Sjögren’s syndrome (SS) is a chronic (long-term) disease that affects the body’s moisture-producing glands and causes a reduction in a variety of body fluids. The most common glands that are affected are the tear glands and salivary glands. People with SS often have dry eyes that can feel gritty and suffer from dry mouth. The dryness usually gets worse as the day goes on. Other glands and organs can also be affected, potentially causing a wide variety of additional symptoms including joint pain, skin rashes, and numbness and tingling in the feet.
SS is an autoimmune disease, meaning that it occurs when the body’s immune system attacks its own healthy cells and tissues. The reason it does this is not well understood. When the body’s immune system is “activated” in this way, it can make a person feel very tired, similar to when they have the flu.
SS is relatively rare (0.5%, or 1 in 200 people, will get it) and it affects women about ten times more frequently than men. Most people who get it notice their first symptoms between the ages of 30 and 50.
Sjögren’s syndrome can be tricky to diagnose because of its range of symptoms and their similarity to other diseases. It is best diagnosed by a rheumatologist or another specialist who knows a lot about SS.
In Sjögren’s syndrome (SS) the body’s immune system primarily attacks its glands, small organs that are responsible for producing a variety of body fluids. SS can also sometimes attack other tissues and organs.
People with Sjögren’s syndrome often have dry eyes that feel gritty because the most common glands that are affected by the disease are the tear glands and salivary glands. The dryness usually gets worse as the day goes on and can require the frequent use of eye drops.
Another common symptom of Sjögren’s syndrome is a dry mouth. This can make swallowing difficult and can also cause dental problems like cavities.
SS can also affect other glands including those in the skin, airways, esophagus (the tube that connects your mouth to your stomach), and the vagina. These tissues can become dry and uncomfortable.
SS can also cause the glands to become swollen. This is most common in glands around the head and neck area.
Fatigue is a very common problem for people with Sjögren’s syndrome because the immune system is activated (“turned on”) all of the time.
The good news is that most cases of Sjögren’s syndrome are mild and symptoms can be limited to dry eyes, dry mouth, achy joints, and fatigue. That being said, there are cases where the condition can be more severe.
Some people with Sjögren’s syndrome also have arthritis. This means the joints are attacked by the immune system, causing pain, swelling, and stiffness. Painful symptoms or fibromyalgia can also affect people with Sjögren’s syndrome.
Other less common symptoms that people with Sjögren’s syndrome sometimes have include:
- Raynaud’s Phenomenon, which causes the ends of the fingers and toes to turn white or blue in the cold
- Vasculitis, or inflammation of the small blood vessel – this can look like a rash with lots of little red dots on the skin, usually on the lower legs
- Neuropathy or painful nerves – this happens when blood vessels supplying the nerves are affected. It can cause a tingling or burning feeling in the feet or sometimes the hands
- Pulmonary fibrosis – a condition affecting the lungs, making it harder to breathe; this is very rare
- Kidney problems are also very rare
- Hypothyroidism – low levels of thyroid hormone
Although it is very rare, people with Sjögren’s syndrome are at higher risk for a type of cancer called lymphoma.
Sjögren’s syndrome (SS) can be properly diagnosed by a rheumatologist, a type of doctor that specializes in arthritis and autoimmune disease, or another specialist who knows a lot about the disease. Sjögren’s syndrome can be tricky to diagnose and having someone experienced is very useful.
To diagnose SS, physicians will take a careful and thorough history. They will ask many questions because Sjögren’s can have so many symptoms.
Next, they will perform a complete physical examination including the head and neck, looking for dry eyes and mouth; as well as the lungs, heart, abdomen, joints, and skin. The exam may include the Schirmer’s test that measures the dryness of eyes. The physician may also recommend a biopsy of a salivary gland in the mouth that produces saliva (spit). A biopsy involves taking a very small piece of tissue so that it can be examined.
Finally, special blood tests are ordered to see the patient’s body is making antibodies to itself, to confirm the diagnosis.
Although it is very rare, people with SS are at a higher risk for a type of cancer called lymphoma. People diagnosed with SS should be screened and monitored for this type of cancer.
Looking for dry eyes – The Schirmer’s test measures the ability of the eye to make tears. People with SS are expected to have very dry eyes because their tear-producing glands are being attacked by their immune system.
Biopsy (tissue sample) looking for signs of SS in the salivary gland: A very small tissue sample is taken from the patient so it can be examined to look for white blood cells (lymphocytes) that are infiltrating the glandular tissue.
Looking for antibodies that attack the body – the anti-nuclear antibody (ANA) test is the most important blood test to do first if Sjögren’s is suspected is the test. This test helps determine if the body is making too many antibodies to itself (having a small amount is normal) and checks if they might be attacking unusual places.
If this test comes back negative in a patient, it is unlikely that they have SS. If it comes back positive, then more tests should be run to confirm the disease because a positive ANA test is possible in healthy people and in people with other diseases.
