What is Sjögren's syndrome?

Sjögren's syndrome was first described in 1933 by a Swedish ophthalmologist (eye specialist) called Henrik Sjögren.

It is a type of autoimmune disease. Normally, our body makes antibodies to fight infections - for example, when we catch a cold or have a sore throat. These antibodies help to kill the cells of the bacteria, viruses or 'germs' causing the infection. In autoimmune diseases the body makes similar antibodies (autoantibodies) that attack its normal cells. The cause of this is uncertain.

In Sjögren's syndrome, these autoantibodies attack the cells of certain glands. The effect is that these glands cannot release their normal secretions. This means that the symptoms of Sjögren's syndrome are mainly due to dryness and lack of gland secretions.

Sjögren's syndrome commonly affects the eyes, mouth, salivary glands, lungs, kidneys, skin and nervous system but all organs of the body can be affected.

Primary Sjögren's syndrome is when Sjögren's syndrome occurs by itself.

Secondary Sjögren's syndrome is when Sjögren's syndrome occurs in association with another autoimmune disease such as rheumatoid arthritis or systemic lupus erythematosus.

Who gets Sjögren's syndrome?

Sjögren's syndrome affects about 4 in 1,000 people in the UK. It is nine times more common in women than men. People usually first start noticing symptoms when they are in their 30s or 40s.

What are the symptoms of Sjögren's syndrome?

The two symptoms that everyone with Sjögren's syndrome will notice are:

• Xerostomia (dry mouth).
• Xerophthalmia (dry eyes, which may feel gritty and uncomfortable).

The dryness of the eyes, mouth and other body parts is known as sicca syndrome. (As well as Sjögren's syndrome, sicca syndrome can also be caused by radiotherapy treatment and other diseases such as sarcoidosis and haemochromatosis.)

Dry mouth can lead to:

• Swallowing problems and dysphagia (the feeling of something getting stuck in the throat on swallowing).
• Thrush (a yeast infection) in the mouth.
• Loss of taste.
• Tooth decay and periodontal (gum) disease. Saliva contains anti-infective agents so when saliva production is reduced, infection in the mouth is more likely.

Other body parts may also be affected by dryness:

• Vaginal dryness can cause dyspareunia (discomfort when having sex).
• Dryness of the trachea and bronchus (the airways) can lead to a dry cough and chest infections.
• Dry skin can occur.

Other symptoms you may notice include:

• Tiredness.
• Muscle aches and aching joints (Sjögren's syndrome often affects people who have other autoimmune diseases that affect the joints, such as rheumatoid arthritis and systemic lupus erythematosus).
• Swelling of the parotid glands (the salivary gland located in both cheek areas, just in front of the ears).
• Swelling of other salivary glands located under the jaw or in the neck area.

Are there any complications of Sjögren's syndrome?

Rarely, you can develop complications such as:

• Infection of the salivary glands.
• Corneal ulcers: dry eyes can lead to infection and the development of ulcers on the surface of the eyes. If not treated, this can lead to loss of vision.
• Pancreatitis: this is inflammation of the pancreas gland noticed by severe pain in the upper part of the stomach (abdomen).
• Peripheral neuropathy: this causes loss of sensation in fingers, hands, arms, toes, feet, legs.
• Cranial neuropathy: this causes loss of sensation in parts of the face.
• Kidney problems: rarely, Sjögren's syndrome can progress to affect the kidneys. It can cause kidney inflammation, disruption in body fluid balance, kidney stones and, if untreated, kidney failure.
• Pseudolymphoma: 1 in 10 people with Sjögren's syndrome can develop a condition called pseudolymphoma. This causes enlargement of the spleen (an organ in the left upper abdomen that helps the body fight infection and get rid of old red blood cells). It also causes enlargement of the lymph glands throughout the body.
• Non-Hodgkin's lymphoma: in 1 in 10 people who develop pseudolymphoma, the pseudolymphoma can progress to a lymphoma, usually non-Hodgkin's lymphoma. This is a type of cancer of the lymph glands. It tends to progress very slowly and in some cases may even regress (go away) without any treatment. The first symptom noticed with non-Hodgkin's lymphoma is usually swelling of the lymph glands, particularly in the neck. If you are diagnosed with Sjögren's syndrome, you should watch for the development of any lumps or swellings in your neck, groin or under your arms and report anything abnormal to your doctor.
• Parotid gland tumours: these may be more common in Sjögren's syndrome. If you notice a hard/firm swelling in either of your parotid glands (located in the cheek area as described above), you should seek an urgent appointment with your doctor.

