What is Sjögren’s Syndrome?

Discovered in Sweden in the early 19th century, Sjögren’s syndrome is named after ophthalmologist Henrik Sjögren when he noticed that a group of women suffering from arthritis also had symptoms characterised by dry eyes and dry mouths.

Today, the exact number of people in the UK with Sjögren’s syndrome is unknown, but it is estimated to be up to 500,000 people. It is one of the most common autoimmune rheumatic diseases in the UK, but one of the most underdiagnosed.

What is Sjögren’s syndrome?

Sjögren’s syndrome is an autoimmune disease, meaning that the human body cannot distinguish between the cells in the body and cells which are not native to the body, thus attacking normal body cells. Sjögren’s syndrome is one of around 80 existing autoimmune diseases.

Pronounced ‘show-grins’, Sjögren’s syndrome is also a rheumatic (inflammatory) medical condition, affecting the production of fluid within the body. It causes dryness in different body parts. Some sufferers of Sjögren’s syndrome experience mild symptoms, whereas for others, it has detrimental effects on their quality of life.

Whilst it is a painful and uncomfortable condition to live with, if people with Sjögren’s syndrome do not develop severe secondary complications, they should not expect a shorter life span than those without the condition.

What causes Sjögren’s syndrome?

Sjögren’s syndrome occurs when white blood cells, which are formed to defend the body from infection, are mistakenly sent to glands that produce fluid, such as saliva and tear glands, causing the production of fluid in those glands to reduce significantly. This affects the entire body. It is not known exactly why Sjögren’s syndrome occurs, but it can develop by itself.

Secondary Sjögren’s syndrome can be triggered by other autoimmune conditions or viral infections. The condition is considered to be relatively common, and one that is typically found to be present in conjunction with other chronic autoimmune conditions, such as rheumatoid arthritis or lupus.

However, genetics can play a part in how likely someone is to develop Sjögren’s syndrome, as the genes IRF5 and STAT4 have been associated with the condition.

Nevertheless, it is still likely that no matter the role of the genes, the condition must be triggered by some kind of environmental factor, such as an infection.

Sjögren’s syndrome overwhelmingly affects women over the age of 40 years old, though it can occur at any age, including during childhood, and also affects men. It is believed that women are much more likely to develop Sjögren’s syndrome due to the connection between the change in sex hormone levels at the menopausal age, and the immune system.

Oestrogen is known to regulate immune responses, and during menopause, the levels of oestrogen in the female body drop significantly.

What are the signs and symptoms of Sjögren’s syndrome?

As previously stated, Sjögren’s syndrome affects the glands that produce and secrete fluid, thus, many areas of the body are affected.

Symptoms include:

• Dry eyes, which causes burning, redness, itchiness and a feeling of roughness, as well as blurred vision.
• A loss or difference in taste and smell, due to the lack of production of saliva and mucus.
• Problems with chewing.
• Issues swallowing.
• Problems talking.
• A dry throat and dry cough.
• Vaginal dryness.
• Tooth loss, tooth sensitivity and tooth decay.
• Dry skin and rashes.
• Swollen salivary glands.
• Sensitivity to light.

Additionally, many people with Sjögren’s syndrome experience muscle and joint pain, due to the lack of lubrication in their body, and fatigue. Issues with chewing and swallowing may also lead to weight loss.

How does Sjögren’s syndrome affect the body?

Sjögren’s syndrome affects the body in a number of ways. Physically, the dryness experienced within the body causes a large amount of pain and discomfort, as fluid is needed for many of the body’s core processes to function well, such as eyesight and eating.

Dryness of the mouth, nose, skin and vagina can not only be irritating, but very painful. Dryness can also affect other internal processes, such as the nervous system and the digestive system.

Many people with Sjögren’s syndrome report similar symptoms to those who have fibromyalgia – chronic pain caused by inflammation, and fatigue. Sjögren’s syndrome can be mild, and can improve, but it can also worsen over time. There is no reported difference in life expectancy between someone suffering from Sjögren’s syndrome and the general population, though there is a large difference in their quality of life, with pain causing physical and mental distress.

People with the condition are more likely to be unable to work than those without it, and rates of depression are far greater. Furthermore, those with the condition spend more time in hospital, experiencing infections more frequently, such as UTIs.

Sjögren’s syndrome can also lead to severe complications such as blindness, toothlessness and non-Hodgkin lymphoma. Blindness can occur as the dryness experienced in the eyes can cause ulcers to build on the cornea. This can be treated if detected early enough, although if left untreated, vision impairment and blindness can occur.

Non-Hodgkin lymphoma is the most severe and grave complication of Sjögren’s syndrome. It is a type of cancer affecting the lymphatic system, and those who suffer from Sjögren’s syndrome are between 6 and 9 times more likely to develop the disease, though the risk still remains small, as it is a relatively rare type of cancer.

People with Sjögren’s disease have overactive B lymphocyte cells, which produce antibodies and attack infected cells, but the overactivity of these cells triggers an inflammatory response, causing damage to fluid-producing glands, which can make the individual more likely to develop lymphoma.

Toothlessness may occur as saliva is needed to maintain the minerals in the hard dental tissue which protect the teeth from bacterial damage. As the rate of saliva flow is reduced, the bacteria remain in the mouth, as there is no fluid to wash them out.

