Introduction

Sjögren's (pronounced Show-grin's) syndrome is an autoimmune disorder. The body’s immune system attacks glands that secrete fluid, such as the tear and saliva glands.

The effects of Sjögren's syndrome can be widespread. Certain glands become inflamed, which reduces the production of tears and saliva, causing the main symptoms of Sjögren's syndrome, which are dry eyes and dry mouth.

In women (who are most commonly affected), the glands that keep the vagina moist can also be affected, leading to vaginal dryness.

Read more about the symptoms of Sjögren’s syndrome

What causes Sjögren's syndrome?

Sjögren's syndrome is an autoimmune condition, which means that instead of protecting the body from infection or illness, the immune system reacts abnormally and starts attacking healthy cells and tissue.

In Sjögren's syndrome, the immune system attacks the tear and saliva glands, and other secretory glands throughout the body.

The reasons for this remains unknown, but research suggests that it's triggered by a combination of genetic, environmental and, possibly, hormonal factors.

Some people are thought to be more vulnerable to the syndrome when they're born and that certain events, such an infection, can trigger the problems with the immune system.

Read more about the causes of Sjögren’s syndrome

Healthcare professionals classify Sjögren's syndrome as being either:

  • primary – when the syndrome develops by itself and not as the result of another condition
  • secondary – when the syndrome develops in combination with another autoimmune disorder, such as lupus or rheumatoid arthritis

Diagnosing Sjögren's syndrome

Sjögren's syndrome can be difficult to diagnose, because it has similar symptoms to other conditions and there is no single test for it.

Your doctor will ask about your symptoms and carry out a test to see how dry your mouth and eyes are.

Read more about diagnosing Sjögren's syndrome

Treating Sjögren's syndrome

There is no cure for Sjögren's syndrome, but treatments can help control symptoms.

Dry eyes and mouth can usually be helped with artificial tears and saliva.

It's important to maintain good eye and mouth hygiene, because your risk of developing an infection is greater. Taking care of your eyes and mouth can help prevent problems such as corneal ulcers and tooth decay.

In severe cases, medication or surgery may be recommended.

Read more about treating Sjögren's syndrome

Complications of Sjögren's syndrome

Sjögren's syndrome can sometimes lead to complications. For example, your eyesight could be permanently damaged if the reduced tear production isn't treated.

Sjögren's syndrome also increases your risk of developing non-Hodgkin lymphoma, which is a cancer of the lymph glands. However, the chances are still low, at around 5%.

Women with Sjögren's syndrome have an increased risk of having children with a temporary "lupus" rash or heart abnormalities. Any pregnancy will be closely monitored for potential problems.

Read more about the complications of Sjögren’s syndrome

Who's affected by Sjögren's syndrome?

Sjögren's syndrome most commonly affects people aged 40-60, with women accounting for about 90% of cases.

It’s difficult to know exactly how many people are affected by the syndrome because many don't see their GP about their symptoms.

However, Arthritis Research UK estimates that there may be up to half a million people in the UK who have Sjögren's syndrome.

Information about you

If you have Sjögren's syndrome, your clinical team will pass information about you on to the National Congenital Anomaly and Rare Diseases Registration Service (NCARDRS).

This helps scientists look for better ways to prevent and treat this condition. You can opt out of the register at any time.

Find out more about the register

Symptoms

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

However, these symptoms can be common in old age, and most people with dry eyes or a dry mouth do not have Sjögren's syndrome.

Many women also experience vaginal dryness, which can make sex painful.

