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What is an Autoimmune Disorder?
An autoimmune disorder is one in which the body's immune system does not function properly. The immune system normally protects the body from foreign matter such as bacteria and viruses. In Sjögren's syndrome the immune cells (lymphocytes) infiltrate and destroy the mucus producing glands (exocrine glands) of the body. The cause of autoimmune diseases is presently not known, but it is thought that several factors are involved. These factors include viral infections, hormones, genetics and stress.
Who gets Sjögren's Syndrome?
Sjögren's syndrome was first described in Europe during the late 1800s and it was named after the Swedish ophthalmologist Henrik Sjogren. Sjögren's syndrome occurs in people of all races and it is a relatively common disorder. More than 90% of people with Sjögren's syndrome are women, with an average age of 50 years at time of diagnosis.
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Symptoms
There are two common presentations of Sjögren's syndrome. One is the rapid development of severe oral (mouth) and ocular (eye) dryness, often accompanied by swelling of the parotid (mumps) glands. The other is an insidious and slowly progressive development of symptoms.
Less common symptoms include involvement of the lungs, gastrointestinal tract, kidneys, skin, nervous system, and lymph glands. People with Sjögren's syndrome may experience mild arthralgias (joint pain) or morning stiffness.
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The Eye
The most frequent eye complaint is the sensation of a foreign body in the eye, best described as a "gritty" or "sandy" feeling. Other symptoms include accumulation of thick ropy strands in the eyes, particularly upon awakening, decreased tears, redness, burning, light sensitivity, eye fatigue, itching and a "filmy" sensation that interferes with vision.
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The Mouth
The symptoms of dry mouth include: adherence of food to surfaces inside the mouth, ulceration of the tongue and buccal (cheek) membranes, difficulty in chewing, swallowing and speaking; burning discomfort of the tongue; altered taste sensation; and the need for frequent ingestion of fluids. Apart from its functions in the previously mentioned symptoms, saliva is important for its antibacterial function - without adequate saliva you may get bacterial overgrowth resulting in dental decay, periodontal disease and Candidiasis.
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Ear and Nose
The ear may develop eustachian tube problems with middle ear fluid. The nose may become dry with crusts, bleeding and diminished sense of smell.
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Vaginal Dryness
This can be quite a distressing symptom and is often overlooked in patients with Sjögren's syndrome. Recurrent vaginal infections and pain on intercourse (dyspareunia) may develop. Explanation and effective lubrication is important to avoid local complications. In menopausal patients who have low estrogen levels the use of vaginal estrogen creams may be helpful. Vaginal lubricants should be used regularly, especially prior to sexual intercourse. A variety of vaginal lubricants are effective including K-Y Jelly, Wet Stuff and Replens, which is a vaginal moisturiser.
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Depression & Fatigue
Depression and fatigue commonly complicate the clinical course of Sjögren's syndrome. Individuals often complain of decreased motivation and lack of enjoyment of life. These symptoms occur in approximately two thirds of patients with Sjögren's syndrome during the course of their disease. This can lead to considerable morbidity. It is important to recognise that this an important clinical feature of Sjögren's syndrome and seek treatment. Patients should be aware that should they develop significant depression that requires drug therapy, then they should warn their treating medical officers that a number of the medications used to treat depression, may also worsen their dryness.
Patients who suffer from Sjögren's syndrome often experience fatigue, either associated with depressive illness or independent of this. This prominent symptom of fatigue has previously been unrecognised by the medical profession, although long recognised by people living with Sjögren's. The cause of fatigue associated with systemic diseases such as Sjögren's syndrome is unknown. There are a number of strategies that may help with fatigue and this will depend on the nature of the disease, whether or not there is an associated autoimmune disease and the type of treatment that the patient is receiving. Individuals with Sjögren's syndrome should be encouraged to consult their treating doctors to discuss the management of this common and difficult problem.
