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Sjögren's Syndrome

Author: Shelley Ensz. Scleroderma is highly variable. Read Disclaimer
What is Sjögren's?
Symptoms of Sjogren's
Diagnosis of Sjogren's
Similar & Overlaps
Treatments for Sjögren's
Overview of Treatments
Treatments for Dry Eyes
Treatments for Dry Mouth
Sjogren's Research
Overview of Research
Antibodies
Dry Eyes
Online Support
Personal Stories
Media Stories

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.

"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 About.com
Sjögren's Synddrome. MedicineNet.com
A current perspective on Sjögren's syndrome. Recent developments are unlocking the mystery of the disease process as well as contributing to our ability to define, diagnose, and develop new treatment modalities for patients with this complex disorder. PubMed. J Calif Dent Assoc. 2011 Sep;39(9).
What Is Sjogren's Syndrome? What Causes Sjogren's Syndrome? Sjogren's syndrome (Sjögrens Syndrome) is a chronic disorder of the immune system - a long-term autoimmune disease - in which the patient's white blood cells attack the saliva and tear glands, leading to dry mouth and eyes because the body's tear and saliva production is reduced. Medical News Today. 09/01/11.
Similarities and Differences Between Primary and Secondary Sjögren's Syndrome (SS). A higher frequency of oral symptoms and parotid enlargement and stronger B cell activity (autoantibody production and lymphocyte infiltration) were observed in primary SS. Gabriela Hernández-Molina. JRheum. March 1, 2010.

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.
Symptoms of Sjogren's: Overview
Symptoms: Dry Eyes
Symptoms: Dry Mouth
Symptoms: Dry Vagina
Symptoms: Fatigue
Complication: Liver Involvement
Symptoms: Nervous System

Dermatofibromas
Newborn Heart Problems
Sjogren's and Pregnancy

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.
Overview of Sjogren's Diagnosis
Antibodies
Biomarkers in Saliva and Tears
Salivary Gland Biopsy
Schirmer's Test
Slit Lamp Test
Ultrasonography of Glands