Associated symptoms of dry mouth

dry mouth can lead to:

  • tooth decay and gum disease
  • dry cough
  • difficulty swallowing and chewing
  • hoarse voice
  • difficulty speaking
  • swollen salivary glands (located between your jaw and ears)
  • repeated fungal infections in the mouth (oral thrush) – symptoms of which can include a coated or white tongue

Associated symptoms of dry eyes

Dry eyes can lead to:

  • burning or stinging eyes
  • itchy eyes
  • a feeling that there's grit in your eyes
  • irritated and swollen eyelids
  • sensitivity to light (photophobia)
  • tired eyes
  • mucus discharge from your eyes

These symptoms can get worse when you're:

  • in a windy or smoky environment
  • in an air-conditioned building
  • travelling on aeroplane

Other symptoms of Sjögren's syndrome

In more serious cases of Sjögren's syndrome, the immune system can attack other parts of the body, causing symptoms and conditions such as:

  • dry skin
  • tiredness and fatigue – which are common and can lead to total exhaustion
  • muscle pain
  • joint pain, stiffness and swelling
  • vasculitis (inflammation of blood vessels)
  • difficulty concentrating, remembering and reasoning

Causes

It's not known exactly what causes Sjögren's syndrome, but it's thought to be linked to a problem with the immune system.

The immune system

Your immune system usually helps protect the body from infection and illness by making antibodies. Antibodies attack bacteria and viruses, which help stop you from becoming ill.

An autoimmune condition causes your immune system to react abnormally. Instead of attacking foreign cells, such as bacteria, the antibodies start attacking your body's healthy cells and tissue.

In Sjögren's syndrome, the parts of the body usually affected are the tear, saliva and vaginal glands, which are collectively known as the exocrine glands.

The antibodies damage the exocrine glands so they can no longer function normally. There's some evidence that the immune system also damages the nerves that control these glands, which further reduces their effectiveness.

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

Possible triggers for Sjögren's syndrome

Primary Sjögren’s syndrome

Most researchers believe that primary Sjögren's syndrome is triggered by a combination of genetic and environmental factors.

Certain people are born with specific genes that make them more vulnerable to a faulty immune system. Then, many years later, an environmental factor, possibly a common virus, triggers the immune system to stop working properly.

The female hormone oestrogen also seems to play a role. Sjögren's syndrome mostly occurs in women, and symptoms usually start around the time of the menopause, when oestrogen levels begin to fall. Falling oestrogen levels can contribute to dryness, and this dryness could make the condition more noticeable.

Secondary Sjögren’s syndrome

Sjögren’s syndrome can be associated with other autoimmune conditions, such as rheumatoid arthritis or lupus. This is known as secondary Sjögren’s syndrome.

The exocrine glands

Your saliva and tear glands play a vital role in protecting your mouth and eyes, which is why the symptoms of Sjögren's syndrome can be widespread and troublesome.

The importance of tears

We usually only notice our tears when we cry, but our eyes are always covered by a thin layer of tears, known as a tear film.

Tears are made up of a mix of water, proteins, fats, mucus and infection-fighting cells. Tears serve several important functions. They:

  • lubricate the eye
  • keep the eye clean and free of dust
  • protect the eye against infection
  • help stabilise your vision

The importance of saliva

Saliva also serves several important functions, including:

  • keeping the mouth and throat naturally lubricated
  • aiding digestion by moistening food and containing enzymes that can break down certain starches
  • acting as a natural disinfectant (saliva contains antibodies, enzymes and proteins that protect against some common bacterial and fungal infections)

Diagnosis

Sjögren's syndrome can be difficult to diagnose because the symptoms are similar to those of other health conditions.

You may see different health professionals for your different symptoms, such as a dentist for a dry mouth, an optician for dry eyes and a gynaecologist for a dry vagina, which can sometimes make it difficult to reach a firm diagnosis.

See your GP if you experience any symptoms of dryness, particularly of your eyes and mouth.

Screening questions

Experts in the field have come up with a series of screening questions, which may be useful if you're worried you may have Sjögren's syndrome.

If you answer "yes" to most of the questions below, you may have Sjögren's syndrome and will probably be advised to have further tests.

  • Have you had daily, persistent, troublesome dry eyes for more than three months?
  • Do you keep having a sensation of grit 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

Tests used to diagnose Sjögren's syndrome include:

  • tear break-up time and Schirmer tests
  • a lip biopsy
  • blood tests
  • salivary flow rate

These are explained below.

