| Sjögren's Syndrome |
| This page was written by Shelley Ensz, and has not yet been medically edited. Scleroderma (SD) affects everyone differently. See Disclaimer. |
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| What is Sjögren's Syndrome? |
Hallmark symptoms of Sjögren's include dry eyes, dry mouth, dry vagina. Sjögren's can also cause fatigue, and it can affect the skin, lungs, heart, kidneys, muscles, and central nervous system. People can also have any of the above symptoms separately, or due to other conditions.
Ninety percent of Sjögren's patients are female. Many Sjögren's patients are undiagnosed, with an average of ten years from onset to diagnosis—for those fortunate enough to be diagnosed at all. |
| Typical diagnostic tests for Sjogren's include Schirmer's eye test, slit-lamp test, tests measuring saliva flow, radiologic salivary scans, and salivary gland biopsy. |
| "Sjögren's may also be referred to as: Dacryosialoadenopathia atrophicans, Gougerot-Houwer-Sjögren, Gougerot-Sjögren, Keratoconjunctivitis Sicca, Keratoconjunctivitis sicca-xerostomia, Secreto-inhibitor-xerodermostenosis, and Sicca Syndrome." NORD |
| Sjögren's Syndrome is a "chronic, systemic inflammatory disorder of unknown cause, characterized by dryness of the mouth, eyes, and other mucous membranes and often associated with rheumatic disorders sharing certain autoimmune features (eg, RA, scleroderma, SLE) and in which lymphocytes infiltrate mucosal and other tissues." Merck Manual |
| Sjögren's Syndrome About.com |
| Sjögren’s Syndrome. Sjögren's (SHOW-grins) syndrome is an autoimmune disease that attacks the exocrine glands (i.e., moisture-producing glands) of the body, specifically the lacrimal and salivary glands. U.S. Pharmacist. Vol. No: 32:3 Posted: 3/21/2007. |
| Sjögren's syndrome (SS) is associated with and not secondary to systemic sclerosis (SSc). SS associated with SSc has the same features as pSS, but SSc seems to be less serious. Moreover, the association of SS and SSc is frequently accompanied by a spreading of autoimmunity. Rheumatology. Vol 46, Number 2. Pp. 321-326. Feb 07. |
| Sjogren's syndrome (SS) in dermatology. SS is a systemic autoimmune disease characterized by dry eyes and dry mouth. The dermatologic manifestations range from drynessness (sicca) and its complications to vasculitis. PubMed. Clin Dermatol. 2006 Sep-Oct;24(5):393-413. |
| Sjögren's Syndrome eMedicine Journal |
| Symptoms of Sjogren's Syndrome |
| Hallmark symptoms of Sjögren's include dry eyes, dry mouth, dry vagina. Sjögren's can also cause fatigue, and it can affect the skin, lungs, heart, kidneys, muscles, and central nervous system. People can also have any of the above symptoms separately, or due to other conditions. |
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| Diagnosis of Sjogren's Syndrome |
| Diagnosis of Sjogren's Syndrome. Typical diagnostic tests for Sjogren's include Schirmer's eye test, slit-lamp test, tests measuring saliva flow, radiologic salivary scans, and salivary gland biopsy. ISN. |
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| Similar and Overlapping Conditions |
| Sjogren's symptoms have been associated with other conditions, including autoimmune or collagen vascular diseases, such as thyroid disease, gastic antral vascular ectasia (watermelon stomach), systemic scleroderma, lupus, Reynold's syndrome, primary biliary cirrhosis, and rheumatoid arthritis. |
| About twenty percent of patients with systemic scleroderma also have Secondary Sjögren's Syndrome. |
| Interstitial Cystitis and Systemic Autoimmune Diseases. Indirect evidence, however, does support a possible autoimmune nature of interstitial cystitis, such as the strong female preponderance and the clinical association between interstitial cystitis and other known autoimmune diseases within patients and families. The strongest association occurs between interstitial cystitis and Sjögren's syndrome. Nat Clin Pract Urol. 2007 Sep;4(9):484-91. (Also see: Interstitial Cystitis) |
| Sjogren's syndrome is associated with and not secondary to systemic sclerosis. SS associated with SSc has the same features as pSS (primary Sjogren's Syndrome), but SSc seems to be less serious. The association of SS and SSc is frequently accompanied by a spreading of autoimmunity. PubMed. Rheumatology (Oxford). 2006 Jul 28. |
