Sjögren’s Syndrome is an auto-immune disease. This is one of many illnesses that develop and progress because the immune system recognizes parts of the body as foreign, the same way it would recognize an invading germ or virus. The immune system attacks these body parts and causes them to become swollen and hot (inflamed). Eventually, those parts of the body become damaged irreversibly and cease to work properly. In Sjögren’s Syndrome, the glands that make tears and saliva are primarily affected. The salivary glands become painful and swollen in some. Eventually these glands fail and cannot produce saliva or tears. This is called Primary Sjögren’s Syndrome, because it is the main illness.
Sometimes Sjögren’s Syndrome is not the primary illness. Instead, it is a part of another illness. Sjögren’s Syndrome can accompany Systemic Lupus Erythematosus, Scleroderma, Rheumatoid Arthritis (known as Connective Tissue Diseases, because they are auto-immune illnesses that attack blood vessels, joints, and other tissues that connect our body together). In this case, we label the condition as “Secondary Sjögren’s Syndrome”.
Some people have dryness of the eyes or mouth, but there is no auto-immune process underlying this. Dryness of this sort can result from age, drug side-effects, radiation, dehydration, anxiety, or for no clear reason.
In general, auto-immune diseases affect more women than men. This is particularly true of Connective Tissue Diseases including Primary Sjögren’s Syndrome. Sjögren’s affects 9 out of 10 women. Furthermore, these are diseases that affect women in the younger years of their lives, with peak incidence between 30 and fifty years. Primary Sjögren’s syndrome can, however, affect children, as well as the elderly. Primary Sjögren’s Syndrome and Secondary Sjögren’s Syndrome together, affect about one in every 100 people. Dry Eye or Dry Mouth, however, is much more frequent, with an incidence of about one in 50 people.
The cause of Primary Sjögren’s Syndrome is unknown. The theory is that in certain people, there is a genetic predisposition that causes them to react abnormally to some environmental factor, such a virus. Whatever triggers Sjögren’s syndrome, usually cannot be identified by the time the Disease is diagnosed. Certain viruses, such as Mono, Epstein Barr, HIV and Mumps are known to be capable of attacking the parotid (major salivary) gland. There are probably many other unidentified infective agents capable of getting the process going. Perhaps because the infecting agent persists in a hidden form, or perhaps because it changes the proteins in the gland making them foreign to the immune system, the auto-immune attack is difficult to turn off once it gets going.
The main symptoms are dry eyes and mouth. The eyes feel as if they have gravel, or a foreign body stuck in them. The bright light is very uncomfortable. The eyelids stick together in the morning, and there is frequent crusting and debris in the eyelashes. The mouth is constantly dry. The tongue clicks when speaking, the lips stick to the teeth. The teeth begin to deteriorate, and it becomes difficult if not impossible to swallow dry food without liquid. Fissures form at the angles of the mouth, and the tongue becomes painful. Some people experience swelling of their parotid glands (located at the angles of the jaw), giving an appearance like mumps. Sometimes this swelling is recurrent, sometimes it is quite painful, and sometimes the swelling persists.
What other ways can Sjögren’s Syndrome affect the body?
Primary Sjögren’s Syndrome is associated with tremendous fatigue. Other parts of the body can be affected as well. For example, the lungs can become inflamed, causing a chronic type of pneumonia, and eventual scarring, the nerves can become impaired, with persisting numbness of the hands and feet, the blood vessels in the skin can become inflamed, leading to crops of red dots on the legs. There can be joint pain, and dryness of the skin and vaginal tract. A low grade lymph node cancer, known as “non-Hodgkin’s B-Cell Lymphoma” can eventually develop in up to 10% of patients with Sjögren’s Syndrome, a frequency that has been estimated at 44 times the general population.
What connective tissue diseases are common with Sjögren’s Syndrome?
