Sjogren's Syndrome

This is an autoimmune condition which causes the body’s immune system to attack healthy cells and tissue, mistaking them for harmful threats. With Sjogren’s syndrome the immune system attacks the glands responsible for producing moisture in the eyes and mouth, which causes them to become dry. Sjogren’s syndrome usually affects the eyes and mouth, but other areas of the body may be affected. It can result in a wide assortment of signs and symptoms.

The primary role of the immune system is to protect the body from illness and infection, which it does by attacking viruses and harmful bacteria. However, in autoimmune conditions, the immune system mistakes healthy cells for threats and starts to attack them. In a person with Sjogren’s syndrome the immune system attacks the salivary and lacrimal glands, preventing them from producing tears and saliva as normal and causing the eyes and mouth to become dry. The medical names for dry mouth and dry eyes are keratoconjunctivitis sicca and xerostomia.

It is possible for Sjogren’s syndrome to affect other glands in the body, including those located in the stomach, intestines and pancreas. The condition can also bring about dryness in other areas of the body, including the nose, airways, throat and skin.

Sjogren’s syndrome is sometimes referred to as a rheumatic disease. Rheumatic diseases cause inflammation and pain in the joints and muscles and similar symptoms can be caused by Sjogren’s disease. Sjogren’s disease may also be classed as a connective tissue disease, with the connective tissues being responsible for supporting the organs and other types of tissue, including muscles and joints.

Sjogren’s disease is classified as primary and secondary. Primary Sjogren’s syndrome occurs alone, while secondary Sjogren’s syndrome occurs in combination with another disease. Both types of Sjogren’s syndrome are systemic, but the symptoms are usually restricted to the eyes and mouth when it is primary Sjogren’s syndrome.

In the case of primary Sjogren’s syndrome, symptoms are usually restricted to the mouth and eyes and a specific form of antibodies, known as SS-B and SS-A can be detected. The level of antibodies is usually higher in individuals with primary Sjogren’s syndrome than those with secondary Sjogren’s syndrome. These antibodies are known as ANA (antinuclear) antibodies, which are produced to fight against threats (in this case, the body).

With secondary Sjogren’s syndrome it is likely that the individual already suffered from an autoimmune condition, such as lupus or rheumatoid arthritis, before the condition developed. Individuals with Sjogren’s syndrome often have more diverse symptoms because their body is affected by two conditions. They are also often found to have lower levels of ANA antibodies than those with primary Sjogren’s syndrome.

What are the symptoms of Sjogren’s disease?

• Dry mouth: when you have dry mouth it feels as though your mouth is stuffed full of cotton and this makes it difficult to speak, swallow and taste food. It is also possible for dry mouth to change your sense of smell and a dry cough may develop. Dry mouth raises your risk of infections as a result of decreased saliva, which contains protective elements.
• Dry eyes: dry eyes can become irritated, itchy and may look red. Many people describe the feeling as similar to having sand in their eyes. Dry eyes can also cause vision to become blurred and you may experience heightened sensitivity to light, especially bright or fluorescent lighting.

Sjogren’s syndrome can also have an effect on other areas of the body and additional symptoms may include:

• Dry skin.
• Rashes.
• Problems associated with the thyroid gland.
• Pain in the joints and muscles.
• Pneumonia.
• Tingling and numbness in the fingers and toes.
• Vaginal dryness.
• Severe fatigue.

When Sjogren’s syndrome affects other areas of the body it is known as extraglandular involvement.

Who is affected by Sjogren’s syndrome?

It is thought that three to four percent of adults in the UK are affected by Sjogren’s syndrome. This kind of statistic takes it just behind rheumatoid arthritis as the most common type of autoimmune condition. The condition most commonly affects women at a rate of nine out of ten cases. Age is no factor but the majority of cases start in people aged thirty to sixty years old. The condition affects all ethnicities equally and it is not unknown for children to be affected.

What are the causes of Sjogren’s syndrome?

Researchers believe that Sjogren’s syndrome is brought on by a mixture of environmental and genetic factors. However, scientists are not sure which specific genes are involved because different genes are believed to affect people in different ways. One gene, for example, has been identified as increasing the risk of a Caucasian person developing Sjogren’s syndrome, while other genes seem to be linked to people of different ethnicities.

Scientists believe that there are certain triggers which cause people with specific genes to develop the condition. Simply having the gene is not enough to develop Sjogren’s syndrome. But if there is a trigger, such as a bacterial or viral infection, this can cause the condition to develop. The theory behind the trigger is that the body’s immune system responds to the infection but the process is interrupted by the gene, which causes cells that are supposed to fight infection (the lymphocytes) to travel to the mouth and eyes and alter the action of the salivary and lacrimal glands. The lymphocytes are intended to die once they have finished attacking a threat (this process is known as apoptosis). However, individuals with Sjogren’s syndrome continue to have lymphocytes attack tissue and cause more damage. Scientists believe that resistance to the natural apoptosis process is caused by genetics. Research is also being carried out into the role of the nervous and endocrine systems.

