Sjögren's Syndrome
An Information Booklet
What is Sjögren's syndrome?
Sjögren's syndrome is named after Henrik Sjögren (pronounced 'Shurgren'), the Swedish ophthalmologist who first described it in 1933. It is an autoimmune disorder, meaning that the body's immune system, which usually fights infections, attacks the body's own tissues. The most common symptoms of Sjögren's syndrome are dry eyes or a dry mouth (sometimes both together), and feeling very tired and aching.
Although there is no cure for this syndrome, most people can be treated quite easily. A few do have other, more serious symptoms. These can also be treated and are described later in this booklet.
There are two types of Sjögren's syndrome. Secondary Sjögren's syndrome is associated with (occurs with) another rheumatic disease such as rheumatoid arthritis (see arc booklet 'Rheumatoid Arthritis'). Primary Sjögren's syndrome is not associated with any underlying rheumatic disease.
This booklet mainly deals with primary Sjögren's syndrome. However, the sections on diagnosis and treatment will be useful to people with dry eyes or a dry mouth that have been caused by other illnesses. Terms that appear in italics when they are first used are explained in the glossary at the end of the booklet.
Is Sjögren's syndrome the same as sicca syndrome?
No. 'Sicca' simply means dryness. Sicca syndrome describes problems of dry eyes and a dry mouth which are not caused by an autoimmune disorder. Sicca syndrome occurs when the tear glands in the eyes are damaged, or when the glands in the mouth which produce saliva are damaged. This damage can be caused by several illnesses, including sarcoidosis, excessive x-ray irradiation and iron overload disease (haemochromatosis).
How common is primary Sjögren's syndrome?
Primary Sjögren's syndrome is probably the second most common autoimmune rheumatic disorder after rheumatoid arthritis. It is estimated that half a million people suffer from this syndrome in the UK. It is difficult to work out exact numbers as many people do not seek medical help and simply put up with their symptoms as a bit of a nuisance.
Who gets Sjögren's syndrome?
It occurs mostly in women between the ages of 40 and 60. It is much less common in men (1 in 10 of those with Sjögren's syndrome are men), and occurs only rarely in childhood. It affects all races.
What are the main symptoms?
The two most common complaints are:
- dry eyes or a dry mouth (sometimes dry eyes and a dry mouth)
- feeling very tired and aching.
Many people do not have any other symptoms. For more information on symptoms and treatments, see 'How are the main symptoms of Sjögren's syndrome treated?' and 'How are the less common symptoms treated?'.
How does Sjögren's syndrome develop?
As mentioned, Sjögren's syndrome is an autoimmune disorder, so the body's immune system attacks the body's own tissues. In Sjögren's syndrome, the immune system mainly attacks the tear glands in the eye and the salivary glands in the mouth. The immune system sends cells into these glands which cause the glands to swell (to become inflamed) – see Figure 1. The body also produces antibodies which react with and damage the tissues in the glands. The nerve signals to the glands are also affected and this reduces the amount of saliva produced.
Usually, once the tear and salivary glands have been damaged the antibodies do not cause any further harm. However, in a few people other parts of the body can be affected, such as the liver and kidneys. Some joints can become inflamed, but they are not damaged as they are in rheumatoid arthritis.
What causes Sjögren's syndrome?
We are not yet sure how Sjögren's syndrome is caused but it has been linked to several viruses (e.g. the Epstein–Barr virus, the retroviruses and the HTLV group of viruses). It is thought that a virus infects the body and then when the virus settles within the body tissues, the immune system reacts and this triggers the start of the inflammation.
Are there any hereditary influences?
There may be some hereditary (genetic) factors which mean that some people find it more difficult to eliminate certain infections from their bodies than others and therefore are more prone to developing Sjögren's syndrome. However, it is uncommon for children to inherit the condition from their mother or father.
Does pregnancy affect the syndrome?
There are generally no increased problems during or following pregnancy. However, when mothers have primary Sjögren's syndrome a few new-born babies may have disturbed heart rhythms. This is because certain antibodies known as anti-Ro and anti-La can be passed from mother to baby, although this is very rare (see 'Blood tests').
Are there any allergic reactions?
People with Sjögren's syndrome are slightly more likely to develop side-effects to common drugs, e.g. antibiotics. We recommend that people who have the syndrome do not take any medications unless they are really necessary.
How is Sjögren's syndrome diagnosed?
Sjögren's syndrome can produce a variety of complaints. As a result, people with the syndrome may need to see various medical professionals, such as their family doctor (GP), rheumatologist, eye specialist, dentist or oral surgeon. After the doctor has asked about all the symptoms and other relevant facts, and examined the person, the main tests for Sjögren's syndrome are:
Schirmer's test
A small piece of blotting paper (sterilised and pre-packaged) is used to measure tear production.
Slit lamp examination
An eye specialist (ophthalmologist) will place eye-drop dye (Rose Bengal dye) in the eye before examining the eye with an instrument called a slit lamp. This is a lamp which shines light through a very narrow slot. The beam produced allows the eye to be examined more effectively.
