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Sjögren's syndrome

sjögren's syndrome, sjögren's syndrome symptoms
Sjögren's syndrome SjS, SS is a long-term autoimmune disease in which the moisture-producing glands of the body are affected4 This results primarily in the development of a dry mouth and dry eyes2 Other symptoms can include dry skin, a chronic cough, vaginal dryness, numbness in the arms and legs, feeling tired, muscle and joint pains, and thyroid problems4 Those affected are at an increased risk 5% of lymphoma27

While the exact cause is unclear, it is believed to involve a combination of genetics and an environmental trigger such as exposure to a virus or bacteria4 It can occur independently of other health problems primary Sjögren's syndrome or as a result of another connective tissue disorder secondary Sjögren's syndrome3 The inflammation that results progressively damages the glands7 Diagnosis is by biopsy of moisture-producing glands and blood tests looking for specific antibodies2 On biopsy there are typically lymphocytes within the glands2

Treatment is directed at the person's symptoms For dry eyes artificial tears, medications to reduce inflammation, punctal plugs, or surgery to shut the tear ducts, may be tried For a dry mouth, chewing gum preferably sugar free, sipping water, or a saliva substitute may be used In those with joint or muscle pain, ibuprofen may be used Medications that can cause dryness may also be stopped such as antihistamines4

The disease was described in 1933 by Henrik Sjögren after whom it is named; however, a number of earlier descriptions of people with the symptoms exist3 Between 02% and 12% of the population are affected, with half having the primary form and half the secondary form7 Females are affected about ten times as often as males and it commonly begins in middle age;23 however, anyone can be affected2 Among those without other autoimmune disorders, life expectancy is unchanged6


  • 1 Signs and symptoms
    • 11 Associated conditions
  • 2 Cause
    • 21 Genetic factors
    • 22 Hormonal factors
    • 23 Microchimerism factors
    • 24 Environmental factors
  • 3 Pathogenesis
    • 31 Genetic predisposition
    • 32 Environmental triggers
    • 33 Inflammation
    • 34 Programmed cell death
    • 35 Hormonal factors
  • 4 Diagnosis
  • 5 Prevention
  • 6 Treatment
    • 61 Eye care
    • 62 Vaginal dryness
    • 63 Musculoskeletal
    • 64 Systemic
    • 65 Dental care
  • 7 Prognosis
  • 8 Complications
  • 9 Epidemiology
  • 10 History
  • 11 Research
  • 12 Notable cases
  • 13 See also
  • 14 References
  • 15 External links

Signs and symptomsedit

The hallmark symptom of SS is a generalized dryness, typically including dry mouth and keratoconjunctivitis sicca dry eyes, part of what are known as sicca "dryness" symptoms Sicca syndrome also incorporates vaginal dryness and chronic bronchitis SS may cause skin, nose, and vaginal dryness, and may affect other organs of the body, including the muscles myositis, kidneys, blood vessels, lungs, liver, biliary system, pancreas, peripheral nervous system distal axonal sensorimotor neuropathy or small fiber peripheral neuropathy and brain Some people have gastrointestinal or esophageal diseases such as GERD, achlorhydria or gastroparesis Chronic pain with accompanying fatigue and brain fog may also occurcitation needed

Skin dryness in some people with SS may be the result of lymphocytic infiltration into skin glands The symptoms may develop insidiously, with the diagnosis often not considered for several years, because the complaints of sicca may be otherwise attributed to medications, a dry environment, aging, or may be regarded as not of severity warranting the level of investigation necessary to establish the presence of the specific underlying autoimmune disorder8

SS can damage vital organs of the body with symptoms that may plateau or worsen, or go into remission as with other autoimmune diseases Some people may experience only the mild symptoms of dry eyes and mouth, while others have symptoms of severe disease Many patients can treat problems symptomatically Others are forced to cope with blurred vision, constant eye discomfort, recurrent mouth infections, swollen parotid glands, dysphonia vocal disorders including hoarseness, and difficulty in swallowing and eating Debilitating fatigue and joint pain can seriously impair quality of life Some patients can develop renal kidney involvement autoimmune tubulointerstitial nephritis leading to proteinuria excess protein in urine, urinary concentrating defect, and distal renal tubular acidosis

