Sjögren's syndrome

Sjögren's (pronounced 'Shurgren's') syndrome is an autoimmune disorder. Your body's immune system usually fights infections, but with Sjögren's syndrome it attacks its own tissues instead – particularly the tear glands and the salivary glands, causing dryness of the eyes and mouth.

It's estimated that half a million people in the UK may be affected by Sjögren's syndrome but the exact figures aren't known as many people never see a doctor about their symptoms. Sjögren's syndrome can occur on its own (primary) but can also occur in association with another rheumatic disease such as rheumatoid arthritis.

Read entire article »

Sjögren's syndrome symptoms

The most common symptoms are dry eyes and/or a dry mouth, feeling tired and achy. Many people don't have any other symptoms. However, the range and severity of symptoms can vary a great deal from person to person.

  • Eye problems – The eyes are dry and may also be sore or irritable. Some people find strong lights can be uncomfortable, while others find their eyes become sticky with mucus
  • Mouth and throat problems – The mouth is dry, and there may be mouth ulcers which can sometimes cause a sticky feeling in the mouth or throat. Swallowing may be difficult and some people find their sense of taste is altered. The voice may be hoarse or weak, and some people have a dry cough. Occasionally a very dry mouth can lead to other problems such as fungal infections, or the salivary glands may be painful

  • Extreme tiredness – Fatigue is one of the most common symptoms, and some people may also feel down or depressed
  • Aches and pains – The joints may be painful and swollen due to inflammation, while some people have a general achy feeling and/or tender spots.

Other problems that can sometimes be associated with Sjögren's syndrome include:

  • dry, itchy skin or increased sensitivity to strong sunlight
  • a dry vagina leading to painful intercourse (known as dyspareunia)
  • fever
  • cold, blue fingers (known as Raynaud's phenomenon)
  • migraine-like headaches
  • lower abdominal pain and irritable bowel syndrome
  • swollen lymph glands in your neck, armpits or groin
  • aggravated menopausal symptoms
  • problems with your nervous system, such as weakness or numbness
  • inflamed blood vessels (known as vasculitis)
  • chest pain (caused by pleurisy) or breathlessness
  • liver or kidney problems.

Who gets Sjögren's syndrome?

Women aged between 40 and 60 are most likely to be diagnosed with Sjögren's syndrome. Only 1 in 10 Sjögren's syndrome patients are men, and the condition occurs only rarely in childhood.

It is possible that some people are more likely to get Sjögren's syndrome because of hereditary (genetic) factors that mean they find it difficult to get rid of certain infections. However, it isn't common for children to inherit the condition from their mother or father.

What causes Sjögren's syndrome?

Sjögren's syndrome is an autoimmune disorder, so the body's immune system attacks the body's own tissues – mainly the tear-producing glands, such as the lacrimal glands behind the eyelids, and the salivary glands in the mouth. The body produces antibodies that react with and damage the tissues and nerve signals to the glands, reducing the amount of saliva and tears produced.

We don't yet know what causes the immune system to behave in this way in Sjögren's syndrome. It's been suggested that it could be triggered by certain viruses, such as the Epstein-Barr virus, the retroviruses and the HTLV group of viruses. However, the evidence for this is weak.

How does Sjögren's syndrome progress?

You aren't likely to be disabled by Sjögren's syndrome, even though the symptoms can be uncomfortable and lasting. In a few people the joints may become inflamed, or the liver or kidneys may be affected. However, usually the condition only damages the tear- and saliva-producing glands.

Certain people with Sjögren's syndrome might have an increased risk of developing cancers of the lymphatic tissues (known as lymphoma), but lymphoma associated with Sjögren's syndrome can be treated. You and your doctor should look out for any early signs, such as enlarged lymph glands.(in your neck, armpits or groin) or salivary glands that are persistently swollen.

How is Sjögren's syndrome diagnosed?

Because you might have a number of different complaints with Sjögren’s syndrome, you might need to see various medical professionals before you are diagnosed, including:

·         your family doctor (GP)

·         a rheumatologist

·         an eye specialist (known as an ophthalmologist)

·         a dentist or oral surgeon.

Dryness of the eyes and mouth can have other causes – for example:

  • sicca syndrome, which can occur as people get older or when the tear- or saliva-producing glands are damaged
  • certain medications such as antihistamines and antidepressants
  • inflammation of the oil-producing glands in the eyelids (blepharitis) which causes excessive evaporation of the tear film.

