Sjögren's Syndrome

Fact: The cause of Sjogren's syndrome remains unknown

What is Sjögren's Syndrome?

Sjögren's syndrome is an autoimmune disease characterized by dryness of the mouth and eyes. Autoimmune diseases feature the abnormal production of extra antibodies in the blood that are directed against various tissues of the body. The misdirected immune system in autoimmunity tends to lead to inflammation of tissues. This particular autoimmune illness features inflammation in glands of the body that are responsible for producing tears and saliva. Inflammation of the glands that produce tears (lacrimal glands) leads to decreased water production for tears and dry eyes. Inflammation of the glands that produce the saliva in the mouth (salivary glands, including the parotid glands) leads to decreased saliva production and dry mouth and dry lips.

Sjögren's syndrome with gland inflammation (resulting in dry eyes and mouth, etc.) that is not associated with another connective tissue disease is referred to as primary Sjögren's syndrome. Sjögren's syndrome that is also associated with a connective tissue disease, such as rheumatoid arthritis, systemic lupus erythematosus, or scleroderma, is referred to as secondary Sjögren's syndrome. Dryness of eyes and mouth, in patients with or without Sjögren's syndrome, is sometimes referred to as sicca syndrome.

If you have an autoimmune disease, your immune system, which is supposed to fight disease, mistakenly attacks parts of your own body. In Sjogren's syndrome, your immune system attacks the glands that make tears and saliva. It may also affect your joints, lungs, kidneys, blood vessels, digestive organs and nerves.
How is Sjögren's Syndrome diagnosed?
Along with the usual medical history, physical examination, and blood tests, certain special tests that may be ordered include:
  • Schirmer test - doctor puts paper strips under lower eyelids and measures wetness on the paper after 5 minutes
  • Slit lamp exam - performed by an ophthalmologist using equipment to magnify and examine the eye, checking for dryness and inflammation
  • Staining with vital dyes - checking for damage to the surface of the eye
  • Mouth exam - the doctor observes the mouth for abnormalities and may obtain a saliva sample for quality check
  • Lip biopsy - doctor removes tiny salivary glands from lower lip and examines them under a microscope
Are there any treatments for Sjögren's Syndrome?

There is no known cure for Sjögren's syndrome nor is there a specific treatment to restore gland secretion. Treatment is generally symptomatic and supportive. Moisture replacement therapies may ease the symptoms of dryness. Nonsteroidal anti-inflammatory drugs may be used to treat musculoskeletal symptoms. For individuals with severe complications, corticosteroids or immunosuppressive drugs may be prescribed.
As with other autoimmune diseases, the severity of Sjögren’s varies from person to person. Many patients have a mild disease that only affects the eyes and mouth. Others have symptoms that wax and wane in severity, or may even go into remission. Some have severe and chronic (long-term) symptoms.

Current treatments focus on managing the symptoms. Moisture replacement therapies help relieve dryness and nonsteroidal anti-inflammatory drugs (NSAIDs) to control inflammation. People with severe Sjögren’s syndrome may receive corticosteroids, which mimic hormones that fight inflammation in the body, or disease-modifying antirheumatic drugs (DMARDs), which suppress the body’s immune response. In addition to a primary care physician, other members of the healthcare team may include a rheumatologist, an ophthalmologist and a dentist.
Symptoms of Sjögren's Syndrome
  • Central Nervous System involvement
  • Dry eyes
  • Dry mouth
  • Digestion
  • Pain and fatigue

Rare complications linked to Sjogren's syndrome include:
  • pain, stiffness, mild swelling in the joints even in patients who do not have rheumatoid arthritis or lupus
  • rashes on arms and legs related to vasculitis
  • inflammation in the lungs, liver, and kidney
  • neurological complications resulting in numbness, tingling, and weakness
  • lymphoma in a small number of patients
What is the Prognosis?Sjögren's syndrome can damage vital organs of the body with symptoms that may remain stable, worsen, or go into remission. Some people may experience only the mild symptoms of dry eyes and mouth, while others go through cycles of good health followed by severe disease. Many patients are able to treat problems symptomatically. Others are forced to cope with blurred vision, constant eye discomfort, recurrent mouth infections, swollen parotid glands, hoarseness, and difficulty in swallowing and eating. Debilitating fatigue and joint pain can seriously impair quality of life.

National Institute of Neurological Disorder and Stroke
Want to learn more?

The following organizations and/or websites offer additional information and education about Sjögren's Syndrome. They are listed in Alphabetical order without any preference or prejudice. Listing these organizations is not a recommendation or referral in any regard for seeking treatment or consultation or support for treatment.

Arthritis Foundation
Mayo Clinic
National Institute of Neurological Disorder and Stroke
Sjogren's Syndrome Foundation
The British Sjogren's Syndrome Foundation