An Overview of Sjogren's Syndrome

A Systemic Autoimmune Disease

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Sjogren's syndrome is a condition that causes joint pain, dry eyes and mouth, and a number of effects throughout the body. It is a chronic autoimmune disease that typically affects adults. There are several markers of inflammation that can be identified with blood tests. Along with your symptoms, the presence of these markers can help in the diagnosis of Sjogren's syndrome

Treatment often includes immunosuppressive medications, such as steroids. You may also need symptomatic treatment for comfort, like eye drops.

According to the Sjogren's Syndrome Foundation, this condition is among the most prevalent autoimmune disorders—it is estimated to affect as many as 4 million Americans. Sjogren's syndrome is more common in women than men, and approximately 90 percent of people who have the condition are women. The average age of onset is over 40 years old—but men, women, and children can develop Sjogren's syndrome at any age.

Sjogren's syndrome symptoms
Illustration by JR Bee, Verywell​​

Symptoms

Sjogren's syndrome can cause a number of uncomfortable symptoms. It is a chronic condition, and the symptoms may come and go, but they tend to flare up and reappear for years.

The effects of Sjogren's syndrome may seem unrelated to each other—many people postpone talking to their doctors about it because it produces mild, somewhat vague-sounding effects. Some of the effects are similar to those of rheumatoid arthritis and lupus—in fact, approximately half of all people who have Sjogren's syndrome also have another autoimmune condition.

Common symptoms of Sjogren's syndrome include:

  • Dry eyes, which may feel gritty or burning
  • Eye irritation and redness
  • Dry mouth
  • Difficulty chewing, swallowing and talking
  • Cracked or sore tongue
  • Dry, burning throat
  • Changes in your taste or smell sensations
  • Dental decay
  • Yeast infections in your mouth
  • Joint stiffness, swelling, and pain
  • Digestive problems
  • Dry nose and skin
  • Chronic cough
  • Swelling of glands around the face, jaw, and ears
  • Vaginal dryness
  • Fatigue
  • Enlarged lymph nodes

Keep in mind that fevers, severe pain, redness, or swelling of the eyes, mouth, joints, or throat can be a sign of an infection—these symptoms require urgent medical attention.

Sjogren's syndrome can produce a number of rare or serious effects, with a variety of distressing symptoms, such as pain, fevers, infections, coughing, breathing difficulties, decreased urine volume, and confusion.

Rare complications of Sjogren's syndrome include:

  • Infections of the eyes or mouth
  • Rashes on arms and legs
  • Vasculitis, which is inflammation of the blood vessels (can contribute to the rashes)
  • Inflammation in the lungs, liver, and kidney
  • Neurological issues, resulting in numbness, tingling, and weakness
  • Lymphoma

Causes

The cause of Sjogren's syndrome is unknown. It is an autoimmune inflammatory condition. This means that the disease occurs as the body's immune system attacks itself, producing inflammation and tissue damage. Sjogren's syndrome is also characterized by decreased production of tears, saliva, and mucus—resulting in dryness of the body's membranes.

Primary and Secondary Sjogren's syndrome

Sjogren's syndrome—named after the Swedish doctor, Henrik Sjögren, who discovered it in the early 1900s— can occur as a primary condition or as a secondary condition. Primary Sjogren's syndrome is diagnosed if you do not have another autoimmune disease.

Sjogren's syndrome is considered a secondary condition when it occurs along with another autoimmune disease.

Risk Factors

If you have rheumatoid arthritis, systemic lupus erythematosus, scleroderma, polymyositis, or dermatomyositis, you are also more likely to have Sjogren's syndrome. These are autoimmune conditions, and experts suggest that the same trigger that causes other autoimmune conditions also cause Sjogren's syndrome.

Genetic factors, hormonal factors, or a viral infection have been considered the root cause of autoimmune disease, but there is no solid evidence that one particular cause is at play in Sjogren's syndrome.

How Sjogren's Syndrome Develops

Inflammatory cells and proteins can damage the mucus and tear glands, impairing their function. Several immune proteins called cytokines are elevated in Sjogren's syndrome, specifically Th-1, Th-2, and Th-17 cytokines.