What is the diagnosis process for Sjogren’s syndrome?

When you see your doctor they will ask you about your symptoms and take a thorough medical history, before carrying out a physical examination to check for symptoms of Sjogren’s syndrome. This examination will involve the doctor checking your mouth and eyes. Your doctor may also advise the following tests to see if the condition is affecting other areas of the body:

• Schirmer’s test: determines how well the tear glands are working and there are two methods. The first method involves putting fine strips of paper under the eyelids and checking for moisture after five minutes. Individuals with Sjogren’s syndrome typically manufacture lower than 8mm of tears. The second method is alike but involves using a cotton bud to stimulate the tear reflex in the nose.
• Staining: staining tests use dyes to highlight harm on the plane of the eyes as a result of dryness. The doctor places a drop of dye on the lower eyelid and looks for signs of damage.
• Slit lamp examination: used to determine the extent of damage to the eye and check for signs of inflammation in the outer eye. An ophthalmologist examines the eye using a special instrument known as a slit lamp.
• Mouth examination: a doctor will examine the interior of the mouth to check for indications of dry mouth, inflamed salivary glands, sticky saliva, mouth redness and cavities. A sample of saliva may also be taken.
• Biopsy: a biopsy sample of salivary gland tissue is taken from the lower lip and analysed under a microscope. This looks for the presence of a series of antibodies, with a positive test result indicating Sjogren’s syndrome.

Most doctors will confirm a diagnosis of Sjogren’s syndrome if a patient has dry mouth and dry eyes and a positive salivary gland biopsy result. However, additional tests may be carried out to see if other areas of the body are impacted, including:

• Blood tests: blood tests will be carried out to check liver and kidney function and monitor blood glucose levels and blood count.
• Immunological tests: used to check for the presence of antibodies and include:
• Antithyroid antibodies: produced when the antibodies move from the salivary glands to the thyroid gland.
• Immunoglobulins and gamma globulins: everyone has these antibodies in their blood but levels are generally much higher in individuals with Sjogren’s syndrome.
• Rheumatoid factors: these are apparent in individuals with rheumatoid arthritis and Sjogren’s syndrome. If antibodies recognised as cryoglobulins are detected there may be an increased danger of lymphoma.
• Antinuclear antibodies.
• Chest X-ray.
• Urinalysis: this is analysis of urine samples.

What doctor provides diagnosis and treatment for Sjogren’s syndrome?

It can take a long time for Sjogren’s syndrome to be diagnosed since the symptoms are linked to many other conditions. The diagnostic process can sometimes take years and a patient may see many different healthcare professionals in this time, including:

• An allergist.
• A dentist.
• A dermatologist (a skin specialist).
• A gastroenterologist (a specialist in digestive conditions).
• A gynaecologist (a specialist in women’s reproductive disorders and problems).
• A neurologist (a brain specialist).
• An ophthalmologist (an eye specialist).
• An otolaryngologist (an ear, nose and throat specialist).
• A pulmonologist (a lung specialist).
• Urologist.

What treatment is available for Sjogren’s syndrome?

Treatment for Sjogren’s syndrome depends on the individual and which areas of the body are impacted. The aim of treatment is to ease symptoms. Those with extraglandular involvement (when other areas of the body are influenced) will also be administered treatment for these problems. Possible treatments for Sjogren’s syndrome include artificial tears, saliva stimulants, non-steroidal anti-inflammatory drugs for joint pain, corticosteroids and immunosuppressant medications to reduce the action of the immune system.

What can be done about dry eyes?

Artificial tears can be helpful to lubricate the eyes. Tears come in various thicknesses and it may be beneficial to try different types to find which ones you prefer. It is advisable to use artificial tears without added preservatives since these can irritate the eyes. It is common for drops without preservatives to be available in single doses to avoid contamination.

Eye ointments can be useful during the night as they provide more moisturisation than artificial tears and last for longer. Ointments may cause mild blurring of vision but this should not be a problem during the night.

A chemical called hydroxypropyl methylcellulose is used to lubricate the surfaces of the eyes. The chemical is contained in a pellet, which is placed in the lower eyelid. The pellet then dissolves at the time you put in artificial tears, forming a protective film over the eye and preventing dryness.