Tear break-up time and Schirmer tests

Tear break-up time and Schirmer tests are usually carried out by an ophthalmologist (a doctor who specialises in treating eye conditions).

The tear break-up time test measures how effective your tear glands are. A non-toxic dye is dropped onto the surface of your eye and the 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.

This test is also carried out using a slit lamp. A slit lamp is a low-power microscope with a high-intensity light source that can be focused to shine in a narrow beam. The specialist will use the slit lamp to examine your tear glands more closely.

In the Schirmer test, small strips of blotting paper are placed into your lower eyelid. After five minutes, the strips are removed to see how much of the paper is soaked with tears.

Lip biopsy

During a lip biopsy, a small tissue sample is removed from your inner lip and examined under a microscope. A local anaesthetic is injected into the inner surface of your lower lip to numb the area, before a small cut is made to remove a few of your minor salivary glands.

Clusters of lymphocytes (a type of white blood cell) in the tissue can indicate Sjögren's syndrome.

Blood tests

Blood tests are carried out to look for antibodies known as anti-Ro and anti-La (or SS-A and SS-B), which are produced when the immune system has been affected by Sjögren's syndrome.

These antibodies are only present in about 60% of people with Sjögren’s syndrome, so it's possible to have a negative blood test result and still have the condition.

Salivary flow rate

A salivary flow rate test measures how much saliva your glands produce.

You'll usually be asked to spit as much saliva as you can into a cup over a five-minute period. The amount of saliva is then weighed or measured. An unusually low flow rate can indicate Sjögren's syndrome.

Treatment

There's no cure for Sjögren's syndrome, but treatments help relieve symptoms such as eye and mouth dryness.

Sjögren's syndrome affects everyone in different ways, so your treatment plan will be tailored to suit you.

Eye care

Artificial tears

Mild to moderate cases of dry eye can usually be successfully treated with eye drops containing "artificial tears" – a liquid that mimics tears. These eye drops are available from a pharmacist, without a prescription.

There are many different types of eye drops, so you can try different brands to find the one that works best for you. If you're using eye drops regularly (more than three times a day), you should use one that doesn't contain preservatives. This is because there's evidence that over-exposure to preservatives can damage the surface of the eye.

A short-term dose of eye drops containing corticosteroids may be recommended if your eyes become irritated. However, long-term corticosteroid use isn't recommended because they can cause serious side effects.

To minimise the chance of experiencing side effects from corticosteroids, you'll be prescribed the lowest effective dose for the shortest possible time.

Moisture chamber spectacles

Wearing glasses reduces tear evaporation by up to 30%, and this effect can be maximised by wearing specially-made glasses called moisture chamber spectacles. These wrap around your eyes like goggles and help retain moisture and protect the eyes from irritants.

Some people used to be embarrassed to wear them, but modern designs look like sports glasses.

Punctal plugs

Punctual occlusion is a widely-used technique that seals the tear ducts (into which the tears drain) with small plugs. This should help keep the eye better protected by tears.

Temporary plugs made of silicone are usually used first to see if they help. If it does, more permanent plugs can be used.

Mouth care

Looking after your mouth

A number of techniques can be used to keep your mouth lubricated, including:

  • maintaining good oral hygiene to prevent tooth decay and gum disease
  • increasing your fluid intake
  • using sugar-free chewing gum to stimulate saliva production
  • sucking ice cubes to help lubricate your mouth and reduce dryness
  • regularly using mouth rinses to soothe your mouth and protect it against infection

If you smoke, you should try to quit. Smoking irritates the mouth and increases the rate at which saliva evaporates.

Read more about how to stop smoking

Saliva substitutes

There are a number of saliva substitute products that can help lubricate your mouth. However, they don't replicate the role of saliva in preventing infection, so you'll still need to maintain excellent oral hygiene.

Saliva substitutes are available as a spray, lozenge (medicated sweet), gel, or gum. Your GP or pharmacist can tell you which product is most suitable for you.

