Sjogren's Syndrome
all information

Sjogren's Syndrome

Pronounced: show-GRENS

En Español (Spanish Version)

Definition

Sjogren's syndrome is an inflammatory condition in which the immune system destroys cells in exocrine glands, most commonly the tear and salivary glands. It is a lifelong condition. There are two types:

Salivary Glands

Nucleus factsheet image

© 2008 Nucleus Medical Art, Inc.

Causes

The causes of Sjogren's syndrome are unknown. Contributing factors may include:

  • Viral infections
  • Heredity
  • Hormones

Risk Factors

A risk factor is something that increases your chance of getting a disease or condition. Risk factors for Sjogren's syndrome include:

  • Sex: female
  • Age: 40-60 years old
  • Other rheumatic or autoimmune diseases

Symptoms

Symptoms may include:

  • Red, burning, itching, and/or dry eyes
  • Dry mouth
  • Difficulty swallowing
  • Loss of taste and smell
  • Dry skin, nose, throat, and/or lungs
  • Swollen salivary glands
  • Severe dental cavities caused by dry mouth
  • Oral yeast infections
  • Vaginal dryness
  • Skin rashes
  • Joint and muscle pain
  • Fatigue

In some cases, other parts of the body are affected as well. These include:

  • Blood vessels
  • The nervous system
  • Organs such as the lungs, liver, pancreas, kidneys, and thyroid

Diagnosis

Due to the systemic nature of this disease, you may have to see several specialist doctors before a final diagnosis is made. Additionally, the prevalence of dry eyes and dry mouth increases with age and is most often due to medication side effects. The doctor will ask about your symptoms and medical history, and perform a physical exam.

Tests may include:

  • Blood tests—including rheumatoid factor and other tests used to detect and monitor autoimmune diseases
  • Chest x-ray—a test that uses radiation to take a picture of structures inside the body, in this case the lungs
  • Lip biopsy—removal of a sample of lip tissue to look for inflammation in the accessory salivary glands located there
  • Schirmer test—placement of small pieces of paper between the lower eyelid and eyeball to see how much moisture is being produced
  • Slit-lamp examination—a detailed exam of the eye with an adjustable light
  • Urine test—to check kidney function

Treatment

There is no cure for Sjogren's syndrome and no treatment that can restore the ability of the glands to produce moisture. Patients with Sjogren’s syndrome have an increased incidence of non-Hodgkin’s lymphoma and should be screened aggressively. The goal of treatment is to relieve symptoms.

Treatments include:

Medication

  • Artificial tears, artificial saliva, and vaginal lubricants can help relieve dryness.
  • Aspirin or nonsteroidal anti-inflammatory drugs may relieve joint and muscle pain.
  • Pilocarpine may help relieve ocular and oral dryness.
  • Cevimeline requires less frequent dosing than pilocarpine, but may cause nausea.
  • Steroids help relieve inflammation and swelling.
  • Methotrexate is used as a steroid-sparing agent.

Lifestyle Measures

  • Mild exercise can help relieve joint stiffness.
  • Sipping liquids frequently and sucking on sugar-free candies can relieve dry mouth.
  • Brushing, flossing, and seeing your dentist regularly can prevent cavities.
  • Using nonscented moisturizers can help relieve dry skin.

While Sjogren's syndrome is generally a benign condition, people with severe cases are at increased risk for developing lymphoma , a cancer of the white blood cells. Your doctor will need to monitor you closely for this possibility.

Prevention

There are no guidelines for preventing Sjogren's syndrome because the cause is unknown.

RESOURCES:

American Autoimmune Related Diseases Association
http://www.aarda.org

Sjogren's Syndrome Foundation
http://www.sjogrens.org

CANADIAN RESOURCES:

Alberta Health and Wellness
http://www.health.gov.ab.ca/

BC Health Guide
http://www.bchealthguide.org/

References:

Arthritis Foundation website. Available at: http://www.arthritis.org/ .

The Cleveland Clinic Foundation website. Available at: http://www.clevelandclinic.org/ .

Fox RI. Sjogren’s syndrome. Lancet. 2005;366:321-331.

Kassan SS, Montsopolous HM. Clinical manifestations of Sjogren’s disease. Arch Intern Med. 2004;164:1275-1284.

National Institute of Arthritis and Musculoskeletal and Skin Diseases, National Institutes of Health (NIH) website. Available at: http://www.niams.nih.gov/ .

Papas, et al. Successful treatment of dry mouth and dry eye symptoms in Sjogren's syndrome patients with oral pilocarpine: a randomized, placebo-controlled, dose-adjustment study. J Clin Rheumatol . 2004;10:169-177.

Sjogren's Syndrome Foundation website. Available at: http://www.sjogrens.org .

University of Washington Orthopaedics and Sports Medicine website. Available at: http://www.orthop.washington.edu/ .

Venables PJ. Management of patients presenting with Sjogren's syndrome. Best Pract Res Clin Rheumatol . 2006;20:791-807.



Last reviewed December 2007 by Jill Landis, MD

Please be aware that this information is provided to supplement the care provided by your physician. It is neither intended nor implied to be a substitute for professional medical advice. CALL YOUR HEALTHCARE PROVIDER IMMEDIATELY IF YOU THINK YOU MAY HAVE A MEDICAL EMERGENCY. Always seek the advice of your physician or other qualified health provider prior to starting any new treatment or with any questions you may have regarding a medical condition.

DiggDeliciousNewsvineRedditStumbleTechnoratiFacebook