What is Sjögren's syndrome?

Sjögren's syndrome is a chronic (long-lasting) disorder that causes insufficient moisture production in some glands of the body, primarily in the eyes and mouth.

Sjögren's syndrome occurs when a person's normally protective immune system attacks her/his body and destroys moisture-producing glands, including salivary (saliva-producing) glands and lacrimal (tear-producing) glands. The lungs, bowel, and other organs may be affected, but relatively less often.

Sjögren's syndrome is named after the Swedish eye doctor, Henrik Sjogren, who first described the condition.

Sjögren’s syndrome is characterized by dry eyes and mouth. In some patients, the parotid glands may become visibly enlarged.

What are the forms of Sjögren's syndrome?

Sjögren's syndrome occurs in two basic forms:

  • Primary Sjögren's syndrome – the disease by itself, not associated with any other illness
  • Secondary Sjögren's syndrome – disease that develops in the presence of another autoimmune disease such as rheumatoid arthritis, systemic lupus erythematosus, or psoriatic arthritis

Who is affected by Sjögren's syndrome?

More than one million people in the United States have Sjögren's syndrome (about 0.5 to 1% of the population). More than 90% of people affected by Sjögren's syndrome are women. The disease can affect people of any race or age, but affects mostly middle-aged individuals.

What causes Sjögren's syndrome?

Normally, the immune system (the body's defense system) protects the body from infection and foreign substances such as bacteria and viruses.

In autoimmune diseases such as Sjögren's syndrome, the immune system triggers an inflammatory response when there are no foreign substances to fight off. This inflammatory response causes the body's white blood cells to attack and destroy its own moisture-producing glands.

The exact cause for the abnormal immune response in Sjögren's syndrome is unknown. There are four factors that may work together to cause the medical problems:

  • abnormal immune response
  • sex hormones
  • inheritance, and
  • environment

Certain people may have a genetic or inherited factor that makes them more likely to develop Sjögren's syndrome.

Can other problems mimic Sjögren's syndrome?

The use of certain medications such as tricyclic antidepressants and antihistamines can cause the symptoms of Sjögren's syndrome. Radiation treatments to the head and neck and other autoimmune disorders can also cause severely dry eyes and mouth. Hepatitic C, sarcoidosis, and HIV infection can also cause these dry symptoms.

What are the symptoms of Sjögren's syndrome?

Sjögren's syndrome is mostly characterized by dry eyes and mouth. In some patients, the parotid glands may become visibly enlarged.

The main symptoms of Sjögren's syndrome are:

  • Extremely dry eyes, causing a feeling of grit or sand, burning, and redness
  • Inner angle thick secretions
  • Extremely dry mouth and throat, causing:
    • difficulty chewing and swallowing, especially dry food such as crackers
    • decreased or altered sense of taste
    • difficulty speaking
    • increase in dental cavities and even tooth loss at early age
    • dry cough or hoarseness
  • Enlarged parotid glands (located at the angle of jaw), looking like an infection
  • Excessive fatigue
  • Aches and pains in muscles and joints, and even the whole body, similar to fibromyalgia pain

Less common features of Sjögren's syndrome are:

  • Irritation of the nerves in the arms, hands, legs, or feet (neuropathy)
  • Feeling of numbness or tingling
  • Thyroid gland abnormalities
  • Skin rashes
  • Memory loss, difficulty concentrating or confusion
  • Gastrointestinal problems, such as acid reflex, bloating, abdominal pain, or diarrhea
  • Inflammation of the lungs, kidneys (unlike lupus nephritis), liver, or pancreas
  • Cancer of the lymphatic tissue (occurs in up to 5% of patients with the disease)

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