Medication for Sjögren's syndrome

Pilocarpine

The medicine pilocarpine is often used to treat the symptoms of dry eyes and dry mouth. Pilocarpine stimulates the glands to produce more saliva and tears.

Side effects of pilocarpine include:

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

Don't take pilocarpine if you have asthma or chronic obstructive pulmonary disease (COPD), or if you're pregnant or breastfeeding.

Hydroxychloroquine

Hydroxychloroquine has been shown to slow down the immune system's attack on the tear and saliva glands. It can also help reduce any associated symptoms of muscle pain, joint pain and stiffness.

You'll 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 usually mild. They include:

  • nausea
  • skin rash
  • loss of appetite
  • stomach cramps
  • vomiting

In very rare cases, hydroxychloroquine can damage the retina, affecting vision. You'll probably be asked to attend an eye examination so that your retina can be checked before you start treatment. Regular eye examinations (usually at least once a year) are also recommended after you begin treatment.

Hydroxychloroquine shouldn't be used by breastfeeding women.

Treating other symptoms of Sjögren's syndrome

Dry skin

Several soaps and creams are specifically designed for people with dry skin. Your pharmacist or GP can advise you.

Vaginal dryness

Vaginal dryness can be treated using a lubricant. Some women also use oestrogen creams or hormone replacement therapy (HRT).

Muscle and joint pains

Muscle and joint pains can be treated with an over-the-counter non-steroidal anti-inflammatory drug (NSAID), such as ibuprofen. If this doesn't work, see your GP, as stronger NSAIDs are available on prescription.

NSAIDs can increase your risk of developing stomach ulcers and internal bleeding, particularly if they're taken on a long-term basis.

If you find swallowing NSAIDs difficult because of your dry mouth, you can try an NSAID cream that's rubbed into affected joints.

NSAIDs aren't recommended for pregnant or breastfeeding women, or for people with pre-existing risk factors for cardiovascular or kidney conditions.

General advice

These simple tips can help prevent many of the problems associated with Sjögren's syndrome:

  • have a dental check-up every six months
  • practise good dental hygiene – brushing, flossing and using mouthwash regularly
  • avoid eating too many sweet foods
  • avoid strong and perfumed soaps – use special creams and soaps from your pharmacist
  • avoid dry environments, such as air-conditioned offices, whenever possible
  • avoid drinking too much alcohol

Complications

Sjögren's syndrome isn't usually life-threatening, but it is linked to more seriousproblems.

Non-Hodgkin lymphoma

It's estimated that people with Sjögren's syndrome are 44 times more likely to develop non-Hodgkin lymphoma than people without the condition.

Non-Hodgkin lymphoma is a cancer of the lymphatic system. The lymphatic system is a series of vessels and glands (lymph nodes) that are spread throughout your body, much like your blood vessels.

While this increased risk may sound alarming, the chance of a person with Sjögren’s syndrome developing non-Hodgkin lymphoma is still small, as it only affects around 5% of people with the syndrome.

However, if you have Sjögren’s syndrome, you should be aware of the main early symptom of non-Hodgkin lymphoma, which is a painless swelling in a lymph node (gland) – usually in the neck, armpit or groin. Report any swollen lymph nodes to your GP.

Eye damage

If dry eyes aren't treated, they can become inflamed and you can develop ulcers on the surface of your eyes (corneal ulcers).

If left untreated, corneal ulcers can lead to loss of vision and permanent sight damage.

Pregnancy

If you're planning to become pregnant and have Sjögren's syndrome, ask your GP to test for certain antibodies that may be present in Sjögren's syndrome and are known to cause a temporary lupus rash in newborn babies. In very rare cases, the antibodies can also cause heart defects in babies.

If these antibodies are found, there should be no reason why you can't continue with the pregnancy, but your child may need additional specialist care during pregnancy and after the birth.

Read more about antenatal appointments

Other conditions

A number of other conditions have been linked to Sjögren's syndrome, including: 

Also on NHS inform