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Sjögren's Syndrome

What is Sjögren's Syndrome?

Sjogren’s syndrome is characterized by dry eyes, mouth, or other body parts. It is an autoimmune disorder where the immune system mistakenly attacks the affected individual’s glands that produce tears (lacrimal glands), saliva (salivary glands), or other fluid secreting glands; preventing these glands from secreting fluids. It causes dryness in areas that need moisture, including the nose, throat, and skin.

 

Synonyms

  • Dacryosialo-adenopathia Atrophicans
  • Gougerot-Houwer-Sjogren Syndrome
  • Gougerot-Sjogren Syndrome
  • Keratoconjunctivitis sicca
  • Keratoconjunctivitis sicca-Xerostomia
  • Secreto-inhibititor-xerodermostenosis
  • Sicca Syndrome
  • Sjogren-Gougerot Syndrome

Sjogren’s syndrome is characterized by dry eyes, mouth, or other body parts. It is an autoimmune disorder where the immune system mistakenly attacks the affected individual’s glands that produce tears (lacrimal glands), saliva (salivary glands), or other fluid secreting glands; preventing these glands from secreting fluids. It causes dryness in areas that need moisture, including the nose, throat, and skin.

Acknowledgement of Sjögren's Syndrome has not been added yet.

Sjogren’s syndrome is most common in adults and women, primarily 40 years or older. It can occur at any age, but it is rarest in children. Women are ten times more likely to be affected. It is unknown why women are more affected, but it could be due to the effect hormones have on the immune system.

Affected individuals that have a family history of autoimmune diseases, such as lupus, have a higher chance of developing Sjogren’s.

It is difficult to determine an exact and accurate prevalence rate because the characteristics or symptoms of Sjogren’s syndrome can be associated with other conditions or disorders.

Name Abbreviation
Dacryosialo-adenopathia Atrophicans Sjogren´s Syndrome
Gougerot-Houwer-Sjogren Syndrome Sjogren´s Syndrome
Gougerot-Sjogren Syndrome Sjogren´s Syndrome
Keratoconjunctivitis sicca Sjogren´s Syndrome
Keratoconjunctivitis sicca-Xerostomia Sjogren´s Syndrome
Secreto-inhibititor-xerodermostenosis Sjogren´s Syndrome
Sicca Syndrome Sjogren´s Syndrome
Sjogren-Gougerot Syndrome Sjogren´s Syndrome

Normally, the immune system fights off infections of foreign materials, such as viruses and bacteria. In an autoimmune disorder, the immune system thinks the body is foreign material by mistake and attacks the body.

Sjogren’s syndrome is an autoimmune disorder. The body thinks its glands or organs are foreign material and attack them. This causes fluid producing glands to not be able to secrete fluids. It is also a rheumatic disease, affecting joints, tendons, ligaments, bones, and muscles.

Sjogren’s syndrome is classified as primary Sjogren’s syndrome (SS) or secondary SS. Primary SS is when the condition exists by itself and is not caused by a preexisting disease. Secondary SS is when the condition develops as a complication of a preexisting connective tissue disease, such as rheumatoid arthritis, lupus, scleroderma, or myositis/ polymyositis (inflammation of muscles or groups of muscles).

The underlying cause of SS is unknown. It can be a combination of environmental and genetic factors, or secondary to a condition. Environmental factors that have been associated to SS are exposure to virus or bacteria, which causes the activation of the immune system.

Women might be more affected because estrogen and/or hormones play a role in the function of the immune system. Post-menopausal women might be more affected because estrogen levels are low.

 

Symptom severity varies from person to person. Some affected individuals may have a mild disease where it only affects the eyes and mouth. Others may have very severe and chronic symptoms. Some also go into remission.

Eyes are the major symptom manifestation of primary and secondary SS. The eyes are extremely dry, causing itching, burning, blurry vision, light sensitivity, inflammation, redness, and a sandy or gritty feeling, especially in the morning. Light sensitivity is caused by a loss of cells that line the conjunctiva, called keratoconjunctivitis sicca. The conjunctiva is the mucosal membrane that covers the front and lines the inside of the eyes.

The mouth is the second most affected part of the body. The mouth is extremely dry, causing chalky or cotton mouth, thick stringy saliva, difficulty tasting food, burning and cracking of the tongue, and swollen salivary glands.  The parotid glands are major salivary glands located behind the jaw. In primary SS, less common in secondary SS, the parotid glands and other salivary glands are enlarged and cannot produce saliva. Saliva helps protect the teeth and tissues of the mouth. Without saliva, painful mouth sores, yeast infection, and tooth decay or cavities can occur. Some signs of mouth infections are burning or painful sores, and red and/or white patches.