Measurement of saliva production
This can be measured by arranging for the person with Sjögren's syndrome to spit into a paper cup over a specific period of time. More sophisticated investigations include isotope scanning of the salivary glands or injecting contrast dyes into the salivary glands.
Blood tests
These are done because two antibodies are found more frequently in primary Sjögren's syndrome than in other disorders. These are the antibodies known as anti-Ro and anti-La, found in about 75% and 60% of people with primary Sjögren's syndrome respectively. Because they occur only rarely in other conditions, these antibodies are useful in diagnosing primary Sjögren's syndrome.
Lip biopsy
One of the tiny salivary glands can be removed very simply from the lower lip without the need for a general anaesthetic. The gland is examined under a microscope to check the diagnosis. This is usually done only if the diagnosis is uncertain based on all the other tests.
Ultrasound scans and magnetic resonance imaging
Ultrasound scans and magnetic resonance imaging (MRI) are sometimes used to help diagnose Sjögren's syndrome.
What is the long-term outlook if you have Sjögren's syndrome?
People with Sjögren's syndrome are not likely to be disabled by the condition (unlike the situation with some other rheumatic disorders). The symptoms are uncomfortable and long-lasting but do not usually cause serious problems (see 'How are the main symptoms of Sjögren's syndrome treated?' and 'How are the less common symptoms treated?'). Research has shown that people with Sjogren's syndrome have a somewhat increased risk of developing certain types of cancer (cancers of the lymphatic tissues – lymphoma). However, your doctor will be aware of this and will look out for any early signs. Lymphoma associated with Sjögren's syndrome can be treated. One of the first signs can be enlarged lymph glands. If you have an enlarged lymph gland in the neck or elsewhere (the lymph glands are also found in the armpits and the groin) you should report this to your doctor.
How are the main symptoms of Sjögren's syndrome treated?
Table 1 summarises the main symptoms and explains how they can be treated. For all these symptoms, people with Sjögren's syndrome should also make sure that they follow the usual dental advice on oral hygiene and reducing dental decay.
| Table 1. The main symptoms of Sjögren's symdrome and how they can be treated | |
Symptom(s) |
How they can be treated |
Eyes are dry, irritable, and may be sore. Strong lights are uncomfortable. |
Use lubricant eye drops, e.g. hypromellose, Sno Tears, Viscotears (either a few times a day or up to hourly if necessary). Wear dark or tinted spectacles if they help. Do not use contact lenses. |
Mucous and debris in the eyes cause stickiness. |
Use mucolytic agents such as acetylcysteine eye drops. |
Eyes are very dry. Note: if there are corneal ulcers or infections these should be referred to an eye specialist (the treatments opposite do not apply). |
Use preservative-free eye drops, ointments (including ciclosporin), pseudoplastic drops (Celluvisc, Viscotears). Have minor surgery by an eye specialist (Lacriserts punctal occlusion). Wear special spectacles which keep in moisture and so reduce dryness of the eyes. |
Mouth is very Tastes are altered. Voice is hoarse or weak. Swallowing is difficult (some-times because the mouth and throat are so dry). |
Use artificial saliva. Luborant mouth spray is recommended for people with their own teeth because it contains fluoride. AS Saliva Orthana lozenges and Glandosane mouth spray can be used by people with their own teeth but, because they contain no fluoride, care should be taken with dental hygiene and a separate fluoride mouthwash should be used. Chlorhexidine or linseed extract mouth rinses may help. Chew non-sugar chewing gum, other dental care products, or lozenges (e.g. anhydrous crystalline maltose). Gels containing xylitol may be useful, e.g. Biotene Oralbalance and BioXtra (Molar). Take proper care of dentures. |
Mouth ulcers. |
Orabase ointment can be applied to the mouth ulcers. If this is not effective your doctor may prescribe corticosteroid pellets for keeping in the mouth for a few times per day. |
Mouth ulcers cause 'sticky' mouth or throat. |
Use diluted chlorhexidine mouthwash (e.g. Corsodyl). Rinse and gargle with mouthwash (mix 1 teaspoon salt and 1 teaspoon baking powder with 1 litre of water). |
Mouth is very severely dry causing other problems such as fungal infection, and dry cough (see below). |
Use antifungal treatment such as nystatin. Your doctor may occasionally try pilocarpine (Salagen). |
Dry cough. |
Humidifiers and plants with large leaves in rooms may help. Avoid drugs which may aggravate symptoms, e.g. antihistamines, travel-sickness pills and antidepressants. |
How are the less common symptoms treated?
| Table 2. The less common symptoms of Sjögren's syndrome and how they can be treated | |
Symptom(s) |
How they can be treated |
Pain in the joints (arthralgia). Sometimes there is general aching over the body but also with tender spots (fibromyalgia).
|
Take painkillers such as paracetamol and/or non-steroidal anti-inflammatory drugs (NSAIDs) such as ibuprofen. Have an injection of steroid and anaesthetic at isolated tender spots or joints. |
Salivary glands are painful.