It’s important therefore to see your doctor to get an accurate diagnosis.

The main tests that can help with diagnosis are:

  • Schirmer's test – A small piece of sterilised, pre-packaged blotting paper is used to measure your tear production
  • Slit lamp examination – An eye specialist puts dye (fluorescein and sometimes lissamine green dye) in your eyes and uses an instrument called a slit lamp, which shines a beam of light through a narrow slot, to examine them. The slit-beam and magnification allows the surface of the eye to be examined more effectively
  • Saliva production measurement – You may be asked to spit into a paper cup over a period of time
  • X-rays – Images of the salivary glands and ducts may be taken after a contrast dye has been injected (sialography). Ultrasound scans or occasionally magnetic resonance imaging (MRI) may also be used
  • Blood tests – Determine whether you have the anti-Ro and anti-La antibodies, which are found in 75% (anti-Ro) and 60% (anti-La) of people with primary Sjögren's syndrome and are rare in other conditions
  • Lip biopsy – Several tiny salivary glands can be removed from your lower lip, under a local anaesthetic, and examined under a microscope – this is usually only done if the diagnosis is still uncertain after the other tests.

Further specialized tests may be required if you develop swelling in the lymph glands (in the neck, armpits or groin), or if you have complications involving the chest or liver.

Sjögren's syndrome treatments

There is no cure as yet for Sjögren's syndrome, but the most common symptoms can usually be treated with a combination of specific medications and self-help measures.

 

Eye symptoms

What could help

Dry/sore/irritable eyes

 

Lubricant eye drops, such as hypromellose, Sno Tears or Viscotears used a few times a day

If  eye drops are needed more than six times a day, up to hourly, a preservative-free formulation should be used (e.g. artelac, hypromellose preservative-free, viscotears single-dose units, celluvisc, optive) as frequent use of drops containing preservative can increase dryness.

Mucous and debris causing stickiness

Mucolytic agents such as acetylcysteine eye drops

Very dry eyes

Note: If you have corneal ulcers or infections you must see an eye specialist and NOT use these treatments.

Preservative-free eye drops, ointments (including ciclosporin) or pseudoplastic drops (Celluvisc, viscotears single dose units, hyaluronate preparations).

Paraffin-based ointments such as Lacrilube at night if eyes are dry and crusty on waking

Punctal occlusion – insertion of punctal plugs (by an eye specialist) to reduce the drainage of tears (and eye drops) from the surface of the eye.  If the eyes remain very dry permanent punctal occlusion by cauterisation can be performed.

Mouth symptoms

What could help

Very dry mouth

Taste, voice or swallowing affected

Artificial saliva such as Biotene OralBalance or BioXtra gels

Luborant or AS Saliva Orthana mouth sprays are recommended if you have your own teeth because they contain fluoride.

Glandosane mouth spray is best avoided in people with their own teeth as it is more acidic and may increase the risk of dental decay.

Chlorhexidine mouth rinses, such as Corsodyl, may help with dental hygiene.

Fluoride mouthwashes or high concentration fluoride toothpastes (Duraphat) can be used to help reduce dental decay.

Sugar-free chewing gum, or other dental care products or lozenges, such as anhydrous crystalline maltose

Pilocarpine (Salagen) can stimulate saliva production in people who are able to produce some saliva.

Mouth ulcers

Adcortyl in Orabase ointment can be applied to the ulcers

If this doesn't help, your doctor may prescribe corticosteroid pellets for you to keep in your mouth for a few times a day.

Difflam oral rinse or spray can help relieve discomfort.

Mouth ulcers causing a sticky mouth or throat

A diluted chlorhexidine mouthwash, such as Corsodyl

Rinsing and gargling with a mouthwash made from a mixture of a teaspoon salt, a teaspoon of baking powder and a litre of water.

Mouth is severely dry, causing other problems such as fungal infection, and a dry cough

An antifungal treatment such as nystatin

 

Dry cough

Humidifiers,  plants with large leaves, or bowls of water can help by increasing humidity in rooms.

Avoiding drugs that may aggravate symptoms, such as antihistamines, travel-sickness pills and antidepressants.

Your salivary glands hurt

 

Ask your doctor for further assessment and advice. S/he will check whether you have an infection. If your salivary glands are not infected, s/he may prescribe a short course of a corticosteroid called prednisolone.

Other symptoms

What could help

Joint pain

 

General aching or tender spots

 

Extreme tiredness, lethargy, low spirits

 

Poor concentration and difficulty in thinking clearly

Painkillers such as paracetamol and/or non-steroidal anti-inflammatory drugs (NSAIDs), such as ibuprofen

Have blood tests to check the thyroid gland and a screen for coeliac disease.

Lifestyle changes to take sensible exercise and pace daily activities to match capacity.

If joints are inflamed an intramuscular steroid injection (Depomedrone) or short course of steroid tablets may be suggested for short-term relief. Hydroxychloroquine can be used for longer-term treatment (for 6 months to many years).

Fever

Exclude more serious problems such as infection, thyroid problem or lymphoma.

Hydroxychloroquine and/or corticosteroids can be tried.

Fingers feel cold and turn blue (Raynaud's phenomenon)

Drugs such as nifedipine which open up the blood vessels

Migraine-like headaches

Painkillers or specific anti-migraine medications

Lower abdominal pain and disordered bowel habits (irritable bowel syndrome)

Antispasmodic drugs, such as mebeverine (Colofac)

Aggravated menopausal symptoms

Hormone replacement therapy (HRT) can be used if necessary, but we recommend you discuss the risk of side-effects with your doctor.

Neuropathy (problems with the peripheral nervous system such as weakness or numbness), vasculitis, or a severe condition overall that is not helped by other treatments.

Painkillers including gabapentin or pregabolin can be used for neuropathies where pain is the main problem. Hydroxychloroquine is also often used.

Patients with severe progressive disease may need high dose corticosteroids and immunosuppressants such as cyclophosphamide.

For progressive neuropathies with weakness or a severe condition overall, newer (still experimental) drugs include rituximab which targets B-cells of the immune system and mycophenolate which is an immunosuppressant that has been helpful in some patients with systemic lupus erythematosus.

Mild liver abnormalities are not common and probably won't require treatment, but you should have regular checks, and specialist treatment may be needed if a condition called primary biliary cirrhosis develops.

The lungs can occasionally be affected by fibrosis, which can cause breathlessness on exertion, a dry cough and chest pain. Fibrosis requires specialist advice but corticosteroids can be prescribed.

Self-help and daily living

This section covers: general advice, exercise, diet and nutrition and sex and pregnancy. Some Sjögren's syndrome symptoms can be helped by simple self-help measures. For example:

  • Wear wraparound sunglasses or glasses with side shields to help retain moisture in the eyes
  • Maintain good hygiene around the eyelid margin to maximize oil secretion from glands in the eyelids
  • Avoid contact lenses if your eyes are dry
  • Increase the humidity in your home. This can help eye symptoms and a dry cough. Humidifiers, large-leaved houseplants or bowls of water all help to increase humidity in a room
  • Consider wearing tinted glasses if you find strong lights uncomfortable
  • Avoid strong soaps if you have dry, itchy skin. Aqueous creams and emollients may be helpful
  • Wear sunscreen (factor 15 or higher) if your skin is sensitive to the sun or comes out in a rash
  • Wear warm gloves if your hands turn blue in the cold (Raynaud's phenomenon). Exercise will help by improving your circulation, but smoking should be avoided because it is bad for the circulation
  • Good dental hygiene, or proper denture care, are recommended for any oral symptoms
  • Only take medications such as antibiotics if it's really necessary as Sjögren's syndrome can slightly increase the risk of side-effects.

Exercise

Exercise is recommended, particularly if you have symptoms in your joints, to maintain mobility. But you will need to find the right balance between rest and exercise, especially if you also suffer from extreme tiredness.

Diet and nutrition

No special diet is recommended for Sjögren's syndrome. However, if you have abdominal pain or bowel problems, increasing the amount of fibre in your diet should help.

Sex and pregnancy

One less common symptom of Sjögren's syndrome may be that you have a dry vagina, which can make sex painful. Lubricants such as KY Jelly or Astrogyde should help and so can oestrogen creams. Treatments for infections such as thrush are readily available from chemists.

There are not usually any increased problems during or after pregnancy with Sjögren's syndrome. A few newborn babies of mothers with primary Sjögren's syndrome may have disturbed heart rhythms, as a result of the antibodies anti-Ro and anti-La being passed on. But this is very rare.

Share |