Associated conditionsedit

SS is associated with a number of other medical conditions, many of which are autoimmune or rheumatic disorders, such as celiac disease,910 fibromyalgia, SLE lupus, autoimmune thyroiditis, multiple sclerosis and spondyloarthropathy,11 and several malignancies, principally non-Hodgkin lymphoma1112


Although the cause of SS is unknown, it is believed to be under the influence of a combination of genetic, environmental, and several other factors, as is the case with many other autoimmune disorders13

Genetic factorsedit

The observation of high rates of autoimmune disorders in families of SS is linked with a genetic predisposition to the syndrome14 Studies on the polymorphisms of human leukocyte antigen HLA-DR and HLA-DQ gene regions in SS patients show differential susceptibility to the syndrome due to different types of the resulting autoantibody production14

Hormonal factorsedit

Since SS is associated with a high prevalence in women, sex hormones, especially estrogen, are believed to affect humoral and cell-mediated immune responses affecting susceptibility to the syndrome14 Androgens are generally considered to prevent autoimmunity15 Studies on mice models suggest estrogen deficiency stimulates presentation of autoantigens, inducing SS-like symptoms14

Microchimerism factorsedit

Microchimerism of fetal cells offspring lymphoid cells in maternal circulation may generate autoimmunity in women who have been previously pregnant1516 Generation of an autoimmune potential via microchimerism may lead to a switch from a silent form of autoimmunity with age-dependent decrease in self-tolerance15

Environmental factorsedit

Viral proteins, engulfed molecules, or degraded self-structures may initiate autoimmunity by molecular mimicry and increase the chances of SS development15 Epstein-Barr virus, hepatitis C, and human T-cell leukemia virus-1 are among the most studied infectious agents in SS15 Damaged self-structures targeted for apoptosis may be mistakenly exposed to the immune system, triggering autoimmunity in exocrine glands which are often prone to autoimmune responses15


The pathogenetic mechanisms of SS have not been fully elucidated, resulting in the lack of pathophysiology knowledge of the management of this autoimmune exocrinopathy Although the numerous factors contributing to the progression of this disease have made it difficult to find out the exact origin and cause, major advances over the past decade have contributed to a proposed set of pathogenic events that occur prior to the diagnosis of SS17

SS was originally proposed to be a specific, self-perpetuating immune system-mediated loss of exocrine glands, specifically acinar and ductal cells Although this explains the more obvious symptoms eg, the lack of salivary and lacrimal fluid, it does not explain the more widespread systemic effects seen in the progression of the disease

In the presence of a susceptible genetic background, both environmental and hormonal factors are thought to be capable of triggering the infiltration of lymphocytes, specifically CD4+ T cells, B cells, and plasma cells, causing glandular dysfunction in the salivary and lacrimal glands17

SS is associated with increased levels in cerebrospinal fluid CSF of IL-1RA, an interleukin 1 antagonist This suggests that the disease begins with increased activity in the interleukin 1 system, followed by an autoregulatory up-regulation of IL-1RA to reduce the successful binding of interleukin 1 to its receptors Interleukin 1 likely is the marker for fatigue, but increased IL-1RA is observed in the CSF and is associated with increased fatigue through cytokine-induced sickness behavior18 SS, though, is characterized by decreased levels of IL-1ra in saliva, which could be responsible for mouth inflammation and dryness19 Patients with secondary SS also often exhibit signs and symptoms of their primary rheumatic disorders, such as systemic lupus erythematosus, rheumatoid arthritis, or systemic sclerosis

Genetic predispositionedit

The genetic locus most significantly associated with primary SS is the major histocompatibility complex/human leukocyte antigen MHC/HLA region, as demonstrated by the preliminary results of the first genome-wide association study GWAS20 This GWAS included data from a discovery cohort of 395 patients of European ancestry with primary SS, and 1,975 healthy control individuals, and from a replication study that comprised 1,234 cases and 4,779 healthy controls Associations with polymorphisms located at six independent loci were also detected; IRF5, STAT4, BLK, IL12A, TNIP1, and CXCR5 This also suggested the activation of the innate immune system, notably through the IFN system, B-cell activation through CXCR5-directed recruitment to lymphoid follicles and B-cell receptor BCR activation involving BLK, and T-cell activation owing to HLA susceptibility and the IL-12-IFN-γ-axis21