Surgery may also be considered. A procedure to shut the tear ducts to prevent tears draining from the eyes may be carried out (known as punctal occlusion). For short-term results silicone or collagen plugs may be inserted into the tear ducts. For a more long-term solution laser surgery can be used to permanently seal the ducts closed.

What can be done about dry mouth?

If the saliva glands are still able to produce saliva you can increase production through the stimulation of the glands by sucking a sweet or chewing gum. Always ensure that the sweets and gum you use are sugar-free, as dry mouth increases the risk of cavities. Try to drink plenty of water as this will keep your mouth moist. Sipping water all through the day and after eating is particularly beneficial. If your lips are sore or chapped you can ease discomfort by using lip balm, but try to avoid perfumed sticks which can cause irritation.

If the glands are no longer producing saliva or producing a small amount, your doctor may advise using a saliva substitute. These aim to mimic natural saliva and keep the mouth moist. It is also possible to buy gels that contain fluoride to help in the prevention of cavities.

Medication can be taken to encourage the salivary glands and there are currently two drugs used for this purpose. They are called pilocarpine and cevimeline. In most cases drugs are taken around three times per day but they are not appropriate for everyone. Your doctor will be able to offer advice on whether the drugs are suitable for you.

Dry mouth increases the risk of infections in the mouth. One of the main infections in persons with Sjogren’s disease is a fungal mouth infection known as candidiasis. Symptoms include white patches or areas of red in the mouth which have a burning sensation. Candidiasis can be treated with antifungal medication.

Which other areas of the body are affected by Sjogren’s syndrome?

The autoimmune response brought on by Sjogren’s syndrome can impact other areas of the body. It is not uncommon for individuals with Sjogren’s syndrome to experience problems associated with their kidneys, skin, lungs and nervous system. Here are some examples of extraglandular involvement:

Skin problems

Around half of people with Sjogren’s syndrome have dry skin. Some people may experience only mild itching, but for others it can be severe and they may develop cracked, sore skin. If the skin is cracked the risk of infection is higher. The skin tends to be darker in areas of infection, but it usually returns to normal after the infection has cleared.

Treatment for dry skin involves using an intensive moisturiser on a regular basis. Creams are better than lotions because lotions evaporate quicker. When you shower try to keep it short and pat your skin dry, rather than rubbing it firmly, and moisturise afterwards. Sleeping with a humidifier in your bedroom can also help to keep the skin moist.

Sjogren’s syndrome can cause increased sensitivity to sunlight (this is particularly common in people who also have lupus) so it is vital to protect the skin when out in the sun. Avoid exposure to the sun for prolonged periods of time and use sun cream (at least factor 15).

Vaginal dryness

Vaginal dryness is a common effect of Sjogren’s syndrome which can cause pain, especially during intercourse. Using a vaginal moisturiser can help, as they attract moisture to the dry tissues. Lubricant should only be used during intercourse, as it does not moisturise and it is better to use water-based lubricants. Oil-based products can result in sores and affect the natural cleansing process. Regular moisturiser can be used to soothe dry skin on the vulva (the outer part of the vagina).

Lung problems

Dry mouth can increase the risk of lung problems, since it affects the ability to swallow properly and bacteria from the mouth can travel to the lungs causing infection. Possible conditions associated with Sjogren’s syndrome include pneumonia. Pneumonia can be treated effectively with antibiotics and different types are used depending on the category of infection. It is important for pneumonia to be treated as it can develop into a lung abscess.

Lung conditions brought about by irritation, including bronchitis, tracheobronchitis and laryngotracheobronchitis, can also be caused by Sjogren’s syndrome. Doctors may advocate using a humidifier and taking medication to reduce inflammation (corticosteroids) and open up the bronchial tubes. Pleurisy is a condition which occurs when the lining of the lungs become inflamed. It can be dealt with by corticosteroids and non-steroidal anti-inflammatory medication.

Kidney problems

Your kidneys are very important as they remove waste from the blood so that it can be excreted through the urine. In persons with Sjogren’s syndrome it is common for the tissue in proximity to the filter section of the kidneys to become inflamed (known as interstitial nephritis). In less common cases the actual filters can become inflamed (known as glomerulonephritis). Some people also develop a condition called renal tubular acidosis, in which they cannot remove some acids by means of urine. This leads to the level of potassium in the blood to fall and this causes an imbalance which may be harmful for the nervous system, heart and muscles.

Doctors will often provide no treatment for kidney issues unless they are causing symptoms or affecting the function of the kidneys. However, they will monitor the situation closely and carry out regular tests. In some cases alkaline agents may be prescribed to balance the chemicals in the blood. In severe cases immunosuppressants or corticosteroids may be prescribed.

