Sjögren Syndrome

Sjögren Syndrome

National Organization for Rare Disorders, Inc.

Important

It is possible that the main title of the report Sjögren Syndrome is not the name you expected. Please check the synonyms listing to find the alternate name(s) and disorder subdivision(s) covered by this report.

Synonyms

  • Dacryosialoadenopathia atrophicans
  • Gougerot-Houwer-Sjogren
  • Gougerot-Sjogren
  • Keratoconjunctivitis Sicca
  • Keratoconjunctivitis sicca-xerostomia
  • Secreto-inhibitor-xerodermostenosis
  • Sicca Syndrome

Disorder Subdivisions

  • None

General Discussion

Sjögren syndrome is an autoimmune disorder characterized by degeneration of the mucus-secreting glands, particularly the tear ducts of the eyes (lacrimal) and saliva glands of the mouth. Autoimmune disorders are caused when the body's natural defenses (antibodies, lymphocytes, etc.) against invading organisms suddenly begin to attack healthy tissue. Sjögren syndrome is also associated with inflammatory disorders such as arthritis or lupus.

Symptoms

Sjögren Syndrome generally has a sudden onset. Primary Sjögren Syndrome is characterized by inflammation of the cornea of the eyes and the delicate membranes that line the eyelids (keratoconjunctivitis) due to insufficient tear production, and dryness of the mouth (sicca xerostemia) due to lack of saliva from the salivary glands. In secondary Sjögren Syndrome, dry eyes and/or mouth may occur with diseases of the tissue that holds together and supports different structures of the body (connective tissue disease). Most often rheumatoid arthritis (RA), Lupus or other autoimmune diseases are present with secondary Sjögren Syndrome.



Most patients with Sjögren Syndrome have the primary type of Sjögren Syndrome. The onset of symptoms is usually sudden. Decreased production of saliva and the resulting dry mouth make chewing and swallowing food difficult. The lack of saliva causes pieces of food to stick to the cheeks, gums and throat. Teeth decay easily, leading to cavities (dental caries), inflammation of the gums (gingivitis) and advanced gum disease (pyorrhea).



As the tear ducts of the eyes (lacrimal glands) waste away (atrophy), the amount of tears produced decreases, causing a feeling of grittiness and burning in the eyes. The eyelids may stick together, glands under the jaw may be swollen and painful, and gastrointestinal symptoms may occur.



Dryness may extend to the skin and to the mucous membranes lining the nose, throat and vagina. Muscle pain and weakness may also occur (Fibromyalgia).



In secondary Sjögren Syndrome, patients may experience arthritis, rash (palpable purpura) on the lower extremities, and light sensitive rashes (photosensitive dermatitis) on the face, arms and other exposed areas. Fever and neurologic symptoms may occur.



Patients with systemic Sjögren Syndrome (symptoms in addition to the eyes and mouth) usually have blood tests that are positive for certain antibodies (anti-nuclear antibodies to Ro and La antigens). Antibodies are substances made by the body that defend the body against bacteria, viruses, or other foreign invaders (antigens).



All patients suspected of having Sjögren Syndrome should be examined by an ophthalmologist, a physician who specializes in the care and treatment of eyes. Patients with Sjögren Syndrome who have positive blood tests for anti- Ro antibodies should be evaluated by a physician who specializes in the care and treatment of inflammatory diseases (rheumatologist) for evidence of disease outside of the eyes and mouth (extra-glandular involvement).

Causes

Sjögren Syndrome is an autoimmune disorder. It has no known cause. Autoimmune disorders are caused when the body's natural defenses (e.g., antibodies) against "foreign" or invading organisms begin to attack healthy tissue for unknown reasons.



People with Sjögren Syndrome often have a genetic predisposition (HLA- DR3). A genetic predisposition means that a person may carry a gene for a disease but it may not be expressed unless something in the environment triggers the disease. Secondary Sjögren Syndrome often occurs in patients with rheumatoid arthritis, systemic lupus erythematosus and other connective tissue diseases.