Diagnosis

Because the symptoms are somewhat vague, it can take years for Sjogren's syndrome to be diagnosed. Your symptoms, physical examination, specialized tests, and blood tests can help identify the condition.

Your doctor or dentist may observe that your mouth is dry. Your nose and skin may appear dry and your eyes may also appear red or dry. Your joints may appear swollen, and you may have a rash on your skin.

Specialized Tests

Along with your symptoms and physical examination, a number of diagnostic tests may help formulate the diagnosis of Sjogren's syndrome.

  • Schirmer's test for dry eyes: Your doctor puts paper strips under your lower eyelids to measure the wetness on the paper after 5 minutes.
  • Slit lamp exam: An ophthalmologist checks for eye dryness and inflammation using a slit lamp to magnify and examine your eye.
  • Staining with vital dyes: A harmless dye is placed on the surface of your eye. A special light can be used to help your doctor see any damage on the surface of your eye. 
  • Mouth exam: You doctor visually checks your mouth for abrasions and swelling and may obtain a saliva sample for quality check.
  • Lip biopsy: Your doctor can surgically remove tiny salivary glands from your lower lip for a microscopic examination.

Blood Tests

Several blood tests can be used to identify inflammatory markers that are commonly elevated in Sjogren's syndrome.

  • Antinuclear antibody (ANA test): ANA is an antibody that attacks the body's cells. It is positive in approximately 70 percent of people who have Sjogren's syndrome. This antibody may also be present in people who do not have any autoimmune disease, however.
  • SSA (anti-Ro) and SSB (anti-La): These antibodies are common with primary Sjogren's syndrome, but you may not test positive for SSA and SSB even if you have the condition.
  • Rheumatoid factor: Rheumatoid factor is a protein found in rheumatoid arthritis, and it is positive among approximately 65 percent of people who have Sjogren's syndrome.
  • Immunoglobulins: Proteins normally produced by antibodies may be elevated in Sjogren's syndrome.

No single test by itself can confirm that you have Sjogren's syndrome. A combination of factors is considered together in your diagnosis.

Treatment

Although there is not a cure for the condition, many of the effects of Sjogren's syndrome can be managed. Symptomatic treatment is tailored for each person, depending on which areas of the body are affected, and how bothersome the symptoms are. For example, joint pain or muscle pain may be managed with non-steroidal anti-inflammatory drugs (NSAIDs), while serious complications such as lymphoma need to be treated with chemotherapy.

Immunosuppressive therapy is often used to reduce the inflammatory process that damages the glands, joints, and organs in Sjogren's syndrome—this approach is often referred to as disease-modifying therapy.

Relief of Oral Symptoms

Mouthwashes, lip balm, saliva substitutes, sprays, gels, and gum can relieve your dry mouth and tenderness. Medication options may include saliva and mucus stimulating drugs, such as:

  • Salagen (Pilocarpine hydrochloride)
  • Evoxac (Cevimeline HCI)

Relief of Eye Symptoms

There are some practical ways to manage dry eyes. Sunglasses can protect your eyes from wind and drafts outdoors, and a humidifier can relieve dry air indoors to help alleviate the symptoms. Smoke and eye makeup can be irritating and should be avoided.

Artificial tears and eye ointments can help relieve chronic dry eyes. Medication options for dry eye associated with Sjogren's syndrome may include:

  • Restasis (Cyclosporine Ophthalmic Emulsion)
  • Hydroxypropyl Cellulose (Eye Drops and Pellets)

Disease-Modifying Therapy

Lung, kidney, blood vessel, or nervous system problems may be reduced with medications that suppress the immune system. These medications can cause side effects, including a predisposition to infections, so they are not necessarily the right option for someone who only has mild effects of Sjogren's syndrome or who is otherwise prone to infections.

Disease-modifying treatments include:

A Word From Verywell

The average time from the onset of symptoms to diagnosis of Sjogren's syndrome is said to be more than six years. Dry mouth and dry eyes are the most common effects associated with Sjogren's syndrome, but there are many other causes of these symptoms.

Sjogren's syndrome is a treatable condition. With effective treatment, most people live healthy lives.

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