Sjögren syndrome

Xerostomia-Sjögren syndrome; Keratoconjunctivitis sicca - Sjögren; Sicca syndrome

The cause of Sjögren syndrome is unknown. It is an autoimmune disorder. This means the body attacks healthy tissue by mistake. The syndrome occurs most often in women ages 40 to 50. It is rare in children.

Primary Sjögren syndrome is defined as dry eyes and dry mouth without another autoimmune disorder.

Secondary Sjögren syndrome occurs along with another autoimmune disorder, such as:

• Rheumatoid arthritis (RA)
• Systemic lupus erythematosus
• Scleroderma
• Polymyositis

Dry eyes and dry mouth are the most common symptoms of this syndrome.

Eye symptoms:

• Itching eyes
• Feeling that something is in the eye

Mouth and throat symptoms:

• Difficulty swallowing or eating dry foods
• Loss of sense of taste
• Problems speaking
• Thick or stringy saliva
• Mouth sores or pain
• Teeth decay and gum inflammation
• Hoarseness

Other symptoms may include:

• Fatigue
• Fever
• Change in the color of hands or feet
• Joint pain or joint swelling
• Swollen glands

A physical exam reveals dry eyes and dry mouth. There may be mouth sores, decayed teeth or gum inflammation. This occurs because of the mouth dryness. Your health care provider will look in your mouth for fungus infection (Candida).

You may have the following tests done:

• Antinuclear antibodies (ANA) test
• Rheumatoid factor (possible)
• Schirmer test of tear production
• Salivary gland biopsy
• Examination of the eyes

The goal is to relieve symptoms.

• Dry eyes may be treated with artificial tears, eye-lubricating ointments, or cyclosporine liquid.
• If Candida is present, it may be treated with sugar-free miconazole or nystatin preparations.
• Tiny plugs can be placed in the tear drainage ducts to help the tears stay on the surface of the eye.

Disease-modifying antirheumatic drugs (DMARDs) similar to those used for rheumatoid arthritis may improve the symptoms of Sjögren syndrome. These include tumor necrosis factor (TNF) inhibiting drugs such as Enbrel, Humira or Remicaide.

Some things you can do to ease symptoms include:

• Sip water throughout the day
• Chew sugarless gum
• Avoid medicines that can cause mouth dryness, such as antihistamines and decongestants
• Avoid alcohol

Talk with your dentist about:

• Mouth rinses to replace minerals in your teeth
• Saliva substitutes
• Drugs that help your salivary glands make more saliva

To prevent dental decay caused by mouth dryness:

• Brush and floss your teeth often
• Visit the dentist for regular checkups and cleanings

The disease is most often not life-threatening. The outcome depends on what other diseases you have.

There is a higher risk for lymphoma when Sjögren syndrome has been very active for a long time.

Complications may include:

• Damage to the eye
• Dental cavities
• Kidney failure (rare)
• Lymphoma
• Pulmonary infections
• Vasculitis (rare)

Call your health care provider if you develop symptoms of Sjögren syndrome.