Other problems associated with Sjögren's syndrome

• Recurrent miscarriage: Sjögren's syndrome can rarely be the cause of recurrent miscarriage. Recurrent miscarriage is when a woman has three or more miscarriages in a row. This is because of a link between Sjögren's syndrome and a condition called antiphospholipid syndrome.
• Drug reactions: people with Sjögren's syndrome may be more prone to developing side-effects when they take certain drugs - for example, antibiotics.
• Raynaud's phenomenon: the extremities of the body, usually the fingers and toes, change colour and may become painful usually due to exposure to the cold.

How is Sjögren's syndrome diagnosed?

There are a number of investigations that your doctor may do to help diagnose Sjögren's syndrome.

• The Schirmer Tear Test: this measures the amount of tears that you form. Special filter paper is placed under the lower lid of your eye and left for five minutes. In Sjögren's syndrome, tear production is reduced.
• Slit lamp examination: a special dye is used to stain the eye temporarily. The doctor will look into your eyes, using a special lamp, to see if there is reduced tear production or eye damage.
• Salivary gland biopsy: one of the tiny salivary glands may be removed from your lower lip for examination. This is a simple procedure and can be done using an injection of local anaesthetic. Special dyes and staining are then used in the laboratory to look for signs of Sjögren's syndrome in this gland.
• Saliva collection: you may be asked to collect the saliva that you make over 10 minutes. A reduced volume suggests Sjögren's syndrome.
• Blood tests: your doctor may also want you to have some blood tests that may suggest inflammation or signs of an autoimmune disease.

Some other tests may be arranged if the diagnosis is not clear or your doctor suspects a complication. For example:

• A scan to look at your internal organs and lymph glands.
• Special tests on your urine to look at your kidney function.

What are the aims of treatment?

There is currently no cure for Sjögren's syndrome. Treatment is aimed at controlling as many of the symptoms that it causes as well as possible.

What are the treatment options for Sjögren's syndrome?

You may be referred to a number of different specialists when you are diagnosed with Sjögren's syndrome depending on the parts of your body that it affects. Rheumatologists (joint specialists) are the main doctors who have specialist knowledge of Sjögren's syndrome. This is because of the association of Sjögren's syndrome with other diseases that affect the joints, such as rheumatoid arthritis and systemic lupus erythematosus. However, you may also be referred to a dentist, an eye specialist, a lung specialist or a kidney specialist. Your GP will continue to provide support for you and will usually prescribe your medication under the guidance of these other specialists.

Treatment of dry eyes

• Avoid situations that make dry eye symptoms worse.These may include:
  • Low humidity and air conditioning.
  • Wind.
  • Dust and smoke (so, if appropriate, try to stop smoking if you are a smoker).
  • Contact lenses.
  • Prolonged reading or staring at a computer screen or television. This makes us blink less often so our eyes don't stay as moist.
• Glasses: special glasses can help to keep in moisture and reduce eye dryness.
• Artificial tears: these provide lubrication and come in the form of eye drops. Preservative-free eye drops help to reduce the risk of eye irritation. Paraffin eye ointments are helpful for use at night time as they are longer lasting. However, if used during the day, paraffin ointment may cause blurred vision.
• Tear duct treatment: if drops alone do not work, you may need some special treatment to the tear ducts in your eyes by an eye specialist. An instrument called a diathermy is used to close up the tear ducts.