Looking for antibodies that attack certain proteins: the Extractable Nuclear Antigen (ENA) Panel is a test that measures antibodies to 6 or 7 other proteins in the body. The antibodies in this panel may include: anti-Ro (also called anti-SSA), anti-La (also called anti-SSB), anti-Sm, anti-RNP, anti-Jo-1, anti-Scl70, and anti-centromere. Things get a bit more tricky here and it’s best for patients to discuss this test and its results with a rheumatologist. In general, anti-Ro and anti-La antibodies can be found in people with Sjogren’s Syndrome.
Rheumatoid Factor: The rheumatoid factor can be found to be positive in some patients with Sjogren’s Syndrome.
Looking for excessive B-lymphocytes: an immunoglobulin (antibody) test measures the total amount of all immunoglobulins (antibodies) a person has. Since people with Sjögren’s have too many of a type of white blood cell called the B-lymphocyte (B-cell), and these cells are responsible for making antibodies, this test is expected to show a high level of them.
Looking for inflammation: Sjoogren’s Syndrome causes inflammation in the body so tests for inflammation are expected to have abnormal results. Common tests include a Complete Blood Count (CBC), Erythrocyte Sedimentation Rate (ESR), and C-Reactive Protein (CRP).
Looking at kidney function: Creatinine is a common blood test that looks at how well the kidneys are working. Abnormally high levels of creatinine can indicate a problem and can mean that the kidneys are involved.
Looking for kidney involvement: urinalysis testing to detect protein or blood in the urine can give a sign that the kidneys might be involved.
Looking for lung involvement: Chest x-ray and/or CT scans can help confirm if the lungs are involved.
Looking for nerve involvement: EMG or Nerve Conduction Studies can be performed to look for nerve involvement.
Sjögren’s syndrome occurs when the body’s immune system begins to attack its own moisture-producing glands.
It’s not known why the immune system gets turned on in people with Sjögren’s syndrome. It is known that the immune system makes too much of a white blood cell called the B lymphocyte (or “B-cell”). These B lymphocytes make lots antibodies that can play a role in causing the symptoms of Sjögren’s syndrome.
Treatment of Sjögren’s syndrome (SS) often involves a combination of good hygiene and medications. As SS can be a variable disease, each patient’s treatment plan is personalized to suit their needs.
Fatigue is one of the most common symptoms in people with SS, and one most difficult to treat. Patients are encouraged to learn how to balance the demands of their life with their need to rest. Medications such as prednisone can be helpful with fatigue but also have side effects.
Eye Hygiene, Eye and Eyelid Care
People with SS should keep their eyes clean by washing them every day with warm water. This is especially important after waking up because debris can build up in and around the eyes during sleep.
A good pair of glasses or sunglasses that stops air from blowing across the eyes helps prevent them from becoming too dry.
Artificial tears during the day and/or a jelly lubricant at night can help keep the eyes moist.
Oral Hygiene, Care for Dry Mouth
People with SS often don’t make enough saliva. They are prone to cavities and other dental problems because saliva helps rinse food and bacteria away from the teeth.
It is important to brush teeth regularly with a fluorinated toothpaste, and use an antimicrobial mouthwash.
When brushing, it is important that toothpaste is in contact with the tooth for at least 2 minutes. A good electric toothbrush with a timer (such as the Sonicare brand) can be helpful.
Going to the dentist on a regular basis is an important part of treatment.
Dry mouth can be relieved by chewing sugar-free gum, sucking on sugarless mints, using a lubricant spray, and/or drinking plenty of fluids like plain water.
Tear Duct Surgery
In some cases, an eye surgeon (ophthalmologist) may perform a simple procedure that blocks the tear ducts (punctual occlusion). This prevents the eyes from draining tears that are formed by the tear glands, and helps keep the eyes moist.
Medications for Sjögren’s Syndrome
The choice of medications depend on a patient’s specific symptoms. Though they cannot cure the disease, they can make living with the condition much more comfortable.
Pilocarpine for Dry Eyes and Dry Mouth
A medication called pilocarpine (Salagen) encourages the glands to produce more fluids. This may be helpful if you have dry eyes and/or a dry mouth. Pilocarpine comes as a liquid solution (eye drops) or a gel that can be applied to the eyes, and as an oral tablet to increase saliva production. Since pilocarpine increases the body’s production of fluids, it can cause excessive sweat production. This can be a bothersome side effect for some people.
Pilocarpine has been around a long time. It has been used for the treatment of an eye condition called glaucoma for over 100 years.
Chronic pain can be treated with a variety of medications.
Analgesic medications only control pain. They do nothing to control the disease or to prevent further damage to the glands. Analgesics can range from simple things like acetaminophen (Tylenol) to more potent narcotics like morphine.
Medications like prednisone can help control inflammation. Prednisone is a very effective medication to control many of the symptoms of Sjögren’s syndrome including fatigue, arthritis, and vasculitis. High doses are typically used when any organs are involved.
