Authored by Dr Jan Sambrook, Reviewed by Dr Adrian Bonsall on 3 October 2016 | Certified by The Information Standard
Sjögren's syndrome can cause various symptoms. The most common ones are dry eyes and dry mouth. These symptoms are due to lack of secretions from glands in the body. In severe cases the lungs, kidneys, nervous system and lymph glands can be affected. Treatment is mainly directed at symptom control.
Sjögren's syndrome most commonly causes dry eyes and mouth. It can also affect other organs including lungs, kidneys, skin and the nervous system. It was first described in 1933 by a Swedish eye specialist (ophthalmologist) 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 germs (bacteria or viruses) causing the infection. In autoimmune diseases the body makes similar antibodies (autoantibodies) that attack its own cells. The cause of this is uncertain but is thought to be a combination of genes and factors in the environment such as infection.
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.
Primary Sjögren's syndrome: this describes Sjögren's syndrome which occurs by itself. About one in a thousand people have this.
Secondary Sjögren's syndrome: this describes Sjögren's syndrome which occurs in association with another autoimmune disease such as rheumatoid arthritis or systemic lupus erythematosus.
It is thought there are about half a million people in the UK with Sjögren's syndrome, although not all of them seek medical help for their symptoms. Nine out of ten people who have the disease are women. People usually first start noticing symptoms when they are in their 30s or 40s.
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.
Swallowing problems and the feeling of something getting stuck in the throat on swallowing (dysphagia).
Tooth decay and gum disease (gingivitis). Saliva contains anti-infective agents, so when saliva production is reduced, infection in the mouth is more likely.
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 salivary glands, including the parotid glands (located in both cheek areas, just in front of the ears) and those under the jaw and in the neck area.
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, characterised by severe pain in the upper part of the stomach (abdomen).
Kidney problems: Sjögren's syndrome can progress to affect the kidneys. It can cause kidney inflammation, disruption in body fluid balance, kidney stones and, if untreated, loss of kidney function.
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 some people who develop pseudolymphoma, the pseudolymphoma can progress to a lymphoma, usually non-Hodgkin's lymphoma. It used to be thought this occurred in about one in ten people with pseudolymphoma but more recent research indicates it may be a lot less common. This is a type of cancer of the lymph glands. It tends to progress very slowly and in some cases may even 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.
Recurrent miscarriage: Sjögren's syndrome can cause recurrent miscarriage. Recurrent miscarriage is the term used 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.
Medication reactions: people with Sjögren's syndrome may be more prone to developing side-effects when they take certain medicines - for example, antibiotic medicines.
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.
The Schirmer tear test: this measures the amount of tears that you form. A piece of 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 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:
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. Joint specialists (rheumatologists) 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.
Prolonged reading or staring at a computer screen or television. This makes us blink less often so our eyes don't stay as moist.
Artificial tears: these provide lubrication and come in the form of eye drops. Preservative-free eye drops such as hypromellose help to reduce the risk of eye irritation. Paraffin eye ointments are helpful for use at night 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 procedure called diathermy is used to close up the tear ducts.
Keeping your teeth, gums and mouth as clean and healthy as possible. Brush your teeth regularly, use dental floss and a mouth wash.
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.
If Sjögren's syndrome progresses to involve organs such as the skin, lungs, kidneys and lymph glands, you may need to take some other 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 medicines 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. You may be prescribed one of these medicines if your kidneys or lungs are affected, or if you develop pseudolymphoma.
Sjögren's syndrome is not usually life-threatening. Some people may only notice mild symptoms such as mildly dry eyes and a mildly 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). 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, a small number of 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.
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.
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Hello, I am a 30ish female and I've been experiencing symptoms of what I believe to be Sjogren's since I was in my early teens. I first went to a doctor for chronic joint pain when I was around 17...
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