Sjögren's Syndrome

Author: Shelley Ensz. Scleroderma is highly variable. See Types of Scleroderma. Read Disclaimer
Similar & Overlaps
Personal Stories

Overview: What is Sjögren's Syndrome?

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

Presented by Amanda Thorpe
*More Sclero Videos*

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 VeryWell.

Sjögren's Synddrome.

Physical activity but not sedentary activity is reduced in primary Sjögren's syndrome (PSS). Physical, but not sedentary, activity is reduced in people with PSS and is associated with symptoms of depression and daytime sleepiness. PubMed, Rheumatol Int, 12/24/2016.

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.


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.

Nailfold Capillaroscopy
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, Raynaud's syndrome, primary biliary cirrhosis, and rheumatoid arthritis.

About twenty percent of patients with systemic scleroderma also have Secondary Sjögren's Syndrome.

Association between objective signs and subjective symptoms of dry eye disease in patients with systemic sclerosis. A significant correlation was found between the Ocular Surface Disease Index (OSDI) as a subjective parameter and disease duration. PubMed, Rheumatol Int, 09/07/2017.

Polyautoimmunity in Sjögren's Syndrome. Polyautoimmunity is a frequent condition in Sjögren syndrome (SS) and follows a grouping pattern and the study of polyautoimmunity provides important clues for elucidating the common mechanisms of autoimmne diseases. PubMed, Rheum Dis Clin North Am, 2016 Aug;42(3):457-72. (Also see Autoimmunity)

Comorbidities in patients with Primary Sjögren's Syndrome (pSS) and Systemic Lupus Erythematosus (SLE): A comparative registries–based study. pSS patients have consistently less serious cardiovascular comorbidity burden and a lower prevalence of severe infection than those with SLE, although in contrast, their risk of lymphoma was greater. PubMed, Arthritis Care Res (Hoboken), 08/26/2016. (Also see Lupus in Overlap and Complications)

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.

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.

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.

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. (Also see:Multiple Autoimmune Syndrome)

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

Systematic review and meta–analysis of the epidemiology of polyautoimmunity in Sjögren's syndrome (secondary Sjögren's syndrome (sSS)) focusing on autoimmune rheumatic diseases. Prevalence rates of sSS vary widely in different populations and the results of this meta–analysis highlight the need for better quality population studies. PubMed, Scand J Rheumatol, 2017 Sep 20:1-14.

Illness Experiences in Women with Oral Dryness as a Result of Sjögren's Syndrome (pSS): The Patient Point of View. Xerostomia and other consequences of pSS can have a profound influence on daily life, however, the severity of the consequences depends on individual experiences with the illness, social influences and the psychological responses of the patient. Wiley Online Library, 04/06/2016.

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.

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

Giving Assistant
United Way of Central New Mexico
United Way of Snohomish County
See ISN News for recent donors, including Pam Hop, Margaret Roof, Winifred Schillberg, Arnold Slotkin, and Juliet Youkhana. Plus donations in honor of Kim Thwaits, and in loving memory of Marta Marx, Jim Miller, and Arlene Marie Petulla.
Click Here to Donate or Shop
or click on the floating green DONATE button.


TOLL FREE HOTLINE, U.S. and Canada: 1-800-564-7099
Ask for our Free Info Packet by email or postal mail!

SCLERO.ORG is the world leader for trustworthy research, support, education and awareness for scleroderma and related illnesses, such as pulmonary hypertension. We are a service of the nonprofit International Scleroderma Network (ISN), which is a 501(c)(3) U.S.-based public charitable foundation, established in 2002. Meet Our Team, or Volunteer. Donations may also be mailed to:

International Scleroderma Network (ISN)
7455 France Ave So #266
Edina, MN 55435-4702 USA
Email [email protected] to request our Welcome email, or to report bad links or to update this page content. Privacy Policy.

The most important thing in the world to know about scleroderma is!

Donate Now
Copyright 1998-2018, International Scleroderma Network. AKA Scleroderma from A to Z and SCLERO.ORG. All Rights Reserved.