Treatment of dry mouth and related symptoms

• General measures that can help include:
  • Sip water throughout the day.
  • Keep your teeth, gums and mouth as clean and healthy possible. Brush your teeth regularly, use dental floss and a mouth wash.
  • Visit your dentist regularly.
  • Using Vaseline® for dry, cracked lips.
  • Chewing sugarless chewing gums.
• Artificial saliva: this can be used to keep the mouth moist and comes in the form of a spray, gel, liquid, lozenge or pastille.
• Saliva stimulants: in some people with Sjögren's syndrome, the saliva glands are only partially affected and can be stimulated to make more saliva. Pilocarpine tablets are saliva stimulants that are sometimes prescribed.

Treatment of other symptoms

• Moisturisers and special bath additives can be used for dry skin.
• Lubricants may be needed when you have sex.
• Non-steroidal anti-inflammatory drugs (NSAIDs) can be taken to help joint and muscle pains.

Treatment of complications

If Sjögren's syndrome progresses to involve organs such as the skin, lungs, kidneys and lymph glands, you may need to take some medication. Such medication may include:

• Steroids: these are tablets taken by mouth that help to reduce inflammation. They may be prescribed if your symptoms are particularly bad.
• Immunosuppressive agents: these are drugs that damp down the abnormal antibody production in Sjögren's syndrome. Names include methotrexate, penicillamine and hydroxychloroquine. As with steroids, they are reserved for severe cases as they do have side-effects and you will need close monitoring with blood tests while you are taking them. For example, you may be prescribed one of these drugs if your kidneys or lungs are affected, or if you develop pseudolymphoma.

What is the outlook (prognosis) of Sjögren's syndrome?

Sjögren's syndrome is not usually life-threatening. Some people may only notice mild symptoms such as mild dry eyes and a mild dry mouth. Other people develop more irritating and disabling symptoms affecting their eyes, mouth, vision and eating and can also have uncomfortable joint pains and tiredness.

Sometimes symptoms can go away for long periods of time (go into remission). Rarely, some people develop more serious problems such as the kidney and lung problems described above.

Some people with Sjögren's syndrome develop another autoimmune disorder such as rheumatoid arthritis, systemic lupus erythematosus or polymyositis.

As mentioned above, about 1 in 100 people with Sjögren's syndrome develop lymphoma, most commonly non-Hodgkin's lymphoma. This lymphoma can usually be treated and your doctor will review you regularly to look for signs of this.

Can Sjögren's syndrome be prevented?

So far, it does not seem that Sjögren's syndrome can be prevented. More research is needed into the factors that actually cause a person to start forming the autoantibodies involved in the disease.

Further help and information

British Sjögren's Syndrome Association

PO Box 15040, Birmingham B31 3DP
Helpline: 0121 478 1133 Web: www.bssa.uk.net

Arthritis Research UK

Copeman House, St Mary's Court, St Mary's Gate, Chesterfield, Derbyshire, S41 7TD.
Tel: 0870 850 5000 Web: www.arthritisresearchuk.org

Arthritis Care

18 Stephenson Way, London, NW1 2HD.
Helpline: 0808 800 4050 Tel: 0207 380 6500 Web: www.arthritiscare.org.uk

The Sjögren's Registry

A national research biobank of people with Primary Sjögren's Syndrome. The aim is to facilitate clinical trials and academic research studies in order to improve our understanding of what causes Primary Sjögren's Syndrome and to find better, more effective treatment for people with this condition.
Web: www.sjogrensregistry.org

References

• Phelan D; Sjogren Syndrome. eMedicine, 2009.
• Thomas E, Hay EM, Hajeer A, et al; Sjogren's syndrome: a community-based study of prevalence and impact. Br J Rheumatol. 1998 Oct;37(10):1069-76. [abstract]
• Manthorpe R; Sjogren's syndrome criteria. Ann Rheum Dis. 2002 Jun;61(6):482-4.