• Sjogren's Syndrome

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

    Pronounced: show-GRENS
    En Español (Spanish Version)


    Definition | Causes | Risk Factors | Symptoms | Diagnosis | Treatment | Prevention


    Definition

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

    Salivary Glands

    Nucleus factsheet image
    Copyright © Nucleus Medical Media, Inc.

    Causes

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

    • Viral infections
    • Environmental factors
    • 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
    • Certain gene markers

    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 pain, swelling, and stiffness
    • 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

    The doctor will ask about your symptoms and medical history. A physical exam will be done. You may be referred to a specialist.

    Your bodily fluids and tissues may be tested. This can be done with:

    • Blood tests
    • Urine tests
    • Lip biopsy

    Your eyes may be tested. This can be done with:

    • Schirmer test
    • Slit-lap examination

    Images may also be taken of your bodily structures. This can be done with a chest x-ray.

    Treatment

    There is no cure for Sjogren's. No treatment can restore the ability of the glands to produce moisture. 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 unscented moisturizers to help relieve dry skin.

    This condition is generally benign. However, people with severe cases are at increased risk for developing non-Hodgkin 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:

    Health Canada

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

    References:

    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.

    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, 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. American College of Rheumatology website. Available at: http://www.rheumatology.org/Practice/Clinical/Patients/Diseases_And_Conditions/Sj%C3%B6gren_s_Syndrome. Updated July 2012. Accessed August 15, 2013.

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

    What you need to know about Sjogren's syndrome. Cleveland Clinic website. Available at: http://www.clevelandclinic.org/health/health-info/docs/0200/0220.asp. Accessed August 15, 2013.

    Last reviewed August 2013 by Rosalyn Carson-DeWitt, MD; Michael Woods, 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.

  • Sjogren's Syndrome

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

    Pronounced: show-GRENS
    En Español (Spanish Version)


    Definition | Causes | Risk Factors | Symptoms | Diagnosis | Treatment | Prevention


    Definition

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

    Salivary Glands

    Nucleus factsheet image
    Copyright © Nucleus Medical Media, Inc.

    Causes

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

    • Viral infections
    • Environmental factors
    • 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
    • Certain gene markers

    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 pain, swelling, and stiffness
    • 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

    The doctor will ask about your symptoms and medical history. A physical exam will be done. You may be referred to a specialist.

    Your bodily fluids and tissues may be tested. This can be done with:

    • Blood tests
    • Urine tests
    • Lip biopsy

    Your eyes may be tested. This can be done with:

    • Schirmer test
    • Slit-lap examination

    Images may also be taken of your bodily structures. This can be done with a chest x-ray.

    Treatment

    There is no cure for Sjogren's. No treatment can restore the ability of the glands to produce moisture. 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 unscented moisturizers to help relieve dry skin.

    This condition is generally benign. However, people with severe cases are at increased risk for developing non-Hodgkin 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:

    Health Canada

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

    References:

    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.

    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, 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. American College of Rheumatology website. Available at: http://www.rheumatology.org/Practice/Clinical/Patients/Diseases_And_Conditions/Sj%C3%B6gren_s_Syndrome. Updated July 2012. Accessed August 15, 2013.

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

    What you need to know about Sjogren's syndrome. Cleveland Clinic website. Available at: http://www.clevelandclinic.org/health/health-info/docs/0200/0220.asp. Accessed August 15, 2013.

    Last reviewed August 2013 by Rosalyn Carson-DeWitt, MD; Michael Woods, 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.