The nose and throat airways are affected as well. The glands of the nose and throat can become dry, causing dry cough, dry nose, hoarseness, difficulty speaking, and difficulty swallowing or eating.

Other symptoms include vaginal dryness in the genital area, dry skin or rashes, poor concentration or memory, and vasculitis or inflammation of the blood vessels.

Other affected organs are the lungs, the kidneys, the thyroid gland, the digestive tract, and lymph nodes. The affected digestive organs can cause heartburn. The lymph nodes can be swollen; a few individuals can develop lymphomas.

Most affected individuals with primary SS, and less commonly in secondary SS, have symptoms that occur outside of mucosal or fluid glands, called extra-glandular symptoms. Extra-glandular symptoms include swollen painful joints, muscle pain and/or weakness, low-grade fevers, numbness or tingling of the nerves in the arms and legs, and fatigue or tiredness.

 

Early diagnosis is important because it can help decrease the effect on the eyes, mouth, and other body parts. Sjogren’s syndrome main diagnostic criteria are dry mouth, dry eyes, and/or blood testing positive for antinuclear antibodies and/or rheumatoid factor.  

A physical examination checks for changes in the eyes, mouth, salivary glands, joint inflammation or pain, muscle weakness, and swelling of the lymph nodes.

A blood test tests for lab abnormalities, such as positive for antinuclear antibodies, positive for rheumatoid factor, elevated protein levels, low albumin levels, and elevated erythrocyte sedimentation rate.

Some genes are common in primary SS; genetic testing can test for histocompatibility antigens, HLA-B8 and HLA-DR3

A physical examination can be performed.

A blood test checks for the presence of antinuclear antibodies and rheumatoid factor, which are indications of the body attacking itself. Elevated protein levels, low albumin levels, and elevated erythrocyte sedimentation rate are all indications of inflammation and an activated immune system.

An eye exam by ophthalmologist called a Schirmer’s test determines how dry the eyes are by placing a piece of filter paper on the corner of the eye and measuring the degree of wetness after five minutes. This indicates how well the eyes are producing tears. Another test is called the slit lamp test.  It checks the surface of the cornea for dryness and damage. Rose-Bengal staining test shows if there is inflammation present in the cornea.

A saliva test measures saliva output and flow rates to determine how well the salivary glands are producing saliva. It also indicates whether inflammation is present. A biopsy of the lip or parotid gland can confirm diagnosis, if necessary.

Genetic testing tests for presents of HLA genes or histocompatibility antigens.

Treatment for Sjogren’s syndrome helps manage symptoms. Some affected individuals can achieve remission. Since Sjogren’s syndrome is an issue with dryness, most treatment options help relieve dryness.

It is very important for affected individuals to work with a healthcare team: the primary care physician, a rheumatologist, an ophthalmologist, an otolaryngologist (ears, nose, and throat specialist), a dentist, and a pharmacist.

For dry eyes, eyedrops or eye pellets can help keep the eyes moist. Cyclosporine (Restasis) is an eye emulsion and hydroxypropyl cellulose (Lacrisert) are slow release inserted into the eyes. Prescription or over-the-counter artificial tears (Refresh, TheraTears, GenTeal, FreshKote) can help keep the eyes moist and redistribute the moisture. In extremely severe cases, a surgery called punctal occlusion may be necessary. It keeps the tears from draining out of the eyes and retains natural moisture by inserting punctal plugs made of silicone or collagen into the tear drainage area of the eyes.

For inflamed eyes, anti-inflammatory eyedrops called NSAIDs can be used.

For dry mouth, pilocarpine tablets or cevimeline capsules help control symptoms of dry mouth by stimulating the salivary glands. These medications pass through the body quickly and can only control symptoms for several hours; several doses a day may be required. Affected individuals with asthma, glaucoma, or prostate disorder should discuss with their healthcare team if it is safe and appropriate to take these medications.

Saliva substitutes or mouth gels can be helpful especially at night when the mouth is drier. There are over-the-counter options such as Glandosane (artificial saliva spray), MedActive (oral lozenges), Moi-Stir (moisturizing mouth spray), or Salivart (moisturizing mouthwash). Some prescription options are Aquoral (mouth spray) or NeurtralSal (mouthwash).

Increasing fluid intake during meals and in between meals helps keep the mouth moist.

For cracked or chapped lips, oil-based lip balm, Aquaphor, or Vaseline can be used.

Fungal or yeast infection of the mouth can be treated with antifungal tablets, lozenges, or liquid solution.