Alpha fodrin is an antigen that is the target of humoral or cell-mediated immune response (autoantigen) and is believed to be a cause of Sjögren Syndrome in some cases. Fodrin is a protein found in internal cell structures (cytoskeleton). It binds a muscle protein (actin) and may also be involved in secretion. It is broken down when it is exposed to certain enzymes that degrade protein (proteases). These enzymes become activated during the process of programmed cell death (apoptosis).

Affected Populations

Sjögren Syndrome affects approximately nine females to every male. Ninety percent of women with the disorder have already gone through menopause (post-menopausal), although symptoms may be apparent at an earlier age. Recent data suggest that men who show symptoms of HIV infection may develop a syndrome similar to Sjögren Syndrome. Although the exact figure is unknown, one estimate suggests that one to two million people may have Sjögren Syndrome in the United States. Sjögren Syndrome is believed to be the second most common autoimmune connective tissue disorder.

Standard Therapies

Diagnosis

A number of tests are available for the diagnosis of Sjögren Syndrome. They may include a careful examination of the eyes, including measurement of tear production; evaluation of salivary gland secretion; X-ray imaging of the salivary glands; and removal and microscopic examination of small samples of tissue from the salivary glands (biopsy).



Diagnostic assessment may also require blood studies to measure the levels of red and white blood cells and platelets; testing to measure the rate at which red blood cells settle in a tube of unclotted blood (erythrocyte sedimentation rate [ESR]), potentially providing a nonspecific indicator of inflammation; and studies to detect abnormal antibodies that may be highly specific to Sjögren Syndrome or characteristic of other autoimmune disorders.



Treatment

The treatment of Sjögren Syndrome is directed toward the specific symptoms and physical findings that are present in each individual. For example, symptomatic measures may include the use of artificial tears in the form of eye drops to alleviate dryness of the eyes, artificial saliva to help moisturize the mouth, and vaginal lubricants to replace insufficient secretions. Administration of certain medications such as corticosteroids, anti-inflammatory drugs, or immunosuppressive agents (e.g., Cytoxan) may be needed for certain extra-glandular manifestations potentially associated with Sjögren Syndrome.



The drug Salagen has been approved by the Food and Drug Administration (FDA) for the treatment of dry mouth (xerostomia) and dry eyes associated with Sjögren Syndrome. Salagen Tablets have been shown to stimulate secretions of certain glands (e.g., certain salivary glands) to increase their activity. Salagen Tablets are manufactured by MGI Pharma. Oral pilocarpine is the active ingredient of Salagen Tablets.



In addition, the FDA has approved the orphan drug Evoxac (cevimeline) for the treatment of dry mouth associated with Sjögren Syndrome. The medication is manufactured by SnowBrand Pharmaceuticals of Rockville, Maryland, and is marketed by Daiichi in the United States.

Investigational Therapies

Information on current clinical trials is posted on the Internet at www.clinicaltrials.gov. All studies receiving U.S. government funding, and some supported by private industry, are posted on this government website.



For information about clinical trials being conducted at the National Institutes of Health (NIH) in Bethesda, MD, contact the NIH Patient Recruitment Office:



Tollfree: (800) 411-1222

TTY: (866) 411-1010

Email: prpl@cc.nih.gov



For information about clinical trials sponsored by private sources, contact:

www.centerwatch.com



Current studies include a natural history of salivary gland dysfunction and Sjögren's syndrome, sponsored by the National Institute of Dental and Craniofacial Resaerch (NIDCR), and a studied sponsored by Allergan Pharmaceuticals of the drug Ophthalmic Emulsion for treating patients with moderate or severe dry eye syndrome. Information on these studies is available at www.clinicaltrials.gov.



Bromhexine is a drug used in Europe and Canada for the treatment of Sjögren Syndrome. However, at this time, no clinical trials are underway in the United States.



The immunosuppressive drug, cyclosporin A, is being developed as a special formulation for use as an eye medication with the hope that it may reduce destruction of the tear ducts in individuals with Sjögren Syndrome. Recent studies have shown that cyclosporin A significantly reduced symptoms associated with dry eye syndrome. Further research is needed to determine the long-term safety and effectiveness of this medication as a treatment for dry eyes associated with Sjögren Syndrome.



