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Sjögren’s Syndrome, named after Dr Henrik Sjögren a Swedish ophthalmologist, is a chronic autoimmune condition which destroys the lacrimal and salivary glands, which produce tears and saliva.
The damage is done when the body’s own white blood cells attack the glands, and results in conditions known as ‘dry mouth’ and ‘dry eyes’ (xerostomia and keratoconjunctivitis respectively).
Although the condition can be classified as either primary or secondary (depending on the absence or presence of any other connective tissue disease) this has no bearing on the particular symptoms a patient will experience, nor on the severity of those symptoms.
Signs and Symptoms
The single most significant symptom of Sjögren’s Syndrome is generalised dryness – particularly of the eyes and mouth, although this may extent to the skin, nose and other organs of the body, including kidneys, lungs, liver, and even the nervous system.
It is possible for Sjögren’s Syndrome to develop over a number of years without being diagnosed, as these common symptoms are often attributed to a dry environment or reaction to food or medication – or simply as a side-effect of the aging process.
Some patients will develop only mild symptoms, whilst others may experience more serious manifestations of the disease. As with other autoimmune diseases, Sjogren’s Syndrome can go into remission spontaneously or may require treatment.
Causes of Sjögren’s Syndrome
Like many autoimmune conditions, the exact causes of Sjogren’s Syndrome are not yet fully understood. However, existing research suggests that it is brought on by a combination of genetic, environmental and other factors.
Genetic Factors
Families of those suffering from Sjögren’s Syndrome have been seen to present a higher than average occurrence of other autoimmune diseases which leads experts to believe that there is a genetic factor involved.
Hormonal Factors
Sjögren’s Syndrome is more commonly observed in female patients, suggesting that there is a hormonal element in its causation. Studies have suggested that estrogen-deficiency can be one contributing factor.
Other external factors, such as glandular viral infection, may also be linked with the onset of Sjogren’s Syndrome, although research is continuing in this area.
Diagnosis
Diagnosing Sjögren’s Syndrome can be complicated by a number of factors. In particular, as the condition generally presents in those aged over 40, the early symptoms of dry eyes and mouth are frequently dismissed as part of the aging process.
Because other symptoms can affect different areas of the body, many patients initially seek with their symptoms help from different specialties, making diagnosis difficult.
In addition, many of the symptoms are also associated with other autoimmune diseases, further complicating the diagnosis process.
There are a number of tests that can be administered to diagnose Sjögren’s Syndrome. The ‘rose bengal’ test measures the function of the lacrimal (tear) glands by placing a non-toxic dye on the eye. The Schirmer test uses a strip of filter paper placed under the lower eyelid to collect tears – its wetness is then measured to determine whether production of tears falls into the diagnosable range.
Similarly, the function of the saliva glands can be measured in a test which involves the patient being asked to spit as much as possible into a cup; the volume is then measured to determine whether the glands are functioning normally or not.
Treatments
Currently there is no cure, nor is there any specific treatment that will restore normal function of the saliva or tear glands.
Consequently most treatment associated with Sjörgen’s Syndrome is designed to alleviate the symptoms are reduce discomfort.
Treatments to increase or replace lost moisture, such as artificial tears, can be prescribed to reduce discomfort around the eyes. In more severe cases, goggles designed to increase ‘local humidity’ around the eyes can be worn.
Cyclosporine may be prescribed to reduce the inflammation which inhibits tear production, and other drugs may be used to stimulate salivary flow.
For musculoskeletal symptoms it is possible to prescribe non-steroidal anti-inflammatory drugs and for patients with severe symptoms or related complications corticosteroids and other immunosuppressive drugs are sometimes prescribed.
Immunosuppressants are also used to treat systemic symptoms associated with Sjögren’s Syndrome, such as chronic fatigue and joint pain.
Often overlooked by patients is the need for preventive dental care. The lack of saliva in the mouth can have severe consequences for both teeth and gums, and xerostomia creates a breeding ground for bacteria which can lead to painful cavities. Sadly, many patients leave this care too late and it is not uncommon for dentists to see patients with severe and untreatable cavities.
Prognosis
Although studies in this area are still rather limited, patients with Sjögren’s typically experience only a slightly below average life expectancy.
However, Sjogren’s Syndromeit is often associated with other diseases which may impact on mortality rates, in particular a higher instance of non-Hodgkin lymphoma.
Facts and Figures
● 3rd most common rheumatic autoimmune disorder
● 500,000 to 2 million patients in the USA
● May affect up to 3% of the population globally
● 90% of patients are female
● Average onset between 40-60 years of age
● Occurs in 30-40% of patients with rheumatoid arthritis and 10-20% of patients with systemic lupus erythematosus.
Conclusion
Whilst Sjögren’s Syndrome is categorised as an ‘incurable’ condition, its symptoms can largely be managed through a combination of medication and lifestyle changes. As with other conditions which can result in chronic fatigue, depression is a risk and patients should make friends and family aware of their condition in order to ensure a solid support network. Although the symptoms of Sjögrens can cause complications, barring more serious secondary illnesses patients can expect to live a normal lifespan.
