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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

It's more than dryness and fatigue: The patient perspective on health–related quality of life (HRQL) in Primary Sjögren's Syndrome (PSS) – A qualitative study. This qualitative study underpins that HRQL in PSS patients is affected by several factors. PubMed, PLoS One. 2017 Feb 9;12(2):e0172056. (Also see Coping with Scleroderma)

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.

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.

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 autoimmune 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.

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

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.

Standardisation of labial salivary gland histopathology in clinical trials in primary Sjögren's syndrome. We provide standardised consensus guidance for the use of labial salivary gland histopathology in the classification of PSS and in clinical trials and identify areas where further research is required to achieve evidence–based consensus. PubMed, Ann Rheum Dis, 2017 Jul;76(7):1161-1168.

Genetics of systemic lupus erythematosus (SLE) and Sjögren's syndrome: an update. SLE and Sjögren's syndrome are two closely related systemic autoimmune diseases that share multiple clinical and molecular aspects, including a significant number of susceptibility genes. PubMed,Curr Opin Rheumatol. 2016 Sep;28(5):506-14. (Also see Causes of Lupus: Genetics)

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.

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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