Sjogren's syndrome

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Sjogren's syndrome

What is Sjogren's syndrome?

Sjogren's syndrome is a chronic autoimmune disorder that involves the glands in the eyes and mouth. This results in chronic dryness in these areas. This disorder is frequently seen together with other autoimmune diseases.

Who can develop sjogren's syndrome?

As many as 3 million Americans are plagued with sjogren's syndrome. Women are more prone to developing this condition, especially older women around the age of 45-55. It can also occur at any age and affects any race. Sjogren's syndrome usually occurs in conjunction with other autoimmune disorders- the most common being rheumatoid arthritis or lupus. About half of the patients with sjogren’s syndrome have one of these other autoimmune diseases as well.

Where does Sjogren's syndrome occur?

Dryness due to Sjogren’s syndrome can occur in areas all throughout the body. Some examples include:

  • Mouth
  • Eyes
  • Nose
  • Throat
  • Vagina
  • Skin

Other areas where Sjogren’s syndrome can affect the body are:

  • Joints
  • Thyroid
  • Kidneys
  • Liver
  • Lungs
  • Nerves

What are the different types of Sjogren's syndrome?

  • Primary- not caused by any other underlying medical condition and develops primarily on its own.
  • Secondary- associated with an underlying autoimmune condition, like rheumatoid arthritis.

Causes

What is the cause of Sjogren's syndrome?

Sjogren's syndrome is an autoimmune disease in which the body attacks its own healthy cells due to chronic inflammation- in this case, the glands. The tear system in our eyes and salivary glands in the mouth are damaged. It is not completely clear why this happens, but there are several risk factors.

What risk factors are there for developing Sjogren's syndrome?

Some risk factors for Sjogren's syndrome include:

  • Autoimmune disease- The chances of developing Sjogren’s syndrome increase when other autoimmune disorders occur.
  • Sex- Women represent the majority of Sjogren's syndrome cases. This may be related to female-specific hormones.
  • Age- This illness is more common in the middle-aged demographic. 
  • Genetics- People with a family history of Sjogren's syndrome develop this condition more than those without a family history.
  • Environmental- Research shows there may be environmental triggers that pose concern for the development of this condition. Some triggers include:
  1. Viruses
  2. Bacteria
  3. Stress

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Symptoms

What are the symptoms of Sjogren's syndrome?

The symptoms associated with Sjogren's syndrome can vary from person to person. As previously mentioned, Sjogren's syndrome usually involves dry eye and dry mouth symptoms. However, there are many other symptoms one should be aware of when suspecting this disease. Other examples of Sjogren's syndrome symptoms include:

  • Muscle pain
  • Joint pain
  • Fatigue
  • Sense of taste affected
  • Rash
  • Vaginal dryness
  • Dental cavities
  • Tooth loss
  • Gingivitis
  • Thrush
  • Redness and burning sensations in eyes
  • Blurred vision
  • Enlarged salivary glands
  • Difficulty chewing, speaking or swallowing
  • Dry skin
  • Pruritus 
  • Dry cough
  • Hearing difficulties
  • Loss of balance
  • Acid reflux
Can serious health problems arise from Sjogren's syndrome?

In very rare cases, Sjogren’s can be responsible for the development of lymphoma, which is a cancer of the lymph nodes. Some numbness, tingling, or burning symptoms may occur in the hands and feet, also known as peripheral neuropathy. The ongoing chronic inflammation associated with Sjogren’s syndrome may also be responsible for the development of pneumonia, hepatitis, or problems with the kidneys.

Diagnosis

How is Sjogren's syndrome diagnosed?

A thorough medical history and physical examination are always recommended when there is a concern for Sjogren's syndrome. Your healthcare provider may ask about your previous medical history to determine if Sjogren's syndrome is related to other autoimmune disorders or from side effects of certain medications. Physical examination may also involve an eye examination from a specialist.

Are there any diagnostic tests that can be performed to confirm Sjogren's syndrome?