|
Ask your doctor for further assessment and advice. S/he will check that you do not have an infection. If the salivary glands are not infected a short course of prednisolone is sometimes prescribed. |
Skin is dry and itchy. Skin is very sensitive to strong sunlight. |
Avoid strong soaps. Use aqueous creams and emollients, e.g. E45, and barrier creams in summer. |
Vagina is dry. Intercourse is painful (dyspareunia). |
Use lubricants, e.g. KY Jelly. Use oestrogen creams. Treat any infection, e.g. thrush. |
Feeling extremely tired all the time (lethargy), often being in low spirits. Joints are inflamed. Fever. These problems are only found in more severe cases. |
Your doctor may prescribe chloroquine or hydroxychloroquine (Plaquenil). Corticosteroids such as prednisolone are often prescribed. |
Fingers feel cold |
Avoid situations which bring on these symptoms – for example, avoid exposure to the cold, do not smoke. Use gloves. Take vasodilators, e.g. nifedipine. |
Migraine-like headaches. |
Take painkillers or specific anti-migraine medications. |
Lower abdominal pain and disordered bowel habits (irritable bowel syndrome). |
Increase the amount of fibre in the diet. Take antispasmodic drugs, e.g. mebeverine (Colofac). |
Liver problems. |
These are not very common – mild liver test abnormalities do not require treatment but must be followed up with regular checks by your doctor. Occasionally a specific liver disorder called primary biliary cirrhosis develops. This should be investigated by a specialist. |
Lungs can be affected by fibrosis. This causes breathlessness on exertion, dry cough (see the section in Table 1) and occasionally pleurisy causing chest pain. |
The lung function must be tested. Fibrosis needs specialist advice. Corticosteroids can be prescribed. |
Lymph glands in the neck, armpits or groin are swollen. |
Report this to your doctor. |
Menopausal symptoms may |
Hormone replacement therapy (HRT) can be used. There are no added risks because of Sjögren's syndrome but you should talk to your doctor about the general risks of HRT, which include increased risk of breast cancer and heart disease. |
Neuropathy (problems with the peripheral nervous system such as weakness or numbness), vasculitis, or a severe condition overall which is not helped by other treatments. |
Newer (still experimental) drugs include oral interferon alfa, anti-TNF drugs (etanercept, infliximab and adalimumab) and zidovudine. |
Glossary
Antibodies – blood proteins which are formed in response to germs, viruses or any other substances which the body sees as foreign or dangerous. The role of antibodies is to attack these foreign substances and make them harmless.
Magnetic resonance imaging (MRI) – a type of scan which uses a strong magnetic field to build up pictures of the inside of the body. It works by detecting water molecules in the body's tissue which give out a particular signal in the magnetic field.
Peripheral nervous system – all parts of the nervous system which are not part of the brain or spinal cord (which form the central nervous system). It includes nerves going to and from the arms, legs, hands, eyes, mouth and other parts of the body.
Ultrasound scan – a type of scan which uses high-frequency sound waves to build up images of the inside of the body.
Sarcoidosis – a disorder which causes small fleshy nodules (granulomata) to form in tissues in the body. This can be in the lungs, liver, spleen, skin, salivary glands and other tissues, and the lymph glands are often swollen. The cause is not known.
Vasculitis – inflammation of the walls of blood vessels. This can cause the blood flow to be reduced. Vasculitis can occur on its own (this is called primary vasculitis) or in people who already have an established disease (this is called secondary vasculitis). Secondary vasculitis can happen with a number of different rheumatic diseases, including Sjögren's syndrome, rheumatoid arthritis and lupus. There are many different types of vasculitis and there is a separate arc booklet on this subject, 'Vasculitis'.
Useful addresses
The Arthritis Research Campaign (arc)
PO Box 177
Chesterfield
Derbyshire S41 7TQ
Phone: 0870 850 5000
www.arc.org.uk
As well as funding research, we produce a range of free information booklets and leaflets. Please contact the address above for a list of titles or, on this web site, see Publications for People with Arthritis.
Arthritis Care
18 Stephenson Way
London NW1 2HD
Phone: 020 7380 6500
Helplines: 020 7380 6555 (10am–4pm Mon–Fri)
or freephone: 0808 800 4050 (12pm–4pm Mon–Fri)
www.arthritiscare.org.uk
Offers self-help support, a helpline service (on both numbers above), and a range of leaflets on arthritis.
BSSA (British Sjögren's Syndrome Association)
PO Box 10867
Birmingham
B16 0ZW
Phone: 0121 455 6532
Helpline: 0121 455 6549 (9.30am–5pm Mon–Fri)
nras (National Rheumatoid Arthritis Society)
Unit B4 Westacott Business Centre
Westacott Way, Littlewick Green
Maidenhead SL6 3RT
Phone: 01628 823524
Helpline: 0845 458 3969
www.rheumatoid.org
A national charity which focuses specifically on rheumatoid arthritis.