For dry nose, throat, and upper airways, water or saline nose sprays are helpful during the daytime and nasal gels can be used at night. Some areas or weather causes dry air, using a humidifier, especially when sleeping, can help.

For vaginal dryness, a water-based lubricant helps with dryness and painful intercourse. Avoid oil-based lubricants. Estrogen creams may be helpful.

For extra-glandular symptoms, anti-inflammatory drugs called NSAIDs or steroids can help control inflammation. Antirheumatic drugs called DMARDs can help suppress the body’s immune system, such as hydroxychloroquine, azathioprine, cyclosporine, methotrexate, or injectable biologics.

 

Affected individuals with mild to moderate symptoms such as dry eyes and dry mouth do not have decreased life expectancy or decrease in general health. Symptoms of secondary SS is milder. Primary SS have a higher chance of developing extra-glandular symptoms, which can affect strength, flexibility, and mobility.

Affected individuals have an increased risk of developing an infection and an increased risk for dental problems.

Early diagnosis and treatment are essential to prevent damage to organs and other parts of the body.

 

It is important to follow up with the healthcare team on a regular basis.

It is important to use eyedrops on a regular basis. It is best to use preservative-free eyedrops because preservatives can cause more dryness and irritation. Practice blinking at least 5-6 times a minute. Limit time using a computer or phone. Wear sunglasses to prevent moisture loss from the eyes. Protect the eyes from wind, fans, or breeze; this can dry out the eyes. Avoid smoke, second-hand smoke, and smoky rooms. Be careful with eye makeup, avoid use if possible.

It is important to maintain oral hygiene by having frequent dental checkups, using an electric toothbrush, and flossing regularly, especially after meals. It is recommended to take small sips of water throughout the day, chew on sugar-free gum, or suck on sugar-free hard candy or lozenges to stimulate saliva flow. Avoid sugar containing products, artificial sweeteners are preferred because they do not cause tooth decay. Ask the dentist for recommendation on fluoride products to protect the teeth.

It is important to perform safe low-impact exercises to relieve joint stiffness and improve movement. However, if the joints are in swollen and painful, it is recommended to rest and ice the joints.

It is important to protect the vocal cords and prevent hoarseness. Avoid straining the vocal cords or clearing the throat before speaking.

Some medications can cause dryness. Speak with a pharmacist or doctor about which medications can cause dryness. Do not stop taking any of your medications without asking your doctor. Some over-the-counter medications that can cause dryness are water-pills, allergy medications, decongestants, antidiarrheal medicines, motion sickness medicines, and sleep-aids.

Sjogren’s Syndrome, American College of Rheumatology, https://www.rheumatology.org/I-Am-A/Patient-Caregiver/Diseases-Conditions/Sjogrens-Syndrome

Sjogren’s Syndrome, Arthritis Foundation, https://www.arthritis.org/about-arthritis/types/sjogrens-syndrome/

Sjogren Syndrome, Genetics Home Reference of the U.S. National Library of Medicine, https://ghr.nlm.nih.gov/condition/sjogren-syndrome  

Sjogren’s Syndrome, National Institute of Arthritis and Musculoskeletal and Skin Disease, https://www.niams.nih.gov/health-topics/sjogrens-syndrome


What You Need to Know About Sjogren’s Syndrome, Lupus Foundation of America, https://resources.lupus.org/entry/what-you-need-to-know-about-sjogrens-syndrome

help Created by jonnell
Last updated 8 Nov 2010, 12:47 AM

Posted by JennyPettit
8 Nov 2010, 12:47 AM

I see that you posted this a long time ago, but I just joined and saw it now. Are you still looking? I'm not a parent, I'm 24, but was diagnosed at 15 and actually trace some symptoms back as far as 2 years old. I've also seen a few parents on other networks, I can try to see if I can come up with anyone. Let me know if you're interested, thanks!

Posted by jonnell
17 Mar 2009, 02:48 PM

im looking to talk with parents of sjogrens syndrome my daughter who is 4 has been diagnosed with it and i cant find anyone with small children with ssj

Adult Stem Cell Therapy Created by skinny13
Last updated 16 Mar 2009, 05:04 AM

Posted by skinny13
16 Mar 2009, 05:04 AM

Here is a list of at least 65 diseases with successful results. http://www.geneforum.org/node/135 Adult stem cell research and therapy has many successful results. Of course there is always the option of a natural botanical that stimulates ones' that enables your body to produce millions more adults stems cells so we can heal ourselves with out invasive therapy. Maureen

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Created by jonnell | Last updated 8 Nov 2010, 12:47 AM

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