Sjögren’s Syndrome can accompany Systemic Lupus Erythematosus, Scleroderma or Rheumatoid Arthritis. Sjögren’s can also be associated with “Primary Biliary Cirrhosis” or “Autoimmune Hepatitis,” two rare forms of liver inflammation. Sjögren’s can be seen with a limited form of scleroderma, known as the “CREST Syndrome.” This condition is associated with calcium deposits in the skin, blanching of the fingers in the cold (Raynaud’s Phenomenon), hiatus hernia symptoms (dysfunction of the esophagus), scarring of the skin on the fingers (sclerodactyly) and marks on the skin due to enlarged clumps of small blood vessels (telengiectases). Rarely, Sjögren’s Syndrome can also be associated with increasing blood pressure on the right side of the heart (pulmonary hypertension) resulting in shortness of breath.
A medical history is taken by your doctor. The doctor will ask you detailed questions about your health, fatigue level, symptoms you are experiencing and conduct a thorough physical examination. Once Sjögren’s syndrome is suspected, you may have a series of tests conducted by different professionals, including:
Tests for Assessing the Presence of Dry Eyes
Ophthalmologic Tests: A careful history is taken about how your eyes feel (gritty, burning, dry, etc.) and for how long they have felt that way. This may be done verbally or with a questionnaire.
The Schirmer test estimates the flow of your tears. It tells us how much fluid your lacrimal gland is able to produce. This test is done by putting thin strips of filter paper on your lower lid and asking you to sit with your eyes closed for 5 minutes. The amount of the tears that you secrete on to the paper in this period of time is measured and if it is less than or equal to 5mm, we know that you have a dry eye.
Rose Bengal/Lissamine Green Stains and Fluoresceine:
Dyes are used to observe abnormal cells on the surface of the eye, a consequence of the dryness of the eye. The staining tests allow us to estimate how many dry spots you have on the surface of your eyes. We are concerned specifically with the area of the eye surface that is exposed when your eyes are open. A yellow dye called fluoresceine is put into the tears and with the use of a bio-microscope or slit lamp (an instrument in which you rest your head while we view a magnified image of the surface of your eye) and a blue light, we grade the amount of staining that is found on the cornea or clear part of the eye. Then a green dye (lissamine green) or a red dye (rose bengal) is instilled into the tears and we look with a white light at the number of cells in the conjunctiva (the mucus membrane that covers the white of the eye) that absorb this dye and estimate the degree of “staining” or the amount of dryness.
Opthalmologic tests indicate dry eye when you have symptoms of dryness for at 3 least months AND if your tear flow is low OR you have significant staining on the surface of your eyes.
Tests for Assessing the Presence of Dry Mouth
The Salivary Gland Tests include:
Parotid Gland Flow:
Measures the amount of saliva produced over a certain period of time. The most accurate way to do this is to measure unstimulated saliva, which requires the patient to be fasting and to be in an unstimulating environment ie a quiet dark room. Patients are considered to have a dry mouth if they produce less than 1.5 ml of saliva over 15 minutes. The measurement of stimulated saliva is considered inferior because it can vary so much depending on a variety of factors, including time of day and time to last meal taken.
Measures the salivary gland function. Poorly functioning glands will demonstrate slowed and reduced uptake of radio isotope with delayed glandular secretion after exposure to a stimulus of something like lemon juice.
Is an X-ray of the salivary glands that can demonstrate abnormalities of the glandular ductal system and the architecture of the glands.
You likely have Sjögren’s syndrome if you have symptoms of dryness for at least 3 months, or recurrent swollen salivary glands AND you have objective evidence demonstrating dry eyes and /or dry mouth (see above).
ANA (Anti-Nuclear Antibody): About 70% of Sjögren’s patients have elevated antibodies that react against normal components of a cell’s nucleus.