Patients of different ethnic origin carry different HLA susceptibility alleles, of which, HLA-DR and HLA-DQ are involved in the pathogenesis of SS For example, patients from Northern and Western Europe and from North America show a high prevalence of B8, DRw52, and DR3 genes22 HLA class II alleles are associated with the presence of specific subsets of autoantibodies, rather than with the disease itself23 Autoantibodies refer to the loss of B-cell tolerance leading to production of antibodies directed against diverse organ-specific and organ nonspecific antigens17 Association between HLA and SS is restricted to patients with anti-SSA/Ro or anti-SSB/La antibodies Seropositivity for anti-Ro and anti-La is associated with greater severity and longer duration of disease, and findings of their high abundance from the salivary glands of SS patients suggests their imperative role in the pathogenesis of SS24

Beyond genetics, epigenetic abnormality related to DNA methylation, histone acetylation, or microRNA expression probably have key roles in the pathogenesis of autoimmune diseases, including SS, though research in this area is very limited and minimal25

Environmental triggersedit

Environmental factors, such as glandular viral infection, could prompt epithelial cells to activate the HLA-independent innate immune system through toll-like receptors26 Although a number of infectious, exogenous agents have been implicated in the pathogenesis of SS, such as Epstein-Barr virus EBV, human T-lymphotropic virus 1, and hepatitis C virus, their association with SS appears weak While EBV is present in the salivary glands of normal individuals, a high incidence of EBV reactivation in SS patients has been reported with increased levels of EBV DNA This indicates viral reactivation and inability of lymphoid infiltrates to control EBV replication in SS, leading to the initiation or perpetuation of an immune response in target organs Nonetheless, it remains to be clarified exactly how reactivation of EBV is induced in lesions of patients with SS, and which specific molecular mechanisms are involved in the process of viral reactivation27


Epithelial cells in SS lesions are active participants in the induction and perpetuation of the inflammatory process Environmental and hormonal factors, in concert with an appropriate genetic background, are believed to trigger SS, which dysregulates epithelial cells and allows aberrant homing and activation of dendritic cells DCs, T cells, and B cells28 Dendritic cells are antigen-presenting cells which process antigen material and present it to other T cells Following the migration of lymphocytes into the glands in response to chemokines and specific adhesion molecules, T cells interact with epithelial cells Epithelial cells are further activated by proinflammatory cytokines IL-1β, IFN-γ, and TNF, which are produced by adjacent T cells The early accumulation of plasmacytoid dendritic cells in the target tissues, which produce high levels of type 1 IFNs, seems to be important, as these cells can further dysregulate the immune response through abnormal retention of lymphocytes in the tissues and their subsequent activation IFN-α stimulates the production of B-cell activating factor BAFF by epithelial cells, DCs, and T cells BAFF stimulates aberrant B-cell maturation, leading to the emergence of self-reactive B cells, which locally produce autoantibodies, in a germinal centre-like structure GC-like, which is also the location of lymphomagenesis origin of lymphoma17

Programmed cell deathedit

Dysregulation of apoptosis programmed cell death is believed to play a role in the pathogenesis of a variety of autoimmune diseases, though its role in SS is controversial Both the Fas and Fas ligand proteins are overexpressed in primary SS patients, while expression of BCL-1, which is known to downregulate apoptosis, was found to be significantly reduced in acinar and ductal epithelial cells of SS patients compared to healthy people2930 In situ studies did not show increased apoptosis among glandular epithelial cells, but did show reduced apoptosis among infiltrating mononuclear cells Reduced apoptosis was also implicated in the accumulation of autoreactive B-cells found in the glands The relationship of autoantibodies expressed in SS with apoptosis is still being researched13

Hormonal factorsedit

Sex hormones seem to influence humoral and cell-mediated immune response, with estrogen being considered one of the biggest factors responsible for sex-immunologic dimorphism31 Estrogen deficiency appears to play a role in development of SS32 It has been hypothesized that androgen administration to the ocular surface may serve as an effective therapy for dry eyes33


Speckled Immunofluorescence staining pattