$Unique_ID{BRK04224} $Pretitle{} $Title{Sjogren Syndrome} $Subject{Sjogren Syndrome Dacryosialoadenopathia atrophicans Gougerot-Houwer-Sjogren Gougerot-Sjogren Keratoconjunctivitis Sicca Keratoconjunctivitis sicca-xerostomia Secreto-inhibitor-xerodermostenosis Sicca Syndrome Xerostomia Mikulicz Syndrome Fibromyalgia Keratomalacia Ligneous Conjunctivitis Lupus (Systemic Lupus Erythematosus or SLE) Rheumatoid Arthritis} $Volume{} $Log{} Copyright (C) 1992 National Organization for Rare Disorders, Inc. 6: Sjogren Syndrome ** IMPORTANT ** It is possible the main title of the article (Sjogren Syndrome) is not the name you expected. Please check the SYNONYMS listing on the next page to find alternate names and disorder subdivisions covered by this article. Synonyms Dacryosialoadenopathia atrophicans Gougerot-Houwer-Sjogren Gougerot-Sjogren Keratoconjunctivitis Sicca Keratoconjunctivitis sicca-xerostomia Secreto-inhibitor-xerodermostenosis Sicca Syndrome Xerostomia Information on the following diseases can be found in the Related Disorders section of this report: Mikulicz Syndrome Fibromyalgia Keratomalacia Ligneous Conjunctivitis Lupus (Systemic Lupus Erythematosus or SLE) Rheumatoid Arthritis General Discussion ** REMINDER ** The Information contained in the Rare Disease Database is provided for educational purposes only. It should not be used for diagnostic or treatment purposes. If you wish to obtain more information about this disorder, please contact your personal physician and/or the agencies listed in the "Resources" section of this report. Sjogren syndrome is an autoimmune disorder characterized by degeneration of the mucous-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. It is also associated with inflammatory disorders such as arthritis or Lupus. Symptoms Sjogren Syndrome generally has a sudden onset. Primary Sjogren 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 Sjogren 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 Sjogren syndrome. Most patients with Sjogren syndrome have the primary type of Sjogren 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 Sjogren 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 Sjogren 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 Sjogren syndrome should be examined by an ophthalmologist, a physician who specializes in the care and treatment of eyes. Patients with Sjogren 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 Sjogren syndrome is an autoimmune disorder. It has no known cause. Autoimmune disorders are caused when the body's natural defenses (antibodies, lymphocytes, etc.), against invading organisms suddenly begin to attack healthy tissue. People with Sjogren 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 Sjogren syndrome often occurs in patients with rheumatoid arthritis, systemic lupus erythematosus and other connective tissue diseases. Affected Population Sjogren syndrome affects 9 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 suggests that men who show symptoms of HIV infection may develop a syndrome similar to Sjogren's. Related Disorders Symptoms of the following disorders can be similar to those of Sjogren Syndrome. Comparisons may be useful for a differential diagnosis: Mikulicz Syndrome is a benign chronic disorder that causes the enlargement of the tonsils, the saliva glands located near the ear (parotid), the glands beneath the upper jaw bone (submaxillary), glands that produce tears (lacrimal glands), and the salivary glands of the mouth. Symptoms may include dryness of the mouth, difficulty swallowing and tooth decay. There may be absent or decreased tears and blurred vision. (For more information on this disorder, choose "Mikulicz Syndrome" as your search term ion the Rare Disease Database). Keratomalacia is an eye disease caused by a deficiency of vitamin A. Vitamin A (retinol) is found mainly in fish liver oils, liver, egg yolk, cream and butter. The human body stores vitamin A mainly in the liver. Once it is released by the liver, vitamin A is converted to light sensitive pigments in the retina of the eye which is involved in night, day and color vision. Vitamin A also helps to maintain healthy body tissues. A lack of Vitamin A may cause night blindness, abnormal dryness of the inner surface of the eyelid (xerosis) and the transparent covering of the eyes (cornea). This dryness may result in a feeling of grittiness in the eyes and a painful sensitivity to light (photophobia). (For more information on this disorder, choose "Keratomalacia" as your search term in the Rare Disease Database). Ligneous Conjunctivitis is a rare disorder that is characterized by lesions in the mucous producing membranes especially of the eye. This disorder usually presents itself in childhood. Mucous membranes of the voice box (larynx), vocal chords, nose, trachea, wind pipe, vagina, cervix and gums (gingiva) may also be affected. The lesions in the membranes have a "wood- like" (ligneous) texture. They are thick, firm, knotty and tough. The cause of this disorder is not known although there have been some cases that suggest an autosomal recessive genetic inheritance. (For more information on this disorder, choose "Ligneous Conjunctivitis" as your search term in the Rare Disease Database). Fibromyalgia is a chronic muscle disorder characterized by muscle pain throughout much of the body. This may begin gradually or have a sudden onset. Other symptoms include muscle spasms, fatigue, muscle tissue stiffness and unrefreshing (non-restorative) sleep. The exact cause of this disorder is not known. Some