SS-A (or Ro) and SS-B (or La) Antibodies to SS-A (or Ro) and SS-B (or La) are antinuclear antibodies that are found in patients with Sjögren’s Syndrome but they are also found in patients with Systemic Lupus Erythematosus (SLE). Seventy percent of Sjögren’s Patients may have Ro (SS-A) antibodies and 40% may have antibodies to La (SS-B). The presence of either of these two antibodies is now specified as a criterion for the diagnosis of definite Sjögren’s Syndrome.
RF (Rheumatoid Factor): 60% – 70% of patients have a positive RF.
ESR (Erythrocyte Sedimentation Rate): Measures inflammation. An elevated ESR can indicate an inflammatory disorder, including Sjögren’s Syndrome.
IGs (Immunoglobulins) are normal blood proteins, sometimes elevated in Sjögren’s.
A lip biopsy provides tissue that will help to confirm the diagnosis of Sjögren’s Syndrome. The lip biopsy is a fairly simple procedure performed using local anesthesia.
An ear nose and throat physician or a dentist will take a biopsy of the small salivary glands of your lower lip. This requires a needle that freezes the lip and the removal of a small amount of tissue. The tissue is then observed through a microscope by a specialist, who determines the presence or absence of inflammation (lymphocytic infiltration) in the glands. The pathological changes found in Sjögren’s Syndrome include a FOCAL infiltrate of lymphocytes that may eventually result in the replacement of glandular tissue. This glandular pathology is quite specific, but a few other diagnoses, including several chronic viral diseases, can produce a similar glandular pathology. The most recent Sjögren’s Syndrome criteria (The American European Criteria) require that these viral syndromes be excluded in order for the patient to be given the diagnosis of Sjögren’s Syndrome.
Talk to your health care provider if you have had dry eyes and/or dry mouth for more than three months and other commonly associated Sjögren’s-type symptoms. Fatigue and joint pain are common complaints of Sjögren’s because this is a systemic inflammatory disease.
No. It can often be difficult to diagnose due to the complexities of the disease and the diversity of symptoms. All symptoms may not be present at the same time. It has been shown that it can take up to 5-9 years to be diagnosed even if patients present with the typical dryness complaints of this syndrome. Depending on the presenting symptoms, an undiagnosed patient may seek treatment from a variety of different specialists who will treat each symptom individually.
Sjögren’s Syndrome may present with various complaints that can mimic other diseases such as rheumatoid arthritis, Systemic Lupus Erythematosus, multiple sclerosis or fibromyalgia, further complicating diagnosis. One of the difficulties with diagnosing Sjögren’s syndrome is that patients frequently have no obvious clinical findings and, unless the physician is particularly astute, they will not be diagnosed appropriately.
People may appear to be quite “normal” on the outside. The invisibility of the disease may add to the delay in diagnosis or not to be taken seriously by professionals. The general lack of awareness about this complicated syndrome may also be a factor in the delay of diagnosis. This syndrome occurs much more frequently than is generally appreciated and contrary to current opinion the quality of life for these patients can be improved by treatments designed to: promote saliva production, reduce inflammation in the eye and dryness of the eye, improve fatigue, and minimize inflammation in joints.
What type of doctor diagnoses and treats Sjögren’s Syndrome?
Many different types of doctors can diagnose Sjögren’s Syndrome. This is because although the criteria for Sjögren’s Syndrome specify subjective and objective features of dry eyes and dry mouth, Sjögren’s Syndrome is in fact a systemic disease and patients may present with a variety of different clinical complaints. Sjögren’s Syndrome may also be diagnosed by astute dentists, as rampant dental caries are a feature of this syndrome.
Rheumatologists usually have primary responsibility for managing and monitoring Sjögren’s patients but a team of specialists, including ophthalmologists, dentists, and ear nose and throat doctors are vital contributors for the over all management of Sjögren’s Syndrome patients.
There are several over-the-counter products that can provide symptomatic relief for various aspects of the disease. There are a few prescription medications that may be helpful in treating dry eye, dry mouth and other symptoms depending on the type and severity of these problems.