Nerve problems

Some people develop problems with their nerves. In most cases this relates to the peripheral nervous system, which controls movement and sensation. Examples of conditions that develop in persons with Sjogren’s syndrome include peripheral neuropathy, carpal tunnel syndrome and cranial neuropathy. Carpal tunnel syndrome is brought on by irritated tissue in the forearm pressing on the median nerve, which causes pain, tingling, numbness and can also result in weakness to muscles in the fingers and thumb. Peripheral neuropathy occurs when the nerves in the arms and legs are attacked, which causes tingling and numbness. Cranial neuropathy causes pain in the face and a loss of sensation in the face and facial features. Nerve problems are usually treated with medication to reduce pain and, where required, reduce inflammation.

Digestive problems

Irritation in the digestive system can cause symptoms including difficulty swallowing, heart burn, abdominal pain, decreased appetite and loss of weight. Sjogren’s syndrome is also linked to cirrhosis (scarring) of the liver, hepatitis and, most commonly, a condition known as primary biliary cirrhosis, which causes itching and extreme tiredness and, finally, cirrhosis. Treatment varies according to the individual but may comprise anti-inflammatory medication, steroids and immunosuppressants.

Connective tissue disorders

Connective tissue forms a framework which supports the tissues and organs in the body. Examples of connective tissue include muscles, joints, bones, skin and the walls of blood vessels. A large proportion of connective tissue disorders are also autoimmune, including:

• Polymyositis: this is irritation of the muscles which causes weakness, pain and restricted movement. If the skin is also inflamed this is known as dermatomyositis. The condition is dealt with through the use of immunosuppressant medication and corticosteroids.

• Raynaud’s phenomenon: this condition causes the blood vessels to constrict in response to the cold, which results in tingling, numbness and the fingers to turn white and then blue as the blood starts to pool in the tissues. Treatment involves taking medication to dilate the blood vessels. Sometimes this condition can occur before dry mouth and dry eyes.

• Rheumatoid arthritis: this is a severe condition which causes the joints to become inflamed and can also cause damage to the blood vessels, muscles and organs. Treatment will come down to the severity of pain, swelling and the areas of the body affected. Possible treatments include non-steroidal anti-inflammatory drugs, physiotherapy, rest, steroids and immunosuppressants.

• Scleroderma: this condition results in the body collecting excessive collagen, a protein which is found in many of the body’s cells. The result of having too much collagen is increasingly thick skin and harm to the muscles, joints and organs, including the lungs, heart, oesophagus and intestines. Treatment is designed to ease pain and may include medication and physiotherapy.

• Systemic lupus erythematosus: this condition results in muscle and joint pains, rashes, muscle weakness and problems with the nerves, heart and kidneys in more harsh cases. Treatment is based on the symptoms but can include physiotherapy, anti-inflammatory medication, rest and dialysis.

• Vasculitis: this condition causes the blood vessels to become inflamed. This eventually causes them to become scarred, constricted and makes it difficult for blood to flow freely. Individuals who have Sjogren’s syndrome are more inclined to be diagnosed with vasculitis if they also have Raynaud’s phenomenon, or liver or lung problems.

• Autoimmune thyroid disorders: it is common for individuals with Sjogren’s syndrome to have autoimmune thyroid disorders, which may include Graves’ disease. This is linked to an overactive thyroid gland or Hashimoto’s disease, which is linked to an underactive thyroid gland. Almost half of individuals with Sjogren’s syndrome have autoimmune thyroid disorders.

Does Sjogren’s syndrome result in lymphoma?

Around 5% of individuals with Sjogren’s syndrome get cancer of the lymph nodes (known as lymphoma). The main symptom of this is painful swelling in the lymph nodes in the neck, groin and underarms. In persons with Sjogren’s syndrome, lymphoma is more common in the salivary glands. If the salivary glands are inflamed on a regular basis or for a prolonged period of time, this should be investigated by a clinician. Other symptoms to look out for include:

• Unexplained weight loss.
• Fever (high temperature).
• Constantly feeling fatigued.
• Night sweats.
• Itchy skin.
• Patches of red on the skin.

These symptoms may be linked to a number of health conditions and do not necessarily mean an individual has lymphoma. However, it is always worth getting checked out as early diagnosis and treatment increases survival rates considerably. If you are worried about lymphoma, speak to your GP.

Medication and dryness

Certain medications can result in dryness of the eyes and mouth. If you have such symptoms and you are taking medication, ask your doctor if the medication could be contributing to your symptoms. It is imperative to not stop taking medication without the advice of your doctor first. Medications which can result in dryness include:

• Diuretics.
• Antihistamines.
• Some drugs used to treat diarrhoea.
• Decongestants.
• Some antipsychotic medications.
• Antidepressants.
• Tranquilisers.
• Some blood pressure medications.