Sjogren's syndrome

Sjogren's syndrome is a condition where the body's immune system malfunctions and begins to attack healthy tissue (an autoimmune condition).

In Sjogren's syndrome the immune system usually targets the tear and saliva glands, leading to a reduction in the production of saliva and tears. This can lead to symptoms of dry mouth and dry eyes.

In women, the glands responsible for keeping the vagina moist can also be affected, leading to vaginal dryness.

Other parts of the body can also be affected, causing a range of symptoms from fatigue, muscle and joint pain, and numbness and pain in the arms or legs (neuropathy).

Types of Sjogren's syndrome

Health professionals classify Sjogren's syndrome into one of two types:

  • Primary Sjogren's syndrome: where the condition develops by itself.
  • Secondary Sjogren's syndrome: where the condition develops in combination with another autoimmune condition, such as lupus (a condition that causes fatigue, joint pain and skin rashes) or rheumatoid arthritis.

Secondary Sjogren's syndrome is the most common type and accounts for 60% of all cases. 

How common is Sjogren's syndrome?

 Sjogren's syndrome is a relatively common condition. Three to four per cent of the population of England are thought to have Sjogren's syndrome, making it the second most common autoimmune condition after rheumatoid arthritis.

90% of cases involve women.

The condition can develop at any age, but most cases will begin in people age between 40-60 years.

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.

Prognosis

 There is no cure for Sjogren's syndrome but there are a number of treatments and self-care techniques that can help control symptoms, such as eye drops and medicines to stimulate saliva production.

Unfortunately, having excessively dry eyes and a dry mouth can cause other symptoms, such as eye irritation, dental decay, oral thrush (fungal infection of the mouth) and difficulties swallowing (dysphagia).

The immune system

The immune system is designed to recognise any foreign body that represents a threat to the body, such as a virus or bacterial infection, and then attack it with special cells known as lymphocytes.

In cases of Sjogren's syndrome, the immune system mistakes healthy tissue for a foreign body and attacks it with lymphocytes. The parts of the body usually affected are the tear, saliva and vaginal glands, which are collectively known as the exocrine glands.

The lymphocyte damages the exocrine glands, meaning that they can no longer function normally. There is also some evidence that the immune system damages the nerves that control these glands, further reducing their effectiveness.

The immune system can go on to damage other part of the body such as muscles, joints, blood vessels, nerves and, less commonly, organs.

Possible triggers for Sjogren's syndrome

The exact cause of Sjogren's syndrome is unknown but most researchers believe that it is triggered by a combination of genetic and environmental factors.

The general thinking is that certain people are born with specific genes which makes their immune system more likely to malfunction. Then many years later, environmental factors, most likely a virus such as the Epstein-Barr virus or hepatitis C, triggers that malfunction.

Hormones, specifically oestrogen, seem to play an important role as well. Sjogren's syndrome occurs mostly in women, and usually occurs around the time of the menopause when levels of oestrogen begin to fall. This may suggest that this reduction in oestrogen somehow disrupts the normal functioning of the immune system. Exactly how this may occur is still unclear.

The importance of exocrine glands

The reason that Sjogren's syndrome can cause such a wide range of troublesome symptoms is that your saliva and tear glands play a vital role in protecting your mouth and eyes.

The importance of tears

We tend to only notice our tears when something happens that makes us cry. But the truth is that our eyes are always covered by a thin layer of tears – known as a tear film.

Tears are not simply made up of water. In fact they are a complex mix of water, proteins, fats, mucus and infection-fighting cells. Tears serve several important functions:

  • they lubricate the eye, keeping it clean and free of dust,
  • they protect the eye against infection, and
  • they aid sight by helping to stabilise your vision.

The importance of saliva

Saliva also serves several important functions:

  • it keeps the mouth and throat naturally lubricated,
  • it aids digestion by moistening food and also contains enzymes that can break down certain starches, and
  • it acts as a natural disinfectant, saliva contains antibodies, enzymes and proteins that provide protection against some common bacterial and fungal infections.