This can cause gingivitis and periodontitis, which can cause teeth to weaken, decay, and even fall out. It can also cause halitosis, though as sufferers of Sjögren’s syndrome may have an impaired sense of smell, they may not recognise the smell. People suffering from dry mouth should have regular dental check-ups.

People with Sjögren’s syndrome may develop issues with their lungs and may be more prone to infections in the lungs, scarring of the lung tissue and bronchiectasis. This will be worsened for people who also smoke.

There can also be complications for women with Sjögren’s syndrome who are pregnant, such as a rash on the baby, and heart complications. Women with Sjögren’s syndrome are not any less likely to become pregnant but may need additional care throughout.

Other health conditions which are associated with Sjögren’s syndrome include irritable bowel syndrome, kidney inflammation and vasculitis. People with Sjögren’s syndrome may also have Raynaud’s syndrome, where the blood flow to the hands and feet is limited, causing numbness and coldness in the extremities, as well as pain. All of these conditions are linked to inflammation.

You can read more about Raynaud’s disease by visiting our knowledge base.

How is Sjögren’s syndrome diagnosed?

Diagnosing Sjögren’s syndrome can be a long process, as there isn’t a singular test that can conclusively show the presence of the condition.

Men can often be misdiagnosed, due to the prevalence of the condition in women, and diagnosis can generally take months, and even years, which can lead to the worsening of the condition, including organ damage, and some of the more severe secondary impacts mentioned previously.

• Doctors can order blood tests to be taken, which search for the presence of antibodies in the blood. However, it is possible that some people with Sjögren’s syndrome might not have these antibodies, which can further delay diagnosis as it may be concluded that the individual does not have Sjögren’s syndrome.
• Doctors may perform a lip biopsy, where they extract a tiny sample of tissue from the inside of the lip to check the lip tissue for the presence of white blood cells. They examine this to see if white blood cells have gathered inside of the cells that usually produce saliva.
• Someone with symptoms of Sjögren’s syndrome may be referred to an ophthalmologist who can examine the tear layer formed on the eyes. The eye is examined using a microscope and eye drops. Someone with Sjögren’s syndrome may display unevenness in their layer of tears.
• A saliva flow test may be performed to determine the sialometry – the measure of saliva flow. The individual must deposit as much saliva as possible into a cup over a certain number of minutes, which is then measured.
• A similar test to the saliva flow test can be performed with tears, called a Schirmer test, or a ‘teardrop test’. Filter paper is placed in the lower eyelid and left there for 5 minutes. When it is removed, its moisture is measured, with the results indicating whether the individual’s eye is producing enough tears.

As the symptoms of Sjögren’s syndrome can overlap with other conditions, the individual may be referred to a specialist to carry out the above tests.

How is Sjögren’s syndrome treated?

Whilst the condition is known to be lifelong, some symptoms of Sjögren’s syndrome can be treated directly.

The following methods may be used:

Dry eyes

People with dry eyes can use eye drops, known as ‘artificial tears’, regularly to moisten their eyes. It is important to speak to a pharmacist about the correct type of eye drop, as some are only suitable for use a few times each day. If eye drops do not help, they can also have a small operation to block their tear ducts with small plugs, so that the tears do not drain, which can help to keep the eyes moist.

Dry mouth

People with Sjögren’s syndrome who are suffering from dry mouth should maintain good oral practices, such as brushing twice daily and using an antibacterial mouthwash. They should drink water to keep their mouth from becoming dry. This can also be helped by chewing gum, or using throat sprays and lozenges.

They should also try to avoid alcohol and any other substance which has a dehydrating effect on the mouth and the body. It is also possible to take pilocarpine, a medicine which is used to help the body create more saliva and tears. Drugs that stimulate the production of fluid in the body are called cholinergic agonists, and take up to two weeks to start working.

Dry skin

Dry skin can be helped by using an emollient cream which rehydrates the skin, and by avoiding perfumed products which tend to dry out the skin.

Vaginal dryness

Oil-based or silicone-based lubricants can be used prior to sexual intercourse, or generally to relieve dryness. There are also vaginal moisturisers available which can create moisture in the vagina. There are also hormone treatments available to increase the levels of oestrogen in the body, which can increase vaginal fluid.

Joint and muscle pain

Joint and muscle pain may be helped through physiotherapy, and further suppressed through the use of strong anti-inflammatory medications, such as naproxen, as well as taking hydroxychloroquine, an anti-malarial drug.

In particularly bad flare-ups, doctors may administer steroids. You can read more about pain management by visiting our knowledge base.

Some people with Sjögren’s syndrome choose to follow an anti-inflammatory diet, eliminating foods that can trigger muscle and joint pain, and inflammatory flare-ups. Trans fats, dairy, gluten and foods high in sugar can make the symptoms of Sjögren’s syndrome worse. Instead, raw foods and healthy fats can be used in a well-balanced diet.

What support is available to people living with Sjögren’s syndrome?

• The British Sjögren’s Syndrome Association (BSSA) is dedicated to raising awareness of the health condition, and has a helpline for information about the syndrome, as well as available support.
• The Wren Project provides programmes of support for people suffering from different types of autoimmune disease, including Sjögren’s syndrome, where people can connect with others suffering from similar conditions, offering up to a year of support.
• The Sjögren’s Foundation offers information for people affected by Sjögren’s syndrome, from the point of diagnosis, and for long-term sufferers.