Sjogren's syndrome

Overview

Sjögren's (pronounced Show-grin's) syndrome is a condition that affects parts of the body that produce fluids like tears and spit (saliva).

It usually starts in people aged 40-60 and is much more common in women than men.

It's a long-term condition that can affect your daily life, but there are treatments to help relieve the symptoms.

Symptoms of Sjögren's syndrome

Symptoms of Sjögren's syndrome include:

dry eyes
a dry mouth
dry skin
tiredness
vaginal dryness
muscle or joint pain
swelling between the jaw and ears (swollen salivary glands)
rashes (especially after being out in the sun)

When to see your GP

See your GP if you have symptoms of Sjögren's syndrome that don't go away or are bothering you.

There are many things that can cause similar symptoms. Your GP can check for some of the more common causes, such as swelling of the eyelids (blepharitis), diabetes or medication.

If needed, they can refer you to a specialist for further tests, such as:

blood tests
an eye examination
a lip biopsy – where a tiny piece of tissue from inside your lip is removed and examined under a microscope

Treatments for Sjögren's syndrome

There's currently no cure for Sjögren's syndrome, but there are several treatments that can help, such as:

eye drops that keep your eyes wet (artificial tears)
sprays, lozenges (medicated sweets) and gels that keep your mouth wet (saliva substitutes)
medicine that helps your body produce more tears and saliva

Things you can do if you have Sjögren's syndrome

If you have Sjögren's syndrome, there are some things you can do to help relieve your symptoms.

These include:

avoiding dry, smoky or windy places
avoiding reading, watching TV or looking at screens for a long time
practising good oral hygiene
avoiding alcohol and not smoking

Causes of Sjögren's syndrome

Sjögren's syndrome is caused by the immune system – the body's defence against illness – damaging healthy parts of the body. This is what's known as an autoimmune condition.

The bits of the body usually affected are those that produce fluids like tears and saliva. But other parts of the body, such as nerves and joints, can also be affected.

It's not clear why the immune system stops working properly. It may be linked to:

genetics – some people may be born with genes that make them more likely to get an autoimmune condition
hormones – the female hormone oestrogen may play a part, as the condition is much more common in women than men

Sjögren's syndrome can occur with other autoimmune conditions like rheumatoid arthritis or lupus. This is known as secondary Sjögren's syndrome.

Primary Sjögren's syndrome is where you don't have any other related conditions.

Living with Sjögren's syndrome

Sjögren's syndrome is a long-term condition that doesn't tend to get better on its own, although the symptoms can often be treated.

For some people the condition may just be a bit of a nuisance, while for others it can have a big impact on their everyday life.

Some people may develop complications of Sjögren's syndrome, such as problems with their vision or lungs.

There's also a slightly increased risk of a type of cancer called non-Hodgkin lymphoma.

If you're diagnosed with Sjögren's syndrome, ask your doctor about what you can expect.

You may also find it useful to contact organisations such as the British Sjögren's Syndrome Association (BSSA)for advice and support.

Symptoms

The main symptoms of Sjögren's syndrome are dry eyes and a dry mouth, but it can also cause several other problems.

Each person is affected differently. For some people the condition may just be a bit of a nuisance, while for others it can have a big impact on their everyday life.

There are many conditions that can cause similar symptoms. See your GP if you have any symptoms you're worried about.

Dry eyes

Signs that you may have dry eyes include:

burning, stinging or itchy eyes
a feeling of grit or sand in your eyes
sore, red and swollen eyelids
discomfort when looking at lights
sticky eyelids when you wake up
blurred vision

These symptoms may be worse when the air is dry – for example, when you're somewhere that's windy, smoky or air conditioned.

Dry eyes can be caused by many conditions besides Sjögren's syndrome.

Dry mouth

Signs that you may have a dry mouth include:

feeling like food gets stuck in your mouth or throat – especially dry food like crackers
needing to drink water while eating to help you swallow food
your tongue sticking to the roof of your mouth
a hoarse voice
a smooth, red tongue
a change in how food tastes
dry, sore and cracked skin at the corners of your lips
problems such as tooth decay, gum disease, mouth ulcers, and oral thrush – a fungal infection that can cause a raw, red or white tongue

Other reasons for a dry mouth include things like diabetes or medicines.

