About Sjögren's syndrome

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What Sjögren's syndrome

Sjögren's syndrome is an inflammatory autoimmune disease (like rheumatoid arthritis) in which the body’s immune system attacks the body’s own organs, causing inflammation and dysfunction. The most commonly affected organs in Sjögren’s syndrome are the tear and salivary glands, resulting in symptoms of dry eyes and dry mouth. However, the disease can affect almost every organ in the body including the liver, kidneys, lungs, skin, joints, nerves, and brain.

The main symptoms of Sjögren's syndrome

Not everyone experiences Sjögren’s syndrome in the same way – presentation of the disease can vary from mild symptoms of dryness to life-threatening and debilitating systemic symptoms. The most common symptoms of Sjögren’s syndrome are the following:
  • Dry eyes (gritty, painful, blurry vision)
  • Dry mouth (difficulty swallowing, frequent dental cavities, painful cracked tongue)
  • Dry skin and nostrils
  • Swollen salivary glands
  • Fatigue
  • Joint pain, swelling, and stiffness
Of note, dry eyes and mouth are very common symptoms, and can be associated with aging, menopause, medications, and a number of medical conditions that are not autoimmune. Only a very small percentage of patients with dry eyes and mouth in fact have Sjögren’s syndrome. It is important to be assessed by your doctor for these symptoms to determine if they need to be further investigated.

Who can develop Sjögren's syndrome?

Sjögren's syndrome affects approximately 1% of the Canadian population. Women are much more commonly affected than men - 90% of those diagnosed are women. Though it can occur in people of all ages, it tends to strike around middle age; most commonly, people are diagnosed between the ages of 45 and 55. Public awareness and recognition of symptoms is the key to earlier diagnosis and treatment.

Approximately half of those diagnosed with Sjögren's syndrome also have another concurrent autoimmune disease, like lupus, scleroderma, or rheumatoid arthritis—this is called "secondary Sjögren's syndrome”. In the other half of cases, Sjögren's syndrome appears in people with no other autoimmune disease. In this case, the disease is referred to as "primary Sjögren's syndrome".

Like other autoimmune disease, the cause of Sjögren's syndrome remains unknown. It is likely a result of genetic susceptibility combined with some environmental trigger such as a viral infection. Because the disease most often affects women at the end of their childbearing years, it has also been speculated that it may have a hormonal connection.

Getting a diagnosis of Sjögren's syndrome

If your doctor believes you may have Sjögren's syndrome, you will usually be referred to a rheumatologist—a specialist in the treatment of autoimmune disease. If your eyes appear to be involved in your disease, you may also be referred to an ophthalmologist—a medical doctor specializing in eyes.

To establish a diagnosis of Sjögren’s syndrome, your rheumatologist will take an extensive history and full physical exam. They will also need to do special tests in the office to measure your tear and saliva production. Not every rheumatologist and ophthalmologist performs these tests, so they may further refer you to a Sjögren’s syndrome clinic if this is available in your area. In addition, you will need a blood test to look for antibodies related to Sjögren’s syndrome, and sometimes even a biopsy of your salivary glands – a minor procedure performed by an ear/nose/throat surgeon in their office, involving a small incision in the inner part of the bottom lip.

Once a diagnosis of Sjögren’s syndrome has been established, it is important to see a rheumatologist at least once a year. The reason for this is that people with Sjögren’s syndrome can develop more serious symptoms affecting almost every organ of their body. They are also at higher risk of a cancer called lymphoma. Your rheumatologist should monitor for these problems with a full physical examination and blood tests on a regular interval.