| Prevalence of Sicca Symptoms and Sjogren's Syndrome in Systemic Sclerosis: Results of a Prospective Two-Center Series of 133 Patients. The prevalence of sicca symptoms and SS, using international classification criteria for SS, has been found respectively in 68% and 14% of SSc patients. J. Avouac. FRI0318 EULAR 2006. (Also see: Limited Scleroderma) |
 Scleroderma Dental Involvement Scleroderma can cause many dental problems, including small mouth, dry mouth, bone reaborption, receding ligaments, and TMJ. ISN. |
| Systemic sclerosis-associated Sjogren's syndrome and relationship to the limited cutaneous subtype: Results of a prospective study of sicca syndrome in 133 consecutive patients. There was a 68% prevalence of sicca syndrome in this prospective series of SSc patients. PubMed. Arthritis Rheum. 2006 Jun 26;54(7):2243-2249. (Also see: Limited Scleroderma) |
| Association of systemic and thyroid autoimmune diseases. Systemic and thyroid autoimmune diseases often overlap with each other. HT (Hashimoto's thyroiditis) and GD (Graves' disease) may be most common among MCTD, SSc and SS patients. Therefore it is clinically important to screen patients with systemic autoimmune diseases for the co-existence of thyroid disorders. PubMed. Clin Rheumatol. 2005 Oct 25. (Also see: Thyroid Disease, MCTD, and What is Scleroderma?) |
| Dry eyes can cause misery but no tears. Autoimmune diseases such as lupus, rheumatoid arthritis or Sjogren's syndrome are the leading causes of the aqueous form, said David Sullivan, a senior scientist at Schepens. The Boston Globe 2/24/2004. (Also see: Lupus and Rheumatoid Arthritis) |
| AUTOIMMUNE: Identification of masqueraders of autoimmune disease in the office. There are several rheumatologic and autoimmune disorders that can masquerade as allergic disease. These conditions include rheumatoid and juvenile arthritis, Sjogren's syndrome, systemic lupus erythematosus, Behcet's and antiphospholipid syndromes, systemic sclerosis, vasculitis, sarcoidosis, chronic fatigue syndrome, and fibromyalgia. PubMed. Allergy Asthma Proc. 2003 Nov-Dec;24(6):421-9. (Also see: Rheumatoid Arthritis, Lupus, Behcet's, Antiphospholipid, Systemic Sclerosis (Scleroderma), Vasculitis, Sarcoiditis, Fibromyalgia and CFS) |
| COLLAGEN DISEASES: Significance of the cardiovascular function tests in patients with collagen diseases, especially for SSc (systemic scleroderma). The purpose of this study is to evaluate the cardiovascular function in patients with incomplete type of SSc (SSSD; Scleroderma Sjogren syndrome associated Spectrum Disorders), SSc (Systemic Scleroderma) and SLE (Systemic Lupus Erythematosus). Iischemic myocardial damage, left ventricular hypertrophy or QT interval prolongation was observed in 33% cases of scleroderma associated and 32% cases of SSc, respectively. Parasympathetic disorders were observed in approximately 20% cases of SSSD, SSc and SLE. PubMed. Rinsho Byori. 2004 May;52(5):401-5. (Also see: Cardiac Involvement) |
| GAVE: Gastric Antral Vascular Ectasia (Watermelon Stomach) in a Patient with Sjögren's Syndrome. Gastric antral vascular ectasia (GAVE), a rare yet treatable cause of upper gastrointestinal bleeding, has been described in a variety of autoimmune diseases. After several fulguration therapies with argon-plasma coagulator, the mucosal lesions improved and her hemoglobin levels returned to normal. J Rheumatol NO. 5 MAY 2003;30:1090-2. (Also see: Watermelon Stomach) |
| REYNOLD's SYNDROME: An Association to Remember. This syndrome constitutes the coexistence of two immunogenetically determined disorders: systemic scleroderma (CREST syndrome) and primary biliary cirrhosis (PBC), and it seems to be a favourable factor for liver disease progression. Association to Sjögren syndrome (SS) was reported. R. M. Morlà. AB0105 EULAR 2004. (Also see: CREST and Liver Involvement) |
| Triad of thymoma, myasthenia gravis and pure red cell aplasia combined with Sjogren's syndrome. The case is the first to be reported with Sjogren's syndrome and the triad of thymoma, myasthenia gravis and pure red cell aplasia. PubMed. Jpn J Thorac Cardiovasc Surg. 2004 Jul;52(7):345-8. (Also see: Myasthenia Gravis) |