Other symptoms

Sjögren's syndrome can also cause a range of other problems.

These can include:

dry, itchy skin
severe tiredness and exhaustion
vaginal dryness in women, which can make sex painful
rashes (especially after being out in the sun)
a dry cough that doesn't go away
swelling between the jaw and ears (swollen salivary glands)
muscle pain
joint pain, stiffness and swelling
difficulty concentrating, remembering and reasoning

Some people have other conditions closely linked to Sjögren's syndrome too, such as Raynaud's phenomenon, a condition that affects the blood supply to the fingers and toes.

Diagnosis

See your GP if you have symptoms of Sjogren's syndrome.

They'll ask you about your symptoms and look at your eyes and mouth to check for any obvious problems.

But as there are many conditions with similar symptoms to Sjogren's syndrome, it can be very difficult for your GP to diagnose.

They can refer you to a specialist for further checks if needed.

Blood tests

Blood tests can be done to look for antibodies in your blood. Antibodies are substances produced by your immune system (the body's defence against illness and infection) to attack germs.

In Sjogren's syndrome, the immune system produces antibodies that attack healthy areas of the body. These can be found during a blood test.

But not everyone with Sjogren's syndrome has these antibodies, so you may still have the condition even if a blood test doesn't find them.

Checking the layer of tears on your eyes

An eye doctor (ophthalmologist) may carry out a test to look at the layer of tears that forms across the front of your eyes.

This involves harmless coloured drops being placed in your eyes to make the layer of tears easier to see for a short time. Your doctor then looks at your eyes using a special microscope with a light.

If the layer of tears is very patchy, it could be a sign of Sjogren's syndrome.

Removing a piece of lip tissue

In people with Sjogren's syndrome, clumps of white blood cells, which are produced by the immune system, can form inside the cells where spit (saliva) is produced.

To check for this, a very small piece of tissue from the inside of your lip may be removed and looked at under a microscope. This is known as a lip biopsy.

Local anaesthetic is injected into your lip to numb it for the procedure.

Other tests

Occasionally, some other tests may be carried out. These may include:

a spit test - you spit as much saliva as you can into a cup over a five-minute period and the amount is then measured or weighed
measuring how many tears you produce - small strips of paper are placed in your lower eyelid for five minutes to see how much of the paper is soaked with tears

Producing less saliva or fewer tears than normal can be a sign of Sjogren's syndrome.

Treatment

There's currently no cure for Sjogren's syndrome, but there are treatments that can help relieve the symptoms.

Treatments for dry eyes

Things you can do

If you have dry eyes, it can help to:

avoid dry, smoky or windy places - it may help to use a humidifier at home or work to keep the air most
avoid reading, watching TV or looking at screens for a long time - this can dry your eyes out
wear wraparound sunglasses or glasses with sides that help stop wind drying out your eyes
clean your eyelids regularly
have regular check-ups with an optician
avoid medicines that can cause dry eyes - check the leaflet that comes with a medicine to see if dry eyes is listed as a side effect

Eye drops and ointments

You can also try eye drops and ointments that keep your eyes wet, sometimes known as artificial tears.

There are several different types of drops that can be bought from pharmacies without a prescription. You may need to try a few types to find out that works for you.

If you use eye drops more than three times a day, avoid drops that contain preservatives as these can damage your eyes if used frequently.

If the surface of your eye is irritated (inflamed), your doctor may prescribe drops containing steroids to use for a short period.

Sometimes other anti-inflammatory drops, such as ciclosporin drops, may be prescribed by an eye specialist.

