definitively diagnose Sjögren syndrome. Usually a health 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 make a diagnosis.

    Laboratory tests to detect autoantibodies 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:


    Non-laboratory tests:

    • Mouth (salivary gland) biopsy — a few small salivary glands may be removed surgically from a lip and then examined under a microscope. This is done to look for inflammation (accumulation of white blood cells called lymphocytes) in the gland. This is characteristic 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 eye (cornea or conjunctiva) — to evaluate the extent to which dryness has damaged the surface of the eye
    • Salivary flow — measures the amount of saliva produced over a specific time period
    • Salivary scintigraphy — a nuclear medicine test in which a radioisotope is injected into a vein and tracked for how long it takes to reach salivary glands
  • 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, and mouth lubricants can be used as necessary. In some cases, medications that increase saliva production may be prescribed.
    • Regular dental care and checkups are important as those affected are prone to cavities.
    • 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.


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

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NOTE: This article is based on research that utilizes the sources cited here as well as the collective experience of the Lab Tests Online Editorial Review Board. This article is periodically reviewed by the Editorial Board and may be updated as a result of the review. Any new sources cited will be added to the list and distinguished from the original sources used.

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