Sjögren's Syndrome

What is Sjögren's syndrome?

Sjögren's (SHOW-grins) syndrome is a chronic autoimmune disease that targets the moisture-producing glands and mucose membranes in the mouth and eyes, resulting in dry eyes and mouth. Sjögren's syndrome often accompanies other autoimmune disorders, such as rheumatoid arthritis and lupus. It is one of the most common autoimmune disorders with more than four million Americans living with the disease. Nine out of 10 patients are women. The average age for onset of this disease is late forties.

There are two types of Sjögren’s syndrome: primary and secondary. Primary Sjögren’s syndrome refers to patients with gland inflammation (resulting in dry eyes and mouth) and no other underlying rheumatologic condition. Secondary Sjögren’s syndrome refers to patients with Sjögren’s and another rheumatologic condition, such as rheumatoid arthritis, systemic lupus erythematosus (SLE or lupus), or scleroderma. The distinction is important because it informs your prognosis and how you are treated.

Although the hallmark symptoms are dry eyes and mouth, Sjögren’s is a systemic condition which means it can also cause serious complications throughout the body. Patients commonly experience fatigue and joint pain. Other organs that may be affected include the kidneys, gastrointestinal system, blood vessels, lungs, liver, pancreas, and the central nervous system. Symptoms may remain steady, worsen, or, uncommonly, go into remission. While some people experience mild discomfort, others suffer debilitating symptoms that greatly impair their ability to function. Early diagnosis and proper treatment can prevent serious complications and greatly improve quality of life.

What causes Sjögren’s syndrome?

In autoimmune diseases like Sjogren's syndrome, your immune system attacks your body's own cells and tissues causing inflammation and pain. Researchers do not know what causes Sjogren's syndrome, though there may be a genetic link to developing autoimmune disorders plus a trigger, like an exposure to an infection that activates the disease. Research is underway to better understand what causes autoimmune disorders so that cures can be developed and lives can be greatly improved.

What are the symptoms of Sjögren’s syndrome?

Sjogren’s syndrome is characterized by inflammation and dysfunction of the glands that produce saliva and tears. Insufficient moisture results in the two hallmark symptoms:

Dry eyes. You may feel a burning or itching feeling, or the feeling that there is something in your eye. People with Sjogren’s syndrome often develop an intolerance to contact lenses and a "gritty" sensation in the eyes.
Dry mouth. Not enough saliva can make it difficult to speak or swallow.

The dry eyes and mouth symptoms may also be caused by other diseases or medications so it is important that a rheumatologist perform a thorough medical history and assessment of your other symptoms. These can include:

Swollen salivary glands
Skin rashes, dry skin, vaginal dryness
Persistent dry cough
Fatigue
Joint and muscle pain, swelling and stiffness
Dental decay

Sjogren’s syndrome is systemic in nature, which means it can involve your nervous system, lungs, reproductive organs, kidneys, and blood. Late onset symptoms can include:

Burning and numbness of the extremities
Anemia or low white blood cell count

How is Sjögren’s syndrome diagnosed?

There is no one diagnostic test for Sjögren’s syndrome. Dry eyes and dry mouth could be associated with many different diseases so these diseases must be excluded. Also, symptoms can mimic the side effects of medications, so these causes need to be ruled out too. First, assessments by a rheumatologist, ophthalmologist, and a dentist or oral medicine specialist are needed to understand why the glands that produce tears and saliva are not working properly. You may also be tested for other autoimmune diseases as well.

These diagnostic tests help rule out other conditions while confirming a Sjogren's syndrome diagnosis:

Blood test. Tests levels of different types of blood cells and antibodies common in Sjogren's syndrome.
Schirmer’s test. This test measures eye dryness and tear production.
Ocular surface staining. A stain is applied topically to the eyes. The areas where the stain conglomerates indicate corneal erosion/damage.
Lip biopsy. A small tissue sample is removed from salivary glands in your lip and examined under a microscope for tell-tale signs of inflammation.
Sialometry. This test measures how much saliva is produced by the glands into your mouth over one or several minutes.
Ultrasound. This involves imaging of the major salivary glands to identify abnormalities.
Neurological tests. Biopsies of nerves, testing nerve conduction, and testing the brain and spinal cord with MRIs and lumbar punctures can help determine whether Sjögren’s syndrome is causing neurologic complications.
Urine test. A urine test can help determine whether Sjögren’s syndrome is causing kidney complications.