| THYROID: Presence of systemic autoimmune disorders in patients with autoimmune thyroid diseases (ATD). ANA (antinuclear antibodies) are detected in 1/3 of patients with autoimmune thyroid disease (ATD). Anti-dsDNA, anti-Ro, and aCL can also be found in ANA positive patients with ATD. SS (Sjogren's Syndrome) occurs in about 1/10 of ANA positive patients with ATD. PubMed. Ann Rheum Dis. 2004 Sep;63(9):1159-61. (Also see: Thyroid Disease and Antibodies) |
| THYROID: Evaluation of salivary gland function in women with autoimmune thyroid diseases. AITD (autoimmune thyroid diseases) may be associated with disturbances in salivary secretion which depends on thyroid hormones production. It can be suggested that autoimmunological processes within salivary glands may influence their function. PubMed. Wiad Lek. 2003;56(9-10):412-8. (Also see: Thyroid Disease) |
| Treatments for Sjögren's |
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| Overview of Treatments for Sjogren's |
| Treatments for Sjogren's may consist of therapies aimed at relieving specific symptoms, such as dry eyes or dry mouth, or targeted at trying to control the underlying disease process. |
| Clinical Trials: Sjögren's Syndrome CenterWatch |
| Dry.Org - Internet Resources for Sjögren's Syndrome Dry.Org |
| Reduction of fatigue in Sjögren syndrome (SS) with rituximab: results of a randomised, double-blind, placebo-controlled pilot study. This is the first double blind study of rituximab in primary SS to show benefit; further studies are justified. Dass S. (PubMed) Ann Rheum Dis. 2008 Nov;67(11):1541-4. |
| Renal Involvement and Followup of 130 Patients with Primary Sjögren's Syndrome (pSS). The combination of corticosteroids and immunosuppressors significantly improves the renal function of patients with pSS. J Rheumatol 2008;35:278-84. |
Lack of efficacy of etanercept in Sjögren’s syndrome correlates with failed suppression of TNF{alpha} and systemic immune activation. Etanercept is an ineffective therapeutic agent in pSS consistent with the absence of suppression of TNF and other indicators of immune activation. Ann Rheum Dis. 15 January 2008. |
| A case of systemic sclerosis and sjogren's syndrome with cardiac tamponade due to steroid-responsive pericarditis. Pericarditis due to SSc has been reported unresponsive to steroid therapy, but several cases of steroid responsive pericarditis due to SSc or SjS have been reported. PubMed. Arerugi. 2006 Jul;55(7):827-31. (Also see: Cardiac Involvement) |
| Treatments for Dry Eyes |
| Dry Eyes: Artificial Tears, Dry Eyes, Tears Naturale, Refresh, Refresh Plus, HypoTears, Lacrisert artificial tear inserts. |
| Lacrisert. Prescription-only, once-daily, preservative-free Lacrisert® lubricates and protects eyes all day, providing continuous relief from moderate to severe dry eye symptoms all day. In some patients, it may also help slow, stop or reverse further damage to your corneal surface. Lacrisert. |
| Gen-Teal Gel by CIBA Vision is an over-the-counter gel for dry eyes. It is best used at night, or along with liquid drops, as it can cause blurriness. |
| Management of Ocular Surface Inflammation in Sjogren Syndrome. Anti-inflammatory therapy in primary Sjogren syndrome significantly improved subjective symptoms and objective ocular signs; however, we did not find evidence that anti-inflammatory treatment increases tear production in patients with Sjogren syndrome. Cornea. 26 Supplement 1:S13-S15, October 2007. |
| Current treatments of xerophthalmia in Sjogren's syndrome. The development of topical cyclosporine and other immunomodulating agents is the most relevant progress in the treatment of keratoconjunctivitis sicca in Sjogren's syndrome. PubMed. Rev Med Interne. 2004 May;25(5):376-382. |
| Therapeutic effect of cevimeline on dry eye in patients with Sjogren's syndrome: a randomized, double-blind clinical study. These results indicate that cevimeline, 20 mg three times daily, is safe and effective in improving symptoms of dry eye in patients with SS. Additional studies, with larger patient populations, are needed to further assess the effectiveness of cevimeline for dry eye. PubMed. Am J Ophthalmol. 2004 Jul;138(1):6-17. |
| Treatments for Dry Mouth |