There are a variety of diagnostic tests available to help with the confirmation of Sjogren's syndrome. However, there is not one single test that will confirm this condition. Some tests that may be ordered include:

  • ANA (antinuclear antibodies)- an antibody test that tests for antibodies present in the majority of lupus patients. If positive, it may also help diagnose lupus which may be the underlying cause for Sjogren's syndrome.
  • Anti-SSA antibodies (anti-Sjogren's syndrome or anti-Ro) - specific blood test for Sjogren’s
  • Anti-SSB antibodies (anti-Sjogren's syndrome type B or anti-La) - specific blood test for Sjogren’s
  • Rheumatoid factor-  an antibody test that tests for antibodies commonly seen in rheumatoid patients. If positive, it may confirm rheumatoid arthritis as an associated condition of Sjogren’s.
  • X Rays- called sialometry, measures the amount of saliva produced after being injected with a special dye.
  • Eye examination- used to measure tear production (Schirmer tear test) and clinical findings for any eye changes.
  • Biopsy- a lip biopsy may show specific changes of Sjogren’s when other tests are negative.

Treatment

What are treatment options for Sjogren's syndrome?

As with many autoimmune disorders, there is no cure for Sjogren’s syndrome. Most treatments help manage the symptoms associated with this condition. Less aggressive treatments may consist of remedies such as eye drops, drinking water, and humidifiers, while more aggressive treatments include oral or topical medications. Here are the different treatment options currently available for dry eye symptoms from Sjogren's syndrome: 

  • Artificial tears and ointments- commonly found over the counter to help alleviate the symptoms from dry eyes.
  • Prescription eye drops- usually used to help reduce irritation from chronic inflammation of the tear glands. It is also helpful with improving tear production. 
  1. Cyclosporine (Restasis)
  2. Lifitegrast (Xiidra)
  • Punctal plugs- is a procedure where an ophthalmologist inserts small silicone plugs into the tear ducts to help with tear production.
  • Autologous serum drops- personalized eye drops are made by mixing your blood serum with a mixed sterile solution.

Here are treatment options available for the treatment of dry mouth associated with Sjogren's syndrome:

  • Artificial saliva producers- common over-the-counter products include gum or candies with sorbitol or xylitol to help produce saliva.
  • Prescription saliva producers- help produce saliva from the use of medication naturally. 
  1. Pilocarpine (Salagen)
  2. Cevimeline (Evoxac)

Treatment options available for vaginal dryness include:

  • Vaginal lubricants/moisturizers- usually found over the counter to add needed moisture to the vagina daily.
  • Vaginal estrogen therapy- prescription therapy for vaginal dryness.

If medications fail, what are other treatment options available?

Surgery may be considered for managing Sjogren's syndrome symptoms when medications or punctal plugs fail to relieve symptoms. The goal of surgery is to permanently close tear ducts. 

Are there any preventative treatments that should be considered for Sjogren's syndrome?

Due to the symptoms of Sjogren’s syndrome, wellness visits with your dentist and ophthalmologist are imperative. Dry mouth places people at increased risk of developing cavities and tooth decay, so regular examination and treatments are needed. Avoiding scented soaps, detergents, and perfumes are also helpful in preventing symptoms of vaginal dryness from Sjogren's syndrome. Lastly, ophthalmology exams are crucial for preventing eye complications such as permanent damage to the cornea due to chronic inflammation and dryness. 

Sjogren's syndrome can have many symptoms associated with the disorder. The good news is although there is no cure, the condition is very manageable. If you feel you may have Sjogren’s syndrome, the first step is to get an accurate diagnosis. Our healthcare providers at TeleMed2U may be able to help you learn more about managing your symptoms.

For more information about living well with Sjogren’s, please visit the Sjogren’s Foundation website at: https://www.sjogrens.org/living-with-sjogrens

Information

Medically reviewed by:

Dr Roy Kedem, MD

Dr Zenon Andreou studied medicine at University College London, graduating in 2006. His postgraduate training was in hospitals in and around London and he trained for four years in Otolaryngology before completing his training in General practice

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