In addition to these tests, your first appointment with a rheumatologist will include a thorough medical history. The medical history of your siblings and parents, especially if you or any relatives have chronic conditions or an autoimmune disorder, should also be discussed. Your rheumatologist will ask you when each symptom first appeared and if there is any pattern to the symptoms, along with questions about diet, exercise habits, and fatigue.

How is Sjögren’s syndrome treated?

There is no cure for Sjögren’s syndrome; it is a lifelong condition. The goal of treating Sjögren’s syndrome is to minimize the impact of symptoms and improve your quality of life. Treatments fall into different categories: regular eye and dental maintenance, enhanced self-care, over-the-counter medications, prescription medications (such as immunosuppressants), and occasionally surgery.

Self-care involves all of the typical personal hygiene practices you already do with a heightened awareness of how they can impact and maybe improve your Sjögren’s symptoms. The first step to manage and treat symptoms includes:

Reduce dry mouth. Fluids, especially water, are often the easiest way to minimize the symptoms of dry mouth. Sugarless gum and hard candies can also boost saliva flow but should be used sparingly. There are sprays and lubricant products that artificially replace saliva and help keep your mouth moist. Also, breathing through your nose, instead of your mouth, and keeping your nasal passages clear with saline spray is another way to reduce dry mouth.
Prevent tooth decay: After every meal, brush and floss your teeth and use mouthwash in the evening for additional fluoride. See your dentist a minimum of twice a year with additional appointments if you experience any symptoms of decay.
Treat and prevent dry eye: Artificial tears and eye lubricants restore moisture to your eye, alleviating dry eye symptoms.
Treat and prevent dry skin: Avoid bathing in hot water or having prolonged exposure to warm water. Use rubber gloves when you do the dishes or clean your house. After bathing or getting wet, pat, don't rub your skin with a towel to dry off and use moisturizer. If you experience vaginal dryness, there are vaginal moisturizers and lubricants to minimize those symptoms.

Talk to your doctor about which over-the-counter treatment is best for you as they each have advantages and disadvantages. Increasing the humidity in your home with a humidifier, staying away from fans or vents that blow air in your eyes, and wearing glasses to protect your eyes are other ways to prevent dry eye. Regular visits to an eye doctor and dentist are important to assure proper treatment of the eye dryness and tooth decay.

If the self-care treatments do not relieve your symptoms, there are some prescription medicines your rheumatologist may prescribe for dry mouth, joint pain, and dry eyes.

When medication and self-care treatments aren't working, there is a minor surgical procedure called punctal occlusion that seals the tear ducts (the part of your eye that drains tears). Lasers can also be used to permanently seal your tear ducts. Nonsurgical barrier methods for managing dry eye involve plugging the tear duct with collagen (which eventually dissolves) or silicone (which is semi-permanent unless it falls out).

When Sjögren’s syndrome affects vital organs such as the kidneys, lungs, or the nervous system, treatment with medications that reduce the activity of the immune system (immunosuppressants) are often needed.

Complications of Sjögren’s syndrome

In the short term, there are consequences of dry eye and mouth for people with Sjögren’s syndrome. Dental cavities can form because without the protective benefit of saliva, teeth are more prone to decay. Similarly, oral yeast infections may develop because of insufficient saliva. Dry eyes can lead to vision problems such as blurred vision, corneal ulcers, and light sensitivity.

Over time, the immune system for people with Sjogren's syndrome can damage other parts of your body, including joints, nerves, skin, and organs such as kidneys, liver, and lungs. These long-term consequences can be quite debilitating. Patients with Sjögren’s are also more likely to develop lymph node cancer (lymphoma) which can be fatal.