What is Sjogren's syndrome?

What are the types of Sjogren's syndrome?

What causes Sjogren's syndrome?

What are the symptoms of Sjogren's syndrome?

How is Sjogren's syndrome diagnosed?

How is Sjogren's syndrome treated?

What is Sjogren's syndrome?

Sjogren's syndrome is an auto-immune condition, where white blood cells attack the body's healthy tear and saliva glands, reducing the amount of saliva and tears produced. This causes a dry mouth and dry eyes, along with other related symptoms.

Women are most commonly affected, and the glands responsible for keeping the vagina moist can also be affected by Sjogren's syndrome, meaning vaginal dryness can be another symptom of the condition. 

The tear, saliva and vaginal glands, are collectively known as the exocrine glands and cannot function correctly after being attacked by the immune system. There is also some evidence that the immune system damages the nerves that control these glands, further reducing their effectiveness.

Damage can also be done to other parts of the body, including muscles, joints, blood vessels, nerves and (less commonly) organs.

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Types of Sjogren's syndrome

Similarly to Raynaud's syndrome, Sjogren's syndrome can either be;  

  • Primary Sjogren's - when the condition develops by itself 
  • Secondary Sjogren's - when it develops in combination with another autoimmune condition, such as lupus or rheumatoid arthritis

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What causes Sjogren's syndrome?

The cause of Sjogren's syndrome remains unknown, but research suggests that the condition is triggered by a combination of genetic, environmental and possibly hormonal factors. 

For primary Sjogren's, it is believed some people are born with specific genetic markers that make them more susceptible to the auto-immune condition. Later in life, they experience an environmental factor, such as a virus, which triggers that genetic marker and causes the immune system to work incorrectly. 

The female hormone oestrogen also seems to play a role, as Sjogren's syndrome occurs more frequently in women. With symptoms of primary Sjogren's usually beginning around the menopause, when levels of oestrogen begin to fall, this may indicate a link between a reduction in oestrogen and the normal functioning of the immune system. Exactly if this link exists, or how it occurs, is unclear.

Secondary Sjogren's syndrome occurs alongside another autoimmune condition, such as rheumatoid arthritis or lupus. If you are seeing a specialist already for these conditions, they will be able to diagnose Sjogren's syndrome should you begin to experience symptoms of dry mouth and eyes.

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What are the symptoms of Sjogren's syndrome?

The most commonly reported symptoms of Sjogren's syndrome are a dry mouth and dry eyes, both of which can lead to other associated symptoms in the eyes and mouth.

Many women also experience dryness of the vagina, which can make sexual intercourse painful.

Associated symptoms of dry mouth

  • tooth decay, leading to an increased risk of tooth loss
  • dry cough
  • difficulty swallowing and chewing
  • hoarse voice
  • difficulty speaking
  • swollen salivary glands (located between your jaw and your ears)
  • repeated fungal infections of your mouth (oral thrush), symptoms of which include the appearance of white, cream-coloured or yellow spots on the inside of your mouth and tongue

Associated symptoms of dry eyes

  • a burning or stinging sensation in your eyes
  • itchy eyes
  • a feeling that there is a piece of sand or gravel in your eyes
  • irritated and swollen eyelids
  • sensitivity to light (photophobia)
  • tired eyes
  • a discharge of mucus from your eyes

Symptoms can become worse when you are in a windy or smoky environment. Air-conditioned buildings and travelling on aeroplanes can also make symptoms worse.

Other symptoms of Sjogren's syndrome

In more serious cases of Sjogren's syndrome, the immune system can attack other parts of the body as well as the tear, saliva and vaginal glands, causing a wide range of symptoms such as:

  • dry skin
  • fatigue
  • muscle pain
  • joint pain, stiffness and swelling
  • pain and numbness in certain parts of the body, usually the arms or legs (periphery neuropathy)
  • restricted blood flow to the hands, which can cause the hands to feel cold, numb and painful (Raynaud's phenomenon)
  • vasculitis (inflammation of blood vessels)

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How is Sjogren's syndrome diagnosed?

The symptoms of Sjogren's syndrome are similar to other health conditions and you may see different health professionals for each them, for example a dentist for dry mouth, an optician for dry eyes and a gynaecologist for a dry vagina. All of this means Sjogren's syndrome can be difficult to diagnose.

It is important to see your GP if you experience any symptoms of dryness, especially of your eyes and mouth.

Screening questions

The following screening questions have been developed for those who feel they may have Sjogren's syndrome. If you answer yes to most of the questions below, it is probably advised to go and talk to your GP and possibly for further testing.

  • Have you had daily, persistent, troublesome dry eyes for more than three months?
  • Do you keep having a sensation of sand or gravel in your eye?
  • Do you need to use eye drops containing tear substitutes more than three times a day?
  • Have you had a daily feeling of dry mouth for more than three months?
  • Do you keep getting swollen salivary glands (located between your jaw and your ears)?
  • Do you frequently drink liquids to help you swallow food?

Further testing

Sjogren's syndrome can usually be diagnosed by looking at the results of the screening questions and carrying out a number of clinical tests. These may include:

  • the Rose Bengal and Schirmer tests - two tests carried out by an ophthalmologist
  • a lip biopsy - looking for clusters of white blood cells that indicate the presence of Sjogren's
  • blood tests - look for special antibodies known as anti-Ro and anti-La (or SS-A and SS-B), however they are only present in 60% of people with Sjogren's, so it is possible to have a negative blood test and still have Sjogren's
  • salivary flow rate - measures how much saliva your glands produce each minute, low rate can indicate Sjogren's

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How is Sjogren's syndrome treated?

Eye care

Mild to moderate cases of dry eyes can generally be treated with eye drops available from a pharmacy without prescription. A short-term dose of Corticosteroids may be prescribed if the over the counter drops don't work or if the eyes are severely dry and irritated. 

Surgery may be an option if other treatments don't work. This involves sealing the tear ducts to avoid the tears draining away, and protecting the eye instead.

Mouth Care

- good oral hygiene will prevent tooth decay and gum disease

- increasing your fluid intake

- use sugar-free chewing gum to stimulate the production of saliva

- sucking ice cubes to help lubricate your mouth and reduce dryness

- regularly using mouth rinses to soothe the mouth and protect it against infection

- smokers should quit, as the smoke irritates the mouth and increases saliva evaporation 

Saliva substitutes do lubricate the mouth but they don't prevent infection in the same way saliva does. Therefore, if you are using them, you must maintain excellent oral hygiene.

There are some medications that can be taken to treat the symptoms of Sjogren's;

Pilocarpine - stimulates the tear and saliva glands

Hydroxychloroquine - slows the immune system's attack on the tear and saliva glands

General advice for people with Sjogren's syndrome;

- visit the dentists every six months

- practise good dental hygiene

- avoid eating too many sweets

- avoid strong, perfumed soaps

- avoid dry environments (air-conditioned etc) when possible

- stop smoking and do not drink alcohol

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For more detailed information about Sjogren's syndrome, contact the Arthritis Ireland Helpline 1890252846/01-6618188 [email protected] 

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