Your Health - Sjogren's Syndrome

Sjogren's Syndrome

(Primary Sjogren's Syndrome; Secondary Sjogren's Syndrome)

Pronounced: show-GRENS

Definition

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

Primary Sjogren's syndrome—occurs alone
Secondary Sjogren's syndrome—occurs with other rheumatic conditions such as rheumatoid arthritis , scleroderma , or systemic lupus erythematosus (lupus)

Salivary Glands

Causes

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

Viral infections
Heredity
Hormones

Risk Factors

Factors that increase your risk for Sjogren's 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

Anti Ro antibodies are often present with Sjogren's. These may cross to the baby during pregnancy. In some cases, they can cause neonatal lupus or infant heartblock.

Diagnosis

This disease can affect many areas on your body. You may have to see several specialist before a final diagnosis is made. Dry eyes and mouth also increase with age. They can also be caused by medicine. The doctor will ask about your symptoms and medical history. A physical exam will be done.

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. No treatment can restore the ability of the glands to produce moisture. Patients with Sjogren’s have an increased incidence of non-Hodgkin’s lymphoma . They should be screened aggressively. The goal of treatment is to relieve symptoms.

Treatments include:

Medication

To help relieve dryness:

Artificial tears, artificial saliva, and vaginal lubricants
Pilocarpine —ocular and oral dryness
Cevimeline —requires less frequent dosing than pilocarpine, may cause nausea

To relieve joint and muscle pain:

Aspirin or nonsteroidal anti-inflammatory drugs (NSAIDS)

To relieve inflammation / swelling:

Plaquenil —antimalarial drug with anti-inflammatory properties
Steroids
Methotrexate —a steroid-sparing agent

Lifestyle Measures

Mild exercise can help relieve stiffness in the joints.
To help relieve dry mouth, sip liquids often and suck on sugar-free candies.
Brush, floss, and see your dentist regularly. This can help to prevent cavities.
Use nonscented moisturizers to help relieve dry skin.

This condition is generally benign. However, people with severe cases are at increased risk for developing lymphoma . This is a cancer of the white blood cells. Your doctor will need to monitor you for this.

Prevention

There are no guidelines for preventing Sjogren's syndrome. 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/

Health Canada
http://www.hc-sc.gc.ca/

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.

Pertovaara M, Korpela M, Uusitalo H, et al. Clinical follow up study of 87 patients with sicca symptoms (dryness of eyes or mouth, or both). Ann Rheum Dis. 1999; 58:423.

Ramos-Casals M, Tzioufas AG, Font J. Primary Sjögren's syndrome: new clinical and therapeutic concepts. Ann Rheum Dis. 2005; 64:347.

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 January 2009 by Jill D. 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.