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Sjögren syndrome

Definition

Sjögren syndrome is an autoimmune disorder in which the glands that produce tears and saliva are destroyed. This causes dry mouth and dry eyes. The condition may affect other parts of the body, including the kidneys and lungs.

Alternative Names

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

Causes

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:

Symptoms

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

Eye symptoms:

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:

Exams and Tests

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:

Treatment

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

Outlook (Prognosis)

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.

Possible Complications

Complications may include:

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

When to Contact a Medical Professional

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

References

Baer AN, Hall JC. Sjogren syndrome. In: Hochberg MC, Silman AJ, Smolen JS, Weinblatt ME, Weisman MH, eds. Rheumatolgy. 6th ed. Philadelphia, PA: Elsevier Mosby; 2015:chap 138.

Kruszka P, O'Brian RJ. Diagnosis and management of Sjogren syndrome. Am Fam Physician. 2009;79(6):465-470. PMID: 19323359 www.ncbi.nlm.nih.gov/pubmed/19323359.

Ramos-Casals M, Brito-Zerón P, Sisó-Almirall A, Bosch X, Tzioufas AG. Topical and systemic medications for the treatment of primary Sjögren's syndrome. Nat Rev Rheumatol. 2012;8(7):399-411. PMID: 22549247 www.ncbi.nlm.nih.gov/pubmed/22549247.

St. Clair WE. Sjogren's syndrome. In: Firestein GS, Budd RC, Gabriel SE, McInnes IB, O'Dell JR, eds. Kelley's Textbook of Rheumatology. 9th ed. Philadelphia, PA: Elsevier Saunders; 2012:chap 73.

Review Date:4/28/2015
Reviewed By:Gordon A. Starkebaum, MD, Professor of Medicine, Division of Rheumatology, University of Washington School of Medicine, Seattle, WA. Also reviewed by David Zieve, MD, MHA, Isla Ogilvie, PhD, and the A.D.A.M. Editorial team.

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