 

When to seek medical advice

Sjogren's syndrome can be challenging to diagnose as the symptoms can be wide ranging and are shared by other health conditions.

The task is often made more difficult, as you may visit different health professionals with different symptoms. For example, a dentist with symptoms of a dry mouth, an optician with symptoms of dry eyes, and a gynaecologist with symptoms of a dry vagina.

Screening questions for Sjogren's syndrome

Experts in the field have come up with a series of screening questions, which may be useful if you are concerned that you may have Sjogren's syndrome.

If you answer yes to the majority of the questions you may have Sjogren's syndrome and should ask your GP for further testing (see below for more details). The questions are reproduced below.

  • Have you had daily, persistent, troublesome dry eye for more than three months?
  • Do you have a recurrent sensation of sand or gravel in your eye?
  • Do you have 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?
  • Have you had recurrently or persistently swollen salivary glands?
  • Do you frequently drink liquids as an aid in swallowing food?

Further testing

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

The Rose Bengal and Schirmer tests

These two tests are usually carried out by an ophthalmologist. An ophthalmologist is a doctor who specialises in the treatment of eye conditions.

The Rose Bengal test is used to measure how effective your tear glands are. In this test a non-toxic dye known as Royal Bengal is dropped on the surface of your eye. The distinctive colour of the dye allows the ophthalmologist to see how well your tear film is functioning and how long it takes for your tears to evaporate.

An alternative to the Rose Bengal test is the Schirmer test. In this test, small strips of blotting paper are placed under your eyelid. After five minutes, the strips are removed and then studied to see how much of the paper was soaked.

Lip biopsy

During a lip biopsy, a small sample of tissue is removed from the salivary glands in your lip and then examined under a microscope. Clusters of lymphocytes in the tissue can indicate the presence of Sjogren's syndrome.

Blood tests

Blood tests are carried out to look for special cells known as anti-Ro and anti-La, which are known to be produced when the immune system has been affected by Sjogren's syndrome.

Salivary flow rate

During this test you are usually asked to spit as much saliva as you can into a cup during a five minute period. The amount of salvia is then weighed. The test is a good way of measuring how much saliva your glands are producing per minute. An unusually low flow rate can indicate the presence of Sjogren's syndrome.

 

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

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

Associated symptoms of dry mouth

Associated symptoms of dry mouth includes:

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

Associated symptoms of dry eyes

Associated symptoms of dry eyes include:

  • a burning or stinging sensation in your eyes,
  • your eyes feel itchy,
  • it feels like there is a foreign body, such as a piece of sand or gravel, in your eyes,
  • your eyelids become irritated, and may swell,
  • you become sensitive to light (photophobia),
  • your eyes feel tired, and
  • you may notice a discharge of mucus from your eyes.

Symptoms can become worse when you are in a windy or smoky environment. Many people have also reported that being in buildings that are air-conditioned or travelling on aeroplanes makes their 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 other than the tear, saliva or vaginal glands, causing a wide range of symptoms.

Some of the most commonly reported of these symptoms are:

  • 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).

The two main goals of treatment are to help replicate or compensate for the reduced activities of your tear and saliva glands.

Eye care

Artificial tears

Mild to moderate cases of dry eye can normally be successfully treated with eye drops that contain 'artificial tears' – a liquid designed to mimic the properties of tears. These eye drops are available from a pharmacist without prescription.

There are many different types of eye drops so you can try a different type if your original choice proves ineffective.

A short-term dose of eye drops containing corticosteroids may be recommended if you experience a particularly severe episode of irritation and inflammation of the eyes. But their long-term use is not recommended as they can cause serious side effects.

Moisture chamber spectacles

Some cases of dry eye can be treated through the use of specialised equipment. This includes specially made glasses known as moisture chamber spectacles. These wrap around the eyes much like goggles and help retain moisture and protect the eyes from irritants.