It is important to seek expert eye and dental care. There are strategies and products that can to help manage or relieve symptoms such as the use of electric toothbrushes, humidifiers, moisture-chamber glasses or goggles.
Often people are better able to cope with a chronic condition like Sjögren’s, when they educate themselves and connect and learn from each other in support groups.
The most important thing that you can do about your dry eyes is to take the condition seriously but remember that you will not become blind because of your dry eye disease. Yes, there are a very few Sjögren’s syndrome patients who have serious corneal infections that are sight threatening. However, the vast majority of patients manage their dry eyes very well.
In managing your dry eye disease think from the inside out. The goal is to make your body as healthy as possible in order to allow the tear secreting glands to function to their maximum and to maintain the surface of the eye in its best form. This starts with a healthy diet of fresh food with lots of fruits and vegetables and plenty of water intake. Eating cold water fish at least 3 times per week has been shown to reduce the inflammation in the body and reduce symptoms of dryness on the eye. The Greek diet of fish, olive oil and tomatoes has been found to be successful in reducing symptoms of joint pain in other autoimmune diseases like rheumatoid arthritis and is likely to help with Sjögren’s syndrome. Get some exercise to help your blood flow to oxygenate the tear glands. Do not smoke.
Moving to the outside of the body, remember to keep the ocular surface as healthy as you can by using good lid hygiene. This means cleaning the lids daily and using hot compresses at least once per day. The lid glands that supply the oil for the tear film, can remain open and functioning if you attend to them regularly with this regime.
Topical eye drops do not cure your dry eyes but do lubricate the surfaces and help to maintain the cells in a healthier fashion. There are so many drops that it is often difficult to find the “best” one. In fact the best one is the one that feels best on your eyes and lasts the longest. Your eye care professional can help you with this choice. If you are using drops more than 4 times a day it is usually best to use a non-preserved drop as the preservatives in the drop may cause harm to the ocular surface. Non-preserved drops usually come in individual unit dose form.
There are thicker gels that can be used at night to coat the eye and prevent the lids from sticking to the cells on the surface of your eye. They should be used just before sleeping as they will blur your vision considerably.
There are prescription medications that are sometimes used for more severe dry eyes. These include topical steroid drops and other anti-inflammatory medications. Restasis is the name of the anti-inflammatory drop that is not approved here in Canada but has been used with some success in the United States with Sjögren’s syndrome patients. We are hoping to have approval in Canada soon.
Some patients will benefit from punctal occlusion. This technique of closing the small holes in the lid that allow the tears to run off the ocular surface can help to maintain the tears that you do secrete and the drops that you instill on your eye longer. This is usually done with a tiny silicone plug that is inserted by your eye doctor. With very dry eyes however, they can fall out.
Next, think about your environment. Humidify your home as much as you can. Put a humidifier in your office and keep it running all the time. When you are in the car keep the air vents pointed to your feet. Wear protective glasses as much as possible and consider wrap around specialized ones or custom made humidity shields to prevent air currents and pollution from reaching the surface of your eyes.
Make sure that you see your eye care professional regularly and that he or she has a special interest in dry eye disease. Dry eyes have there ups and downs and it is important that they be looked at routinely. Also your doctor can keep you informed of the latest drops and management techniques for dry eyes.
Again, remember that dry eye is a serious disease that is chronic. There are no cures but you can maintain the health of your ocular surfaces and good vision throughout your life by following these guidelines and seeing your eye care practitioner regularly.
Dry mouth and Sjögren’s syndrome can be a difficult and expensive condition to control. However there are many strategies that may help to prevent and manage the oral symptoms.
Extra care and particular attention to good oral hygiene are essential including frequent dental checkups for dental cleanings, tooth maintenance and fluoride application.
It is important to keep your mouth hydrated by sipping water or a sugar-free drink. Drinking large volumes of liquids are often not helpful and wash down the mucus in your mouth and, in the end, make your mouth dryer. Limit your sugar consumption and avoid drinking carbonated beverages.
