Sjögren's Syndrome

Author: Shelley Ensz. Scleroderma is highly variable. See Types of Scleroderma. Read Disclaimer
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Overview: What is Sjögren's Syndrome?

Overview of Sjogren's Syndrome Video
Video: Overview of Sjogren's Syndrome

Presented by Amanda Thorpe
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Sjögren's is an autoimmune disease that attacks fluid-secreting glands. When it affects the salivary glands, it causes dry mouth, and when it involves tear glands, it results in dry eyes. It can also cause vaginal dryness in females. Thus, the hallmark symptoms of Sjögren's are dry eyes, dry mouth, and dry vagina.

Sjögren's is also a systemic disease, which means that it can affect the skin, lungs, heart, kidneys, muscles, and central nervous system. Sjögren's can also cause fatigue,

People can also have any of the above symptoms separately, due to other conditions, or in overlap with other diseases.

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 ever be diagnosed.

Sjögren's can also occur along with the systemic forms of scleroderma. (Also see: What is Scleroderma? and Types of Scleroderma)

"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

Sjögren's Synddrome.

Epidemiology of primary Sjögren's syndrome (pSS): a systematic review and meta-analysis. Incidence and prevalence rates of pSS vary widely around the world and these results help us better understand the global epidemiology of pSS. PubMed, Ann Rheum Dis, 2015 Nov;74(11):1983-9.

Advances in the understanding and treatment of systemic complications in Sjögren's syndrome. Dryness of the mucosal surfaces is the pivotal, but not only, clinical involvement that characterizes primary Sjögren's syndrome. PubMed, Curr Opin Rheumatol, 2014 Sep;26(5):520-7.

New advances in the classification, pathogenesis and treatment of Sjogren's syndrome. A new set of classification criteria for Sjogren's syndrome has been suggested and significant progress has been made in understanding the activation of innate and adaptive immune responses. PubMed, Curr Opin Rheumatol, 2013 Sep;25(5):623-9.

Sjögren's syndrome, the old and the new. An extensive review the postulated aetiologies, pathogenesis and new insights related to Sjögren's syndrome. Best Pract Res Clin Rheumatol, 2012 Feb;26(1):105-17.


Symptoms of Sjogren's. 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. ISN.

Central Nervous System
Dry Eyes
Dry Mouth
Dry Vagina
Newborn Heart Problems
Sjogren's and Pregnancy


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.

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.

Hyperviscosity in primary Sjögren's syndrome: clinical implications. High viscosity was present in almost half of the patients and was associated with vasculitis and higher activity scores. Wiley Online Library, 10/07/2015.

Identification of lymphoma predictors in patients with primary Sjögren's syndrome: a systematic literature review and meta-analysis. Patients with primary Sjögren syndrome have an increased risk of lymphoma or lymphoproliferative disease compared to the general population and ascertaining relevant and reliable predictors would greatly facilitate the identification of patients at elevated risk for closer monitoring. PubMed, Rheumatol Int, 2014 Jun 5.

Autoimmune Disease Linked to Estrogen Deficiency. Estrogen may have a new purpose in treating or preventing certain types of autoimmune disease, according to a study. News Fix, 08/16/2013. (Also see Causes of Scleroderma: Hormones and Chromosomes)

Signal Hyperintensities (SH) on Brain Magnetic Resonance Imaging in Patients with Primary Sjogren Syndrome (pSS) and Frequent Episodic Tension-type Headache (FETH). Periventricular SH seem to be associated with increased platelet serotonin release in pSS patients with FETH and correlated with disease duration and might be linked with chronic immunoinflammation of low-grade intensity and vasculitis in pSS. PubMed, J Rheumatol, 2013 Jun 1.

The clinical and laboratory characteristics of Sjögren's syndrome (SS) that progresses to systemic lupus erythematosus (SLE). The SS/SLE patients had an earlier age of onset, higher incidences of arthritis, leucopenia, proteinuria and low complement levels, and lower incidences of xerostomia and interstitial lung disease compared with Primary Sjogren's Syndrome patients. PubMed, Int J Rheum Dis, 2013 Apr;16(2):173-7. (Also see Lupus in Overlap)

Overlap of ACA positive systemic sclerosis (SSc) and Sjögren's syndrome (SS): a distinct clinical entity with mild organ involvement but at high risk of lymphoma. This is characterized by a benign SSc clinical course but a high risk of non-Hodgkin's lymphoma. PubMed, Clin Exp Rheumatol, 2013 Jan 18.