Moisture chamber spectacles used to be unpopular as they had a strange looking design and people were embarrassed to wear them. Now they are becoming a more popular treatment option as the modern designs look like sports sunglasses.

Surgery

If your dry eyes fail to respond to other forms of treatment, surgery may be an option.

One widely used technique is known as punctual occlusion, and involves using small plugs to seal the tear ducts into which the tears drain. This should help keep the eye better protected by tears. Temporary plugs made of silicone are normally used first to see if the surgery has a positive effect. If it does, more permanent plugs can be used to replace the silicone plugs.

Mouth care

Self-care techniques

There are a number of self-care techniques that you can use to keep your mouth lubricated and to deal with any associated symptoms. These are listed below.

  • It is important to maintain a rigorous and meticulous oral hygiene regime to help prevent tooth decay and gum disease. See the 'prevention' section for more information.
  • Increase your fluid intake.
  • Many people find that using sugar-free chewing gum helps stimulate the production of saliva.
  • Sucking ice cubes can help lubricate the mouth and reduce unpleasant feelings of dryness.
  • If you are a smoker you should give up. Smoke both irritates the mouth and increases the rate at which saliva evaporates.
  • Regular use of mouth rinses can help soothe the mouth and protect against infection.

Saliva substitutes

There are a number of products that can help replicate the lubricating function of saliva. However, they do not replicate the role saliva plays in preventing infection so you will still need to maintain excellent oral hygiene.

These products are usually available as a spray, in lozenge form or in gel or gum form. Your GP or pharmacist will be able to advise which product is most suitable for you.

Medicines for Sjogren's syndrome

Pilocarpine

The medicine pilocarpine is often used to help treat the symptoms of dry eyes and mouth. Pilocarpine works by stimulating the tear and saliva glands to produce more saliva and tears.

Side effects of pilocarpine include:

  • excessive
  • sweating,
  • nausea,
  • diarrhoea,
  • heartburn,
  • abdominal pain,
  • headaches,
  • dizziness, and
  • an increased need to go to the toilet.Less common side effects include:
  • vomiting,
  • palpitations,
  • high blood pressure, and
  • blurred vision.

For some people, the side effects are mild. Others find that the disadvantages of the side effects outweigh the benefits of pilocarpine.

Hydroxychloroquine

You may also be recommended to take a medicine called hydroxychloroquine. Hydroxychloroquine has been shown to slow the immune system's attack on the exocrine glands. It can also help reduce any associated symptoms of muscle and joint pain and stiffness.

You will need to take hydroxychloroquine for several weeks before you notice any improvements and it could be six months before you experience the full benefit of the treatment.

Side effects are uncommon and, if experienced, usually mild. They include:

  • headache,
  • nausea,
  • skin rash,
  • itching,
  • loss of appetite,
  • stomach cramps, and
  • vomiting.

Very rarely, hydroxychloroquine can damage the retina, leading to impairment in vision. You will probably be asked to attend an eye examination so the state of your retina can be checked before you commence treatment. Regular eye examinations are also recommended after you begin treatment; usually once every year.

Hydroxychloroquine should not be used by pregnant and breastfeeding women.

Treating other symptoms of Sjogren's syndrome

Dry skin

There are a number of specially designed soaps and creams designed for people with dry skin. Your pharmacist or GP will be able to advise you.

Vaginal dryness

The symptoms of vaginal dryness can be treated using a lubricant, such as KY Jelly.

Muscle and joint pains

Muscle and joint pains can be treated by taking the over-the-counter painkillers known as nonsteroidal anti-inflammatory drugs (NSAIDs) such as ibuprofen. If these prove ineffective, you should see your GP as stronger NSAIDs are available on prescription.

Ideally, you should take NSAIDs, either with food or shortly after eating. This will help minimise the risk of the medicine upsetting your stomach

Some people find swallowing NSAIDs difficult due to their symptoms of dry mouth. In these circumstances, NSAIDs are available in cream form which you can rub into any affected joints. Alternatively, NSAIDs are available as suppositories.