Stimulate your natural saliva by chewing sugar-free gum or sucking a sugar-free candy. There are mouthwashes, mouth sprays and gels that may provide temporary relief of dry mouth symptoms but generally are not long lasting. Your doctor may prescribe a medication to stimulate your saliva flow.
With dryness of the mouth resulting from Sjögren’s Syndrome, candida infections can begin to grow superficially in many of the tissues, including the top of the tongue, the roof of the mouth, the soft palate, the cheeks and at the corners of the lips.
Once candida or yeast has established itself in the mouth, the mouth often feels drier with a burning sensation. Individuals often complain of loss of taste when the candida is on the top of the tongue, and are unable to eat certain foods, including spicy or citrus foods, because of the increase in pain.
Individuals with less intense dry mouth can develop infections but this is commonly after a long course of antibiotics, or as a result of using corticosteroids, often from puffers as needed for asthma, or as a result of using immunosuppressants for other diseases or disorders. In this case, the yeast infection often looks different and is often seen as small white colonies scattered around the mouth and usually localized to the soft palate, around the tonsillar area and on the sides of the tongue. This is in contrast to the more generalized and widespread yeast infection seen with dry mouth resulting from Sjögren’s, when colonies of yeast are often not seen, but instead, there is usually evidence of redness and pain.
It is essential to control yeast infections. Although candidiasis in Sjögren’s syndrome is a nuisance, the good news is that it can be controlled with a course of systemic anti-fungals, including Fluconazole or Ketoconazole, which will usually quickly eradicate the infection and bring the tissues back to normal. Oral anti-fungals (i.e. Nystatin) often are not effective and should be avoided due to their high sugar content. Although the mouth will continue to be dry, the result will be a decrease in burning pain and taste loss.
Sjögren’s syndrome is a serious disease but is generally not fatal if complications are diagnosed and treated early. As reported in one study, non-Hodgkins lymphoma (lymph node cancer) occurred at a rate 44 times greater in Sjögren’s patients as compared to the general population. Lymphoma may occur in up to 10% of patients with Sjögren’s syndrome, but it is generally a low grade tumour and easily treated with the new drugs we now have available. It is important for Sjögren’s patients to be monitored closely for the possible complications, development of related autoimmune phenomena and lymphoma.
This is because there are many factors that contribute to the development of a disease, these include, genetic background of the patient, environmental exposure of the patient, sex of the patient and other factors that may influence the immune system. We do not yet understand how all of these factors contribute to the development of a disease, but great strides have been made in recent years that will ultimately allow us to manage Sjögren’s Syndrome and many other connective tissue diseases more appropriately with more specific and less toxic therapies.
What research is being done on Sjögren’s Syndrome?
Much of the research being done is attempting to understand the roles of the genetic background, environmental triggers and the influence of sex hormones on the expression of this disease. It is important to note that certain chronic viral infections can produce glandular pathology that is very similar to that seen in Sjögren’s syndrome and the role of viruses in the pathogenesis of other connective tissue diseases is also stimulating a lot of interest.
Trying to predict which patients will develop the more serious complications and the development of more specific tests that will make it easier to identify patients that are destined to develop Sjögren’s Syndrome, hopefully will promote the development of preventative measures.
The last few years have seen an absolute explosion of new biologic agents that can alter the course of some of the chronic rheumatic diseases. Unfortunately, to date, these agents have not been shown to be of benefit for patients with Sjögren’s Syndrome, but many new and some more specific therapeutic agents are in various stages of development. Some of these will come to the market in the next few years giving the physician better therapeutic choices for treating Sjögren’s Syndrome.
Thank you to the Sjögren’s Society of Canada’s Medical Advisory Board for graciously contributing to the website information and continued support. We would like to extend a special thank you to our co-chairs, Dr. Ann Parke, MD and Dr. Arthur Bookman MD, FRCPC for their submissions.