Sjögren's syndrome at the crossroad of polyautoimmunity. This study discloses a high prevalence of polyautoimmunity in Sjogren's Syndrome, its associated risk factors and the grouping pattern of such a condition. PubMed, J Autoimmun, 2012 Jun 30. (Also see:Multiple Autoimmune 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. PubMed, Assoc Physicians India, 2011 Feb;59:114-5.

Red Lips Photography by Nolan LaTourelleScleroderma Dental Involvement Scleroderma can cause many dental problems, including small mouth, dry mouth, bone reabsorption, receding ligaments, and TMJ. ISN.


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. ISN.

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

Sjogren's Research

Sjogren's Research encompasses vital topics such as the causes, subtypes, biomarkers, genetics, antibodies, and progression. ISN.

Overview Sjogren's Research: Antibodies

Research Overview

Familial risk of Sjögren's syndrome and co-aggregation of autoimmune diseases in affected families: A nationwide population study. The risk of Sjögren's syndrome and other autoimmune diseases is increased in relatives of people with Sjögren's syndrome, and family factors explain half the phenotypic variance of the disease. PubMed, Arthritis Rheumatol, 05/04/2015.

Evaluation of Germinal Center (GC)-like Structures and B Cell Clonality in Patients with Primary Sjögren Syndrome with and without Lymphoma. GC-like structures seen in minor salivary glands may represent various subtypes of CD21+ infiltrates. A clear association between cellular infiltrates, B cell clonality, and lymphoma development was unable to be detected. PubMed, J Rheumatol, 2014 Oct 1.

Sjögren's syndrome (pSS): still not fully understood disease. Laboratory biomarkers and useful imaging tools for predicting the development of complications associated with Sjogren's. PubMed, Rheumatol Int, 2014 Jul 2.

Implication of Epstein-Barr virus (EBV) infection in disease-specific autoreactive B cell activation in ectopic lymphoid structures of Sjogren's syndrome (SS). Active EBV infection is selectively associated with ectopic lymphoid structures in salivary glands of Sjogren's syndrome and appears to contribute to local growth and differentiation of disease-specific autoreactive B-cells. PubMed, Arthritis Rheumatol, 2014 May 28. (Also see B Cells and T Cells)

Genome-wide association studies in Sjögren's syndrome (SS): What do the genes tell us about disease pathogenesis? Genetic findings are discussed with particular attention paid to the genes identified, the strength of associations, and how the SS-associated genes compare to what has been discovered previously in systemic lupus erythematosus (SLE). PubMed, Autoimmun Rev, 2014 Jul;13(7):756-761. (Also see Causes of Lupus: Genetics)

Early events in Sjögren's Syndrome pathogenesis: The importance of innate immunity in disease initiation. This review explores the relationship between innate immunity and SS pathogenesis prior to overt disease onset and discusses therapeutic strategies to mitigate disease progression. PubMed, Cytokine, 2014 Mar 19.

Genetics of Sjögren's syndrome in the genome-wide association era. Here we review the fundamental scientific principles employed in genome-wide association (GWA) scans as well as the limitations of this tool, and we discuss the application of GWA scans in determining genetic variants at play in complex disease. J Autoimmun, 2012 Jan 28.

Sjogren's Research: Antibodies

Etiopathogenic Role of Surfactant Protein D (SP-D) in the Clinical and Immunological Expression of Primary Sjögren Syndrome (pSS). High SP-D levels were found with severe glandular involvement, hypergammaglobulinemia, leukopenia, extraglandular manifestations, and positive anti-Ro/La antibodies. PubMed, J Rheumatol, 2014 Nov 1.

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.

Online Support

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.

Patient Stories

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...

Go to Sjogren's Syndrome: Symptoms

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