Scientists have studied OcuNex ophthalmic solution for severe dry eyes associated with Sjögren Syndrome.



Amarillo Biosciences is investigating the use of low dose oral interferon alpha (IFNalpha) as a treatment for Sjögren Syndrome. The drug entered Phase III clinical trials in 2001. More studies are needed to determine the long-term safety and effectiveness of this therapy as a treatment for individuals with Sjögren Syndrome.

References

TEXTBOOKS

McKusick VA., ed. Mendelian Inheritance in Man. Baltimore. MD: The Johns Hopkins University Press; Entry No: 270150, 1994:2195-96.



Thoene JG., ed. Physicians' Guide to Rare Diseases. Montvale, NJ: Dowden Publishing Company Inc; 1995:783-84.



Bennett JC, Plum F., eds. Cecil Textbook of Medicine. 20th ed. Philadelphia, PA: W.B. Saunders Co; 1996:1488-90.



JOURNAL ARTICLES

Stevenson D, et al., Efficacy and safety of cyclosporin A ophthalmic emulsion in the treatment of moderate-to-severe dry eye disease: a dose-ranging, randomized trial. The Cyclosporin A Phase 2 study. Ophthalmology. 2000;107:967-74.



Sall K, et al., Two multicenter, randomized studies of the efficacy and safety of cyclosporin ophthalmic emulsion in moderate to severe dry eye disease. CsA Phase 3 Study Group. Ophthalmology. 2000;107:631-9.



Ship JA, et al., Treatment of primary Sjögren's syndrome with low-dose natural human interferon-alpha administered by the oral mucosa route: a phase II clinical trial. IFN Protocol Study Group. J Interferon Cytokine Res. 1999;19:943-51.



Bell M, et al., Sjögren's syndrome: a critical review of clinical management. J Rheumatol. 1999;26:2051-61.



Nusair S, et al., The use of oral pilocarpine in xerostomia and Sjögren's syndrome. Semin Arthritis Rheum. 1999; 28:360-7.



Papas AS, et al., Oral pilocarpine for symptomatic relief of dry mouth and dry eyes in patients with Sjögren's syndrome. Adv Exp Med Biol. 1998;438:973-8.



Haneji N, et al., Identification of alpha-fodrin as a candidate autoantigen in primary Sjögren's syndrome. Science. 1997;276:604-7.



Simmons TJ, et al., Molecular characterization of a major auto-antibody associated cross-reactive idiotype in Sjögren's syndrome. J Immunol. 1989;142:4261-8.



Fox RI, et al., Treatment of primary Sjögren's syndrome with hydrochloroquine. Am J Med. 1988;85:62-7.



Molina R, et al., Primary Sjögren's syndrome in men. Clinical, serologic, and immunogenetic features. Am J Med. 1986;80:23-31.

Resources

American Autoimmune Related Diseases Association, Inc.

22100 Gratiot Ave.

Eastpointe, MI 48021

Tel: (586)776-3900

Fax: (586)776-3903

Tel: (800)598-4668

Email: aarda@aarda.org

Internet: http://www.aarda.org/



Sjogren's Syndrome Foundation, Inc.

