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Sjögren's Syndrome

Author: Shelley Ensz. Scleroderma is highly variable. See Types of Scleroderma. Read Disclaimer
Overview
Symptoms
Diagnosis
Similar & Overlaps
Research
Treatments
Support
Personal Stories

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 VeryWell.

Sjögren's Synddrome. MedicineNet.com

An Innovative Online Qualitative Study to Explore the Symptom Experience of Patients with Primary Sjögren's Syndrome (pSS). Improving physician education of pSS may help to alleviate frustrations and delays associated with diagnosis; the advent of novel effective treatments would be welcomed by patients with pSS. PubMed, Rheumatol Ther, 2020 Sep;7(3):601-615. (Also see Coping with Scleroderma)

Symptoms

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.

Overview
Cardiovascular
Central Nervous System
Dry Eyes
Dry Mouth
Dry Vagina
Fatigue
Fibromyalgia
Liver
Pulmonary
Renal
Skin
Newborn Heart Problems
Sjogren's and Pregnancy

Diagnosis

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
Antibodies
Biomarkers
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.

Serological Evidence for the Association Between Epstein-Barr Virus Infection and Sjögren's Syndrome (SjS). The findings from this study provide strong serological evidence for the association between EBV infection and SjS. PubMed, Front Immunol, 2020 Oct 30;11:590444. (Also see Infections)

Two subgroups in systemic lupus erythematosus (SLE) with features of antiphospholipid (APS) or Sjögren's syndrome (SS) differ in molecular signatures and treatment perspectives. Stratifying SLE patients based on an autoantibody profile could be a way forward to understand underlying pathophysiology and to improve selection of patients for clinical trials of targeted treatments. PubMed, Arthritis Res Ther, 2019 Feb 18;21(1):62. (Also see Lupus in Overlap and Antiphospholipid Syndrome)

Association between primary Sjögren's syndrome, arterial stiffness, and subclinical atherosclerosis: a systematic review and meta-analysis. Our study suggests that PSS is associated with arterial stiffness and subclinical atherosclerosis. PubMed, Clin Rheumatol, 09/03/2018.

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 autoimmune diseases. PubMed, Rheum Dis Clin North Am. (Also see Autoimmunity)

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

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

Ultra-high frequency ultrasonography (UHFUS)-guided minor salivary gland biopsy: a promising procedure to optimize labial salivary gland biopsy in Sjögren's Syndrome. UHFUS seems a promising tool in SS diagnostic algorithm, being able to provide a valuable support to the biopsy procedure. PubMed, J Oral Pathol Med, 01/27/2021.

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, 2018 Mar;47(2):141-154.

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.

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.

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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