Overview of Sjögren’s Syndrome

Sjögren’s syndrome, also known as Sjögren’s and Sjögren’s disease, is a chronic (long-lasting) autoimmune disorder that happens when the immune system attacks the glands that make moisture in the eyes, mouth, and other parts of the body. The main symptoms are dry eyes and mouth, but other parts of the body may be affected as well, with many people reporting fatigue and joint and muscle pain. In addition, the disease can damage the lungs, kidneys, and nervous system. Sjögren’s syndrome predominantly affects women.

Sjögren’s syndrome can occur by itself or alongside other autoimmune conditions such as rheumatoid arthritis or systemic lupus erythematosus.

There is no cure for Sjögren’s syndrome, but there are several ways to treat and manage the symptoms.

Who Gets Sjögren’s Syndrome?

Most people with Sjögren’s syndrome are women. You can get it at any age, but it is most common in people in their 40s and 50s. It occurs across all racial and ethnic backgrounds.

Types of Sjögren’s Syndrome

Doctors divide Sjögren’s syndrome into two categories:

  • Primary form. You have this form if you do not have another rheumatic disease.
  • Secondary form. You have this form if you also have another rheumatic disease, such as rheumatoid arthritis, systemic lupus erythematosus, scleroderma, or polymyositis.

Symptoms of Sjögren’s Syndrome

Sjögren’s syndrome may have different effects on the body, and the symptoms vary from person to person. In some people, symptoms cycle between mild and severe.

The classic symptoms are:

  • Dry eyes. Your eyes may burn or itch or feel like they have sand in them. Sometimes, the dryness causes blurry vision or sensitivity to bright light. You may get irritated, itchy eyelids due to inflammation.
  • Dry mouth. Your mouth may feel chalky, and you may have trouble swallowing, speaking, and tasting. Because you lack the protective effects of saliva, you may develop more dental decay (cavities) and mouth infections, such as candidiasis (also called thrush).

In some people, the main problem is dry mouth, while for others it is dry eyes, and some people experience both problems equally. In some cases, Sjögren’s syndrome affects other tissues and organs and has more widespread effects on the body. These other effects may cause:

  • Fatigue.
  • Joint pain.
  • Dry skin.
  • Dry nasal passages and throat, and a dry cough.
  • Skin rashes.
  • Muscle aches.
  • Acid reflux.
  • Vaginal dryness.
  • Swelling of the glands around the face and neck.
  • Trouble sleeping.
  • Poor concentration and memory problems.
  • Numbness, tingling, and weakness, especially in the extremities.
  • Shortness of breath or trouble breathing.
  • Muscle weakness.

The symptoms can be severe, with some people reporting debilitating pain and fatigue.

People with Sjögren’s syndrome have a higher chance of developing a type of cancer called lymphoma, but the risk of developing it is low.

Causes of Sjögren’s Syndrome

Sjögren’s syndrome is an autoimmune disorder that happens when the immune system attacks healthy tissues. Normally, the immune system protects the body from infection and disease.

Researchers do not know what causes the immune system to turn on the body, but they believe that both genetic and environmental factors are involved. Studies have linked Sjögren’s syndrome to variants (changes) in several genes, many of which are involved in immunity.

In Sjögren’s syndrome, the immune system attacks the glands that make tears and saliva. The resulting inflammation damages the glands, limiting their production of the fluids that normally keep the eyes and mouth moist. In some cases, the immune system attacks additional parts of the body, damaging other organs and tissues and causing a range of other symptoms.