6707 Democracy Blvd

Suite 325

Bethesda, MD 20817

Tel: (301)530-4420

Fax: (301)530-4415

Tel: (800)475-6473

Email: tms@sjogrens.org

Internet: http://www.sjogrens.org



Arthritis Foundation

1330 West Peachtree Street, Suite 100

Atlanta, GA 30309

USA

Tel: (404)872-7100

Tel: (800)283-7800

Email: arthritisfoundation@arthritis.org

Internet: http://www.arthritis.org



NIH/National Institute of Arthritis and Musculoskeletal and Skin Diseases

Information Clearinghouse

One AMS Circle

Bethesda, MD 20892-3675

USA

Tel: (301)495-4484

Fax: (301)718-6366

Tel: (877)226-4267

TDD: (301)565-2966

Email: NIAMSinfo@mail.nih.gov

Internet: http://www.niams.nih.gov/



NIH/National Eye Institute

31 Center Dr

MSC 2510

Bethesda, MD 20892-2510

United States

Tel: (301)496-5248

Fax: (301)402-1065

Email: 2020@nei.nih.gov

Internet: http://www.nei.nih.gov/



NIH/National Institute of Dental and Craniofacial Research

Building 31, Room 2C39

31 Center Drive, MSC 2290

Bethesda, MD 20892

USA

Tel: (301)496-4261

Fax: (301)480-4098

Tel: (866)232-4528

Email: nidcrinfo@mail.nih.gov

Internet: http://www.nidcr.nih.gov/



National Sjogren's Syndrome

Box 147

Svedala, 233 23

Sweden

Tel: 046255925

Email: harry.paulnitz@telia.com

Internet: http://www.sjogrensyndrom.se



Sjögren's Syndrome Association

3155 Rue Hochelaga, Suite 001

Montreal

Quebec, H1W 1G4

Canada

Tel: 5149343666

Fax: 5149341241

Tel: 8779343666

Email: sjogren.montreal@qc.aira.com

Internet: http://www.sjogrens.ca/



International Painful Bladder Foundation

Burgemeester Le Fèvre de Montignylaan 73

Rotterdam, 3055 NA

The Netherlands

Tel: 31104613330

Fax: 31104613330

Email: info@painful-bladder.org

Internet: http://www.painful-bladder.org



CNS Vasculitis Foundation

9930 Morningfield

San Antonio, TX 78250-3743

USA

Tel: (210)523-8234

Email: info@cnsvf.org

Internet: http://www.cnsvf.org



Genetic and Rare Diseases (GARD) Information Center

PO Box 8126

Gaithersburg, MD 20898-8126

Tel: (301)251-4925

Fax: (301)251-4911

Tel: (888)205-2311

TDD: (888)205-3223

Internet: http://rarediseases.info.nih.gov/GARD/



Lupus Society of Alberta

Suite 200, 1301 - 8 St. SW

Calgary Alberta, T2R 1B7

Canada

Tel: 4032287956

Fax: 4032287853

Tel: 8882429182

Email: lupuslsa@shaw.ca

Internet: http://www.lupus.ab.ca



Center for Peripheral Neuropathy

University of Chicago

5841 South Maryland Ave, MC 2030

Chicago, IL 60637

Tel: (773)702-5659

Fax: (773)702-5577

Internet: http://peripheralneuropathycenter.uchicago.edu/



Madisons Foundation

PO Box 241956

Los Angeles, CA 90024

Tel: (310)264-0826

Fax: (310)264-4766

Email: getinfo@madisonsfoundation.org

Internet: http://www.madisonsfoundation.org



Autoimmune Information Network, Inc.

PO Box 4121

Brick, NJ 08723

Fax: (732)543-7285

Email: autoimmunehelp@aol.com



International Scleroderma Network

7455 France Ave So #266

Edina, MN 55435-4702

Tel: (952)583-5735

Tel: (800)564-7099

Email: isn@sclero.org

Internet: http://www.sclero.org



European Society for Immunodeficiencies

1-3 rue de Chantepoulet

Geneva, CH 1211

Switzerland

Tel: 410229080484

Fax: 41229069140

Email: esid@kenes.com

Internet: http://www.esid.org



AutoImmunity Community

Email: moderator@autoimmunitycommunity.org

Internet: http://www.autoimmunitycommunity.org



Cogan's Contact Network

PO Box 145

Freehold, NJ 07728-0145

USA

Tel: (732)409-1031

Internet: http://www.coganssyndrome.info/



For a Complete Report

This is an abstract of a report from the National Organization for Rare Disorders, Inc.® (NORD). Cigna members can access the complete report by logging into myCigna.com. For non-Cigna members, a copy of the complete report can be obtained for a small fee by visiting the NORD website. The complete report contains additional information including symptoms, causes, affected population, related disorders, standard and investigational treatments (if available), and references from medical literature. For a full-text version of this topic, see http://www.rarediseases.org/search/rdblist.html.

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