Treatment of Sjögren's syndrome1

While there is no known cure for Sjögren's syndrome, treatments to manage your symptoms are available, and your rheumatologist is the best person with whom to discuss these and formulate a treatment plan to address all aspects of Sjögren's syndrome.
  1. Treating Dry Eyes
    Presently, there are unfortunately no medications that reverse or slow down the inflammation of tear glands leading to dry eyes. Treatment is targeted towards reducing symptoms and preventing complications. Use of artificial tears throughout the day is recommended, preferably preservative-free tears, especially if using more than 4 times a day. This is because the preservatives in eye drops can actually worsen irritation of the eye surface. Gels and ointments can be used at nighttime for longer lasting effect. It is also recommended that you apply warm compresses and a gentle cleanser to the eyes twice a day, as this relieves clogging of the oil glands in the eyelids that can exacerbate dry eye symptoms. Regular follow-up with an ophthalmologist or optometrist with expertise in dry eye management is important, as they can monitor for any complications of dry eye like corneal ulcers. They can also prescribe various medicated drops to reduce inflammation of the eye, and can perform procedures such as plugging the tear ducts to keep tears pooled in the eye.
  2. Treating Dry Mouth
    In Sjögren's syndrome, the immune system is attacking the glands that keep the eyes, mouth, and other body parts moist. Because your body can’t produce saliva readily, it may be difficult to swallow or speak, or to taste food. It is important for your doctor to review your medications, as a number of both prescribed and over the counter medications can worsen dry mouth. Avoid oral irritants when possible such as coffee, tea, alcohol, tobacco, and cannabis. Avoid low-humidity environments and consider using a humidifier at nighttime. For mild dry mouth, using sugarless gums, candies, and lozenges can increase saliva production. A prescribed medication called pilocarpine can be used to increase saliva production in those with moderate dry mouth. For those with severe dry mouth where there is little to no saliva production, artificial saliva products can be helpful – these can be purchased over the counter, and come in many different formulations. You may need to experiment to find one that is most tolerable for you.

    An important part of managing dry mouth is to prevent complications due to reduced saliva production. The most important of these is dental decay, which can occur very rapidly and severely in patients with dry mouth. Meticulous brushing and flossing should occur at least twice a day. We recommend the use of high fluoride preparations including mouthwashes, toothpastes, and gels, with professional application of fluoride by a dentist every 3 months. Oral thrush is another complication of dry mouth. This is a fungal infection can present as a white coating on the tongue, or in patients with dry mouth, can also present as a painful, red, and enlarged tongue. This often requires anti-fungal treatment prescribed by your doctor. Finally, reduced saliva production can result in more viscous saliva and salivary stones, which can clog your saliva glands and cause painful enlargement and inflammation. This can sometimes be prevented with regular massage of the salivary glands, located over the angle of the jaw just in front of the ear, after every meal. If symptoms do not resolve, it is important to see your doctor right away, as an infection of the gland can occur.

  3. Treating systemic symptoms of Sjögren’s syndrome
    While there is no role for the use of anti-inflammatory and immunosuppressive medications in the treatment of dry eyes and dry mouth, these medications can be prescribed for people with Sjögren’s syndrome who have systemic symptoms of the disease, depending on what body part is involved and the severity of the involvement. An example of these medications include the following:
    • Non-steroidal anti-inflammatory drugs (NSAIDs), such as ibuprofen (for example Advil® or Motrin IB®), naproxen (or Naprosyn®), diclofenac (or Voltaren® and Arthrotec®)
    • Corticosteroids, such as prednisone
    • Disease-modifying anti-rheumatic drugs (DMARDs) such as methotrexate, sulfasalazine, hydroxychloroquine, azathioprine, and rituximab
General healthy lifestyle recommendations

As with any form of autoimmune disease, maintaining a healthy lifestyle is an important part of any treatment plan. Poor diet, lack of exercise, and high levels of stress may make disease activity worse, so healthy eating, appropriate levels of aerobic and strengthening exercise, and relaxation are highly recommended. As well, since smoking can cause dryness in the skin, mouth, nose, and eyes, it is highly recommended that people with Sjögren's syndrome do not smoke. Keeping skin from getting too dry is important. Try using creams, or mineral oils to protect skin and keep it from becoming uncomfortable and dry.

Thank you to Dr. Alice Mai, rheumatologist and Director of the Sjogren’s Clinic at the Mary Pack Arthritis Centre in Vancouver, British Columbia, for her medical review of the content on this page.

1Luo, H., Li, X., Liu, J., Andrew, F., & George, L. (2012). Chinese herbal medicine in treating primary Sjögren’s Syndrome: A systemic review of randomized trials. Evidence-Based Complementary and Alternative Medicine, 2012.