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.
Sjogren's thrombocytopenia. Clinically important immune-mediated cytopenia (or a combination of cytopenias) may be the first manifestation of an occult SS and have only been rarely described with Sjogren's. Kamath V J. (PubMed) Assoc Physicians India. 2011 Feb;59:114-5.
Coexistence of two different types of lymphoma in a patient with Sjögren's syndrome. The usefulness of the PET-CT. Sjögren syndrome is a chronic systemic autoimmune disease in which there is an increased risk of developing non-Hodgkin's lymphoma. Infante De La Torre.(PubMed) JR Rev Esp Med Nucl. 2011 Jul 5.
A Clinical Prediction Rule For Lymphoma Development In Primary And Secondary Sjögren's Syndrome. This study allowed us to create a prediction model which might be useful in improving the follow-up management of patients with primary and secondary Sjogren's (pSS and sSS-SSc). C. Baldini. EULAR 2011 OPO039. Ann Rheum Dis 2011;70(Suppl3):80. (Also see: Scleroderma and Cancer)
Watermelon stomach in a patient with primary Sjögren's syndrome. This is the first report of WS in a patient with primary SS without the presence of coexisting autoimmune disorder. Recognition of this rare, but clinically important, cause of gastrointestinal bleeding may decrease comorbidity in patients with autoimmune disorders including primary Sjögren's syndrome. Krstić M. (PubMed) Vojnosanit Pregl. 2010 Mar;67(3):256-8. (Also see: Watermelon Stomach)
Secondary Sjögren's Syndrome (SS) in Systemic Lupus Erythematosus (SLE) Defines a Distinct Disease Subset. The subset of patients with SLE and SS has a distinct clinical and laboratory phenotype, with a higher frequency of older white women with photosensitivity, oral ulcers, Raynaud's phenomenon, anti-Ro antibodies, and anti-La antibodies and a lower frequency of renal disease, anti-dsDNA antibodies, and anti-RNP antibodies. A. Baer. JRheum. April 1 2010. (Also see: Lupus and Overlapping Diseases)
Comorbidities in Patients with Primary Sjögren's Syndrome (pSS): A Registry-based Case-control Study. The prevalence of several comorbidities was increased among the patients with pSS. J. Horng-Kang. JRheum. April 1 2010.
Anticentromere antibody positive Sjogren's Syndrome: a retrospective descriptive analysis. A subgroup of patients with primary Sjogren's syndrome (SS) and positive anticentromere antibodies (ACA+) were recognized as having features intermediate between SS and systemic sclerosis (SSc). ACA+/SS has a clinical phenotype intermediate between ACA-/SS and SSc and shows little tendency to evolve to SSc. Vasiliki-Kalliopi K Bournia Arthritis Research & Therapy 19 March 2010. (Also see: Sjogren's)
Sjögren's syndrome (SS) -onset lupus (SLE) patients have distinctive clinical manifestations and benign prognosis: A case control study. Different from SLE-only patients, SS/SLE patients have distinctive clinical manifestations and benign prognosis that require less vigorous treatment with glucocorticoids and/or immunosuppressants. D. Xu. Lupus. February 1 2010. (Also see: Lupus and Overlapping Diseases)
Hematologic Manifestations and Predictors of Lymphoma Development in Primary Sjogren's Syndrome: Clinical and Pathophysiologic Aspects. Anemia of chronic disease and hypergammaglobulinemia are common hematologic manifestations at diagnosis and during the course of pSS. Neutropenia and cryoglobulinemia at diagnosis are significantly associated with an increased risk of lymphoma development. Evangelia Baimpa, MD. Medicine. September 2009.
Lung involvement in patients with primary Sjögren's syndrome (pSS): what are the predictors? The presence of hypergammaglobulinemia and lymphopenia, positivity for RF, anti-La and anti-Ro, and impaired (FVC) and/or FEV1 values could be the predictive parameters with a high specificity despite the low sensitivity rates. Smoking history, male gender and age are also risk factors. These parameters may be helpful to distinguish pSS-associated lung involvement from lung disorders unrelated to pSS. Veli Yazisiz. Rheumatology International. October 21, 2009.
Pulmonary Manifestations of Sjögren's Syndrome. The histopathologic patterns of Primary Sjögren's Syndrome associated Interstitial Lung Disease included lung interstitial involvement and small airway involvement or both. Corticosteroid therapy combined with cyclophosphamide was administered with a favorable response in the majority of patients. (Karger) Ju-Hong Shi. Respiration. April 22 2009.
Systemic Autoimmune Diseases (SAD) in Patients with Hepatitis C Virus (HCV) Infection: Characterization of 1020 Cases (The HISPAMEC Registry). In the selected cohort, the SAD most commonly reported in association with chronic HCV infection were Sjögren's syndrome (nearly half the cases), Rheumatoid Arthritis (RA) and Systemic Lupus Erythematosus. M. Ramos-Casals. J. Rheum. April 15, 2009. (Also see: RA, and Lupus).
Hemolytic uremic syndrome (HUS) and pericarditis as early manifestations of primary Sjögren's syndrome (pSS). We describe the unusual case of a pSS patient with the initial presentation of HUS and pericarditis and a fatal progression of pulmonary hemorrhage. (SpringerLink) Hung-An Chen. Clinical Rheumatology. March 5, 2009.
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)
Red Lips Photography by Nolan LaTourelleScleroderma Dental Involvement Scleroderma can cause many dental problems, including small mouth, dry mouth, bone reaborption, receding ligaments, and TMJ. ISN.

Treatments for Sjögren'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.
Treatments of Sjogren's: Overview
Treatments for Dry Eyes
Treatments for Dry Mouth
Treatment for Fatigue
Treatment for Renal Involvement

Sjogren's Research

Overview of Sjogren's Research
Sjogren's Research: Antibodies
Sjogren's Research: Dry Eyes
Overview of Sjogren's Research
Impact of primary Sjögren's syndrome (SS) on smell and taste: effect on quality of life (QoL). Clinically important impairment of chemosensory perception occurred in the SS group compared with age- and gender-matched controls contributing to the reduced health-related QoL that characterized these individuals. U.F. Kamel. Rheumatology Advance Access. September 14 2009.
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.
Pulmonary Manifestations in Sjögren's Syndrome (SS): Correlation Analysis Between Chest Computed Tomographic (CT) Findings and Clinical Subsets with Poor Prognosis in 80 Patients. Cysts are significantly associated with anti-SSB/La seropositivity and clonally derived lymphoproliferative disorder (cLPD). The presence of lung cysts revealed by chest CT might be a prognostic clinical feature, a clue, or a predictor of cLPD in patients with SS. Maiko Watanabe J. Rheum. Dec. 15, 2009.
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)

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.
 
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