In the short-term, prednisone works very well to control symptoms caused by inflammation. When used for long periods of time, prednisone can have side effects. Patients should to discuss the risks and benefits of using prednisone with their rheumatologist. Some patients with joint involvement can benefit from cortisone injections directly into the affected joints.
Medications for Arthritis or Vasculitis Symptoms
Swollen and inflamed joints may be treated with medications typically used for people with rheumatoid arthritis. These include prednisone, steroid injections, and disease-modifying anti-rheumatic drugs (DMARDs) like methotrexate, sulfasalazine, Plaquenil (hydroxychloroquine), Gold (myochrisine), Imuran (azathioprine), Arava (leflunomide), and Cellcept (mycophenolate).
Vasculitis or inflammation of small blood vessels can also be relieved by prednisone and disease-modifying anti-rheumatic drugs (DMARDs).
Living with SS
The pain and stiffness caused by SS can sometimes limit people’s normal activities, including work. There are many things people can do to lessen the impact of SS on their work and daily routine.
We recommend adjusting features of the workplace to help make working with SS easier. For example, people that sit for much of the day can adjust the position of chairs and desks for proper posture. Vehicle seats can be adjusted to make driving more comfortable and reduce stress on joints and affected tissues.
Those who find out they have SS while they’re still young can consider choosing work that isn’t physically demanding on their joints and back.
Exercise is important to overall health. It also helps keep the joints moving properly, and helps protect them by strengthening the muscles around them.
The level and amount of exercise people with SS can do depends on the activity of their disease.
A trained arthritis physiotherapist is the best person to help design an exercise program tailored to the needs of patients with SS.
The following are some useful articles on exercising with arthritis:
Exercise and Arthritis: An article by arthritis physiotherapist Marlene Thompson
Exercising in a Flare: Another excellent article by Marlene Thompson on how to cope with flares through your exercise routine.
Alcoholic beverages are not an effective treatment for SS, and they can interact with many medications.
People with SS that plan on drinking should ask their doctor about possible negative interactions with the medications they take.
Some examples of medication that mixes poorly with alcohol includes, and is not limited to: the DMARDs methotrexate, Arava (leflunomide), and Imuran (azathioprine).
Smoking is not healthy for people with SS. It can make symptoms worse and harder to treat.
Research has shown that some arthritis medications do not work as well in people who smoke.
We recommend that smokers quit immediately to reduce the impact of their symptoms, improve the effectiveness of their medications, and improve their overall health.
Smoking significantly increases the risk of cardiovascular disease. The chronic (long-term) inflammation caused by rheumatic diseases like SS already increases this risk without a big extra boost from smoking.
Travel is still possible when you have SS.
It is best for people with this disease to get organized well in advance of a trip to ensure a smooth, comfortable, and enjoyable time.
Check out our Travel Checklist page.
Though SS does not particularly cause a loss of sex drive, it can cause pain, fatigue and emotional hardships. These hardships risk creating barriers to sexual needs, ability, and satisfaction.
People with SS can take comfort knowing that sex and intimacy can be maintained. In many cases, it can help draw partners closer together, especially through improved communication.
For more information on intimacy and arthritis, a great book is: Rheumatoid Arthritis: Plan to Win by Cheryl Koehn, Taysha Palmer and John Esdaile.
Sjögren’s Syndrome (SS) has no effect on fertility. The chances of getting pregnant for people with SS are the same as with people that don’t have it. SS also has no known effects on the developing fetus or the newborn.
The two main issues doctors worry about with SS and pregnancy are:
Patients with SS might be treated with a number of medications that can affect pregancies including NSAIDs, anti-TNF biologics, and occasionally DMARDs. It is important for people with SS who want to become pregnant to discuss their medications with their doctor and create a pre-pregnancy plan. A plan is important for a number of reasons. For example, discontinuing a particular medication for safety of the newborn might cause SS to flare, so its wise to have a plan in place to handle these possibilities.
Any type of arthritis that affects the pelvis or the hips can make vaginal delivery difficult. It is important for those planning pregnancy to discuss delivery options with an obstetrician.
Following the basics of healthy eating can help improve health and well-being in everyone, including those with SS. Keeping a healthy weight helps reduce the load on weight-bearing joints including the spine, hips, and knees, making it easier to live with the disease.
Unfortuantely, no special diet has ever been proven to significantly alter the course of SS or any other types of arthritis.
In addition, no known natural remedies or complementary therapies have been proven to help SS in any significant way.
People with SS should consult with their doctor to make sure that any supplements or alternative therapies they consume will not negatively interact with their medications.
Its very important for people with SS to attend regularly scheduled appointments with their rheumatologist, and promptly get any tests that are ordered by their doctors.
It is often very important that medications are taken exactly as prescribed. Patients should always talk to their doctor about any issues they experience, or if they would like to change anything.
Sjögren’s Syndrome is a chronic disease and a rheumatologist is the best partner in helping patients achieve the best quality of life possible.