| Dry Mouth: Biotene products such as toothpaste, mouthwash, and gel; Salivert, Optimoist, Saliva Substitute, Moi-Stir, and Salix Lozenges. |
| Dental Involvement ISN. |
| Evoxac was approved in the U.S. in January 2000 for treatment of dry mouth in patients with Sjögren's. CenterWatch |
| Green tea may delay onset of type 1 diabetes. A powerful antioxidant in green tea may prevent or delay the onset of type 1 diabetes. They found it also worked well in their original disease focus, as it reduced the severity and delayed onset of salivary gland damage associated with Sjogren's syndrome, which has no known cure. (GEN) EurekAlert!. 10/23/08. (Also see: Diabetes) |
| Green tea may help prevent autoimmune diseases. Green tea may help protect against autoimmune disease. Researchers studied an animal model for type I diabetes and primary Sjogren's Syndrome. Further study could help determine green tea's protective role in other autoimmune diseases, including lupus, psoriasis, scleroderma and rheumatoid arthritis. EurekAlert! 04/19/07. (Also see: Diabetes and Alternative Therapies) |
| Gene Therapy Rebuilds Bone, Tissue in Dental Patients. Clinical human gene transfer studies for head and neck cancer treatment are already underway. Experts in the dental application of gene therapy say patients with these conditions will be among the first to undergo gene therapy to repair damaged salivary glands due to cancer radiation treatment or because of the autoimmune disease, Sjogren's syndrome. Expired article from U.S. Newswire. 10/21/03. |
| Sjogren's Research |
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| Overview of Sjogren's Research |
| Sjogren's syndrome. Hypofunction rather than destruction of these cells is now regarded as the main mechanism of secretory failure in Sjogren's syndrome. PubMed. Best Pract Res Clin Rheumatol. 2004 Jun;18(3):313-29. |
| Gene-altered mouse is model for rare autoimmune syndrome. By knocking out a single gene in mice, immunologists at Duke University Medical Center have mimicked a little-understood autoimmune disorder in humans. According to researchers, the achievement not only offers insight into Sjögren's syndrome, but into the general developmental machinery of the immune system. EurekAlert! 10-19-04. |
| Sjogren's Research: Antibodies |
| Sjögren's Antibodies (Anti-SS-A/Anti-SS-B). SS-A(Ro) is found in 60% to 70% of patients with Sjögren's syndrome and 30% to 40% of patients with SLE. SS-B(La) is found in 50% to 60% of Sjögren's syndrome and 10% to 15% of SLE. LabCorp. |
| Epithelial CXCR3-B Regulates Chemokines Bioavailability in Normal, but Not in Sjogren's Syndrome, Salivary Glands. Sjogren's syndrome (SS) is an autoimmune disease characterized by a mononuclear cell infiltrate of activated T cells around the duct in the salivary gland. Epithelial CXCR3 may play a critical role in the pathogenesis of SS and its agonists may be potential therapeutic targets. PubMed, J Immunol. 2006 Feb 15;176(4):2581-9. |
| Yale scientists identify structure for RNA quality control. A report by Yale scientists in the journal Cell sheds new light on how the protein Ro, a major autoantigen in patients with autoimmune disease, recognizes misfolded RNAs, creating a RNA quality control system for cells. Medical News Today. 06/02/05. (Also see: Lupus Antibodies) |
| Sjogren's syndrome. The salivary and lacrimal glands from patients with SS harbor unique and highly selected T- and B-cell populations. B-cell activation may also provide a basis for lymphoma development observed in up to 5% of the patients with SS. PubMed. Eur J Oral Sci. 2005 Apr;113(2):101-13. |
| Sjogren's Research: Brain Involvement |
| An autopsied case of Sjogren's syndrome with massive necrotic and demyelinating lesions of the cerebellar white matter. The combination of Sjogren's syndrome, granulomatous angitis, and foci of perivenous demyelination suggests that an autoimmune mechanism played an important role in causing the necrotic lesions in the cerebellar white matter in this case. PubMed. J Neurol Sci. 2004 Oct 15;225(1-2):143-8. (Also see: Scleroderma Brain Involvement) |
| Sjogren's Research: Causes |