Medicines and procedures

If self-help measures and eye drops aren't helping, your doctor may recommend:

a medicine called pilocarpine - tablets that help the body produce more tears and saliva (read about medicines for a dry mouth for more information)
a procedure to block the tear ducts with tiny man-made plugs to stop tears draining away - this can help keep your eyes covered with a layer of tears, so they don't feel as dry

Treatments for a dry mouth

Things you can do

If you have a dry mouth, it can help to:

practise good oral hygiene - including brushing your teeth with fluoride toothpaste twice a day
avoid sugary food and drinks, and avoid snacking between meals
use antibacterial mouthwash
drink plenty of water
regularly chew sugar-free chewing gum or suck on ice cubes
use lip balm if your lips are dry and cracked
avoid alcohol
stop smoking if you smoke
have a dental check-up at least every six months
avoid medicines that can cause a dry mouth - check the leaflet that comes with a medicine to see if dry mouth is listed as a side effect

Saliva substitutes

There are also products you can buy from pharmacies that help keep the mouth moist - known as saliva substitutes.

There are several different types available, including sprays, lozenges (medicated sweets) and gels. You may need to try a few types to find one that works for you.

But these products don't help prevent mouth infections in the same way that saliva does, so it's important to practise good oral hygiene.

Medicines

The medicine pilocarpine can be used to treat a dry mouth and eyes. It comes as tablets that help the body produce more saliva and tears.

But it isn't suitable for everyone and can cause some side effects, such as sweating, headaches, and needing to pee more often than normal.

If your doctor recommends pilocarpine, talk to them about the benefits and risks of taking it.

Treatments for other common symptoms

Dry skin

If you have dry skin, it may help to use a moisturising cream (emollient) every day.

It's also a good idea to avoid strong, perfumed soaps. Use emollient soap substitutes instead.

Vaginal dryness

Treatments for vaginal dryness include:

lubricants - liquids or gels that you apply to your vagina just before having sex for immediate relief from dryness
moisturisers - creams that you apply inside your vagina to keep it moist for a few days
hormone treatments - such as oestrogen medication you place in your vagina, or hormone replacement therapy (HRT)

Muscle and joint pain or stiffness

If you have pain or stiffness in your joints or muscles, it can help to:

exercise regularly - a mixture of aerobic exercises (such as cycling) and strength and flexibility exercises may be helpful; a physiotherapist can recommend a suitable exercise plan
take anti-inflammatory painkillers, such as ibuprofen - your GP may prescribe stronger painkillers if these don't work
lose weight if you're overweight

A medication called hydroxychloroquine is sometimes recommended by Sjogren's syndrome specialists as a treatment for joint pain or stiffness if other methods haven't helped.

But it isn't suitable for everyone and can take several months to work. It can also cause side effects such as tummy pain and feeling sick.

Complications

Sjogren's syndrome can sometimes lead to further problems or occur alongside other conditions.

Eye problems

If you have very dry eyes and they're not treated, there's a risk the front layer of your eyes could become damaged over time. This could lead to permanent vision problems.

There are several treatments for dry eyes that can help reduce this risk. You should also have regular check-ups with an optician so any problems are picked up early on.

Contact your GP as soon as possible if you have problems with your vision.

Lung problems

Sometimes Sjogren's syndrome can affect the lungs and cause problems such as:

lung infections
widening of the airways in the lungs (bronchiectasis)
scarring of the lungs

If you smoke, stopping may help reduce the risk of these conditions.

See your GP if you develop a cough, wheezing or shortness of breath that doesn't go away.

Pregnancy complications

Most women with Sjogren's syndrome can get pregnant and have healthy babies.

But if you're planning a pregnancy, it's a good idea to get advice from your GP or specialist because there's a small risk of complications in some women.

These include:

a rash in the baby that lasts a few weeks
serious heart problems in the baby

These problems can occur if you have certain antibodies (produced by the immune system) sometimes found in people with Sjogren's syndrome. A blood test can be done to look for these.

If these antibodies are found, you can still get pregnant, but you may need additional specialist care during pregnancy and after the birth.

Cancer

People with Sjogren's syndrome have an increased risk of developing a type of cancer called non-Hodgkin lymphoma.

This affects the lymphatic system, a network of vessels and glands found throughout the body.

Research suggests people with Sjogren's syndrome are about five times more likely to get non-Hodgkin lymphoma than those who don't have the condition, but the chances of getting it are still small.

See your GP if you develop symptoms of non-Hodgkin lymphoma, such as:

painless swollen glands, usually in the neck, armpit or groin
night sweats
unintended weight loss

Non-Hodgkin lymphoma can often be cured if it's caught early on.