patients with Fibromyalgia may have chest pain, headaches, diarrhea, constipation, dryness in the eyes and mouth (Sjogren syndrome), swelling of a tendon (tendinitis), or swelling of the connective tissue surrounding a joint (bursitis). (For more information on this disorder, choose "Fibromyalgia" as your search term in the Rare Disease Database). Lupus (Systemic Lupus Erythematous or SLE) is a multi-system inflammatory disease of the connective tissue of the body. Sjogren syndrome may occur in conjunction with Lupus. Fatigue is an early and frequent symptom. Other symptoms may include fever, swollen glands, skin rash, profound weight loss, headaches, hair loss (alopecia) and water retention (edema). Arthritis, joint and muscle pain occurs in 90 percent of the cases. These symptoms may occur years before the illness is actually diagnosed. The arthritis symptoms come and go and tend to appear most often in either knees, fingers, or wrist joints. There is no bone loss associated with this joint involvement. (For more information on this disorder, choose "Lupus" as your search term in the Rare Disease Database). Rheumatoid Arthritis (RA) is an autoimmune inflammatory disorder. It's cause is unknown. It is characterized by morning stiffness and arthritis mainly in the hands, wrists, knees, feet, shoulders and hips. Once a joint is involved, it may remain painful for weeks, months, and even years. About 25 percent of RA patients also have Sjogren syndrome (secondary). (For more information on this disorder, choose "Rheumatoid Arthritis as your search term in the Rare Disease Database). Therapies: Standard A number of tests are available for the diagnosis of Sjogren syndrome. They include a careful examination of the eyes, including the measurement of tear production; measurement of saliva production after stimulation with lemon juice; X-ray of the glands under the jaw and ears (parotid glands); examination of the cells of the lip to determine if a special type of small white blood cells (lymphocytes) are present in the salivary glands (biopsy); blood tests (including ANA anti-nuclear antibody and Immunoglobulin levels or Ig levels). An ophthalmologist or a rheumatologist should be contacted for testing. Treatment of Sjogren syndrome is dependent on symptoms and usually is much the same as for other autoimmune disorders. No treatment, however, has yet been found to restore the secretions of the glands involved. The insufficient secretions can be replaced by artificial tears in the form of eye drops, artificial saliva which can be used to wet the mouth, and vaginal lubricants. Medications such as corticosteroids, anti-inflammatory drugs or cytoxan may be needed for certain complications. Therapies: Investigational Medical research is seeking to determine the exact cause of this disorder, as well as development of new treatments. The National Institute of Dental Research (NIDR) is conducting studies on several drugs for treatment of Sjogren Syndrome. Patients interested in participating in these studies should ask their physician to contact: Alice Macynski, RN NIH/National Institute of Dental Research (NIDR) 9000 Rockville Pike Bldg. 10, Rm. 1B-21 Bethesda, MD 20892 (301) 496-4371 Bromhexine is a drug used in Europe and Canada for the treatment of Sjogren syndrome. However no clinical trials are underway in the United States. Trials of the drug Pilocarpine for treatment of dry mouth has been suggested by researchers. The drug increases the salivary flow rate in test subjects. The immune suppressive drug, Cyclosporine, is being developed as a special formulation for use as an eye medication with the hope that it may reduce destruction of tear ducts in Sjogren Syndrome. As with any drug, more study is needed to determine the long-term safety and effectiveness of these experimental treatments. This disease entry is based upon medical information available through August 1992. Since NORD's resources are limited, it is not possible to keep every entry in the Rare Disease Database completely current and accurate. Please check with the agencies listed in the Resources section for the most current information about this disorder. Resources For more information on Sjogren Syndrome, please contact: National Organization for Rare Disorders (NORD) P.O. Box 8923 New Fairfield, CT 06812-1783 (203) 746-6518 Sjogren Syndrome Foundation 382 Main St. Port Washington, NY 11050 (516) 767-2866 National Sjogren Syndrome Association 3201 W. Evans Dr. Phoenix, AZ 85023 (602) 993-7227 (800) 395-6772 The Arthritis Foundation 1314 Spring Street NW Atlanta, GA 30309 (404) 872-7100 NIH/National Institute of Dental Research (NIDR) 9000 Rockville Pike Bethesda, MD 20392 (301) 496-4261 NIH/National Arthritis and Musculoskeletal and Skin Diseases Information Clearinghouse Box AMS Bethesda, MD 20892 (301) 495-4484 References MENDELIAN INHERITANCE IN MAN, 9th Ed.: Victor A. McKusick, Editor: Johns Hopkins University Press, 1990. Pp. 1477-1478. CECIL TEXTBOOK OF MEDICINE, 19th Ed.: James B. Wyngaarden, and Lloyd H. Smith, Jr., Editors; W.B. Saunders Co., 1990. Pp. 1535-1537. PRIMARY SJOGREN'S SYNDROME IN MEN. CLINICAL SEROLOGIC, AND IMMUNOGENETIC FEATURES. R. Molina, et al.; Am J Med, (January, 1986, issue 80(1)). Pp. 23-31. TREATMENT OF PRIMARY SJOGREN'S SYNDROME WITH HYDROCHLOROQUINE. R.I. Fox, et al; Am J Med (October 14, 1988, issue 85 (4A)). Pp 62-67. MOLECULAR CHARACTERIZATION OF A MAJOR AUTO-ANTIBODY ASSOCIATED CROSS- REACTIVE IDIOTYPE IN SJOGREN'S SYNDROME. T.J. Kipps, et al.; J Immunol (June 15, 1989, issue 142 (12)). Pp. 4261-4268.