| Specific immunotherapy-induced Sjogren's syndrome. This is the first case to be reported of Sjogren's syndrome following SIT (Allergen-specific immunotherapy). Patients undergoing SIT must be carefully followed up for the development of autoimmunity and an autoimmune disease. PubMed. Rheumatol Int. 2005 Jun 17. |
| Hepatitis C virus, Sjogren's syndrome and B-cell lymphoma: linking infection, autoimmunity and cancer. Viruses have been proposed as possible etiologic or triggering agents of systemic autoimmune diseases, with hepatitis C being one most frequently associated with autoimmune features and with systemic autoimmunes such as mixed cryoglobulinemia or Sjogren's Syndrome. Association between HCV infection and hematologic malignancies, mainly non-Hodgkin's lymphoma, is supported by several studies. PubMed. Autoimmun Rev. 2005 Jan;4(1):8-15. (Also see: Liver Involvement, Cancer and Scleroderma, and Causes of Scleroderma: Infection) |
| Sjogren's Research: Dry Eyes |
| Antibody Protein Array Analysis of the Tear Film Cytokines. Preliminary results using tears collected from patients with Sjogren's syndrome reveal methodological advantages of protein array technology and support the concept that autoimmune-mediated dry eye disease has an inflammatory component. Li, Shimin PhD. Optometry & Vision Science. August 2008. (Also see: Autoimmune Eye Diseases) |
| The role of the lacrimal functional unit in the pathophysiology of dry eye. Tear secretion is controlled by the lacrimal functional unit consisting of the ocular surface (cornea, conjunctiva, accessory lacrimal glands, and meibomian glands), the main lacrimal gland and the interconnecting innervation. If any portion of this functional unit is compromised, lacrimal gland support to the ocular surface is impeded. PubMed. Exp Eye Res. 2004 Mar;78(3):409-16. |
| Online Support for Sjögren's |
| SWA Invitation Do you have a Sjögren's web site? If so, you are invited join the free international Scleroderma Webmaster's Association. |
| Sjögren's Support Email List (TalkSjo) provides support and sociability for Sjögren's patients worldwide. Dry.Org |
| Sjögren's Syndrome Medical Information List is a moderated list subscribed to by hundreds of Sjögren's patients and professionals worldwide. The main focus of the list is medical information and anything that does not have to do specifically with Sjögren's does not get posted. |
| Sjögren's Email Support Lists on Yahoo Groups |
| Sjögren's Patient Stories |
| Submit your Sjögren's and/or Scleroderma Story (in any language) for inclusion on SCLERO.ORG. It's as easy as writing an email, using our simple online form. ISN. |
| Annette G: MCTD, Systemic Scleroderma, Sjogren's, Chronic Kidney Disease, CFS, Fibromyalgia, Trigeminal Neuralgia, TMJ People think that because I am a nurse I have all the answers. Well guess what? I don't... |
| (Italiano) Antea: Sindrome di Sjogren e il Lupus Eritematoso Sistemico Sono Angela dalla provincia di pesaro, ho 29 anni e da ieri ho scoperto di avere la Sindrome di Sjogren... |
| More Sjogren's Personal Stories.. . |
| Media Stories, Misc. |
| Women with little-known disease work to raise awareness. Dana Moreno and Connie Roberts have made it their mission to educate doctors - and everyone else - about Sjogren's Syndrome, an autoimmune deficiency that debilitates through myriad avenues: the central nervous system first, then on to major organs and the lymphatic system. St. Petersburg Times, Hernando. 03/22/07. |
| More than just dry eyes. Sjogren's syndrome is often misdiagnosed. It may occur alone or with a connective tissue disease, such as rheumatoid arthritis, lupus or scleroderma. It affects the entire body, ranging from mild to disabling. Deseret Morning News. 03/04/07. |
| International Group Tackles Sjögren's Syndrome (SS). The Sjögren’s International Collaborative Clinical Alliance is working toward establishing standardized diagnostic criteria for SS and building an international registry of clinical data and biospecimens that are available to scientists all over the world. UCSF Today. 07/19/06. |
| Sjogren's Syndrome - an often underdiagnosed condition. Sjogren's syndrome occurs by itself or can and often does occur with other autoimmune diseases such as lupus, rheumatoid arthritis, scleroderma, and other lesser known rheumatic autoimmune diseases. Irishhealth.com 01-04-06. |