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Diagnosis
The doctor needs to take a good history, seeking out features of dryness. The physical examination is helpful looking at the eyes and the mouth and observing whether they are dry, red and irritated and the skin is dry. A specialist eye examination is valuable and occasionally kidney and lung function tests are needed. Dry mouth and eyes alone may result from aging, medication or a condition known as Amyloidosis.
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Blood Tests
Blood tests provide pointers to Sjögren's syndrome; however, by themselves they do not make the diagnosis. A full blood count may be helpful. Some patients are mildly anaemic, the white cell count may be reduced and the erythrocyte sedimentation rate (ESR) is usually elevated. Measuring your antibody levels may be useful. Commonly patients with Sjögren's syndrome have high levels of serum autoantibodies. Antinuclear antibodies occur not only in lupus, but also in patients with Sjögren's syndrome. There are some more specialised types of antinuclear antibodies- SSA and SSB antibodies. These are typically found in patients with Sjögren's syndrome.
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Other Tests
An eye examination is very helpful and can be performed by your family doctor without needing a specialised ophthalmologist. The Schirmir filter paper test provides a crude measure of tear production and is a useful screening test for people with Sjögren's syndrome. Normal people wet the filter paper very rapidly, but in Sjögren's syndrome even after five minutes the filter paper may be dry. An ophthalmologist may perform the SLIT LAMP EXAMINATION where a special lamp scans the surface of the eye and identifies abnormalities of secretion or of the cornea. Ophthalmologists also perform the Rose Bengal test. This is a dye that is put into the eye that identifies mucous material in a pattern characteristic of Sjögren's syndrome. Some people find it a bit irritating but it is not harmful. A lip biopsy may be taken under local anaesthesia to sample the salivary glands. Examination of the biopsy sample under the microscope shows collections of white cells and damage to the glands in people with Sjögren's syndrome.
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Treatment
Treatment of Sjögren's syndrome is aimed at symptomatic relief, at this stage there is no cure.
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Dry Eyes |
A variety of artificial tear preparations can be used to treat dry eyes. The severity of the dryness will determine how frequently you need to use the drops. Some preparations have preservatives - these last longer but may be irritating to the eye. It is a matter of trying one brand and seeing how you go. Lubricating ointments are longer acting preparations that are useful at night whilst sleeping. These tend to be greasy so are not appropriate for daytime use. |
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Dry Mouth |
Dry mouth is harder to treat than dry eyes. There are some artificial oral lubricant preparations available, but none are entirely satisfactory. Some people find chewing sugarless gum is helpful, whilst others prefer simply to spray water into the mouth. Dietary modification using soft or moist foods may be of some assistance. The use of sugar should be avoided as it contributes to the development of dental caries. Good dental hygiene and frequent dental visits are essential. |
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Dry Skin |
Sorbolene cream and moisturisers may be helpful. For dryness of the vagina, lubricating creams or oestrogen creams may be helpful. |
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Oral Medications |
Oral medications are used in a majority of people. Non-steroidal anti-inflammatory drugs, or the anti-malarial drug Plaquenil, may be used to treat joint symptoms. Prednisone and immunosuppressant drugs are seldom used but are useful where there is severe lung or kidney involvement. |
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Conclusion
Sjögren's syndrome is frequently difficult to diagnose as it often overlaps with other diseases such as lupus, Rheumatoid Arthritis, Scleroderma and Polymyositis. People with Sjögren's syndrome may be more susceptible to drug allergies and care is needed to monitor their condition if medication is required. Drugs such as antihistamines and antidepressants can cause mucosal dryness and should only be taken in consultation with your doctor. It is important to remember that air-conditioning dries you out, and dust and smoke are irritating - avoid these wherever possible. Like all chronic diseases it is important to have regular contact with your doctor and eye specialist to monitor your condition. Regular dental check ups are essential. Although Sjögren's syndrome is not life threatening, careful attention to the problems it causes can help minimise the "nuisance" aspect of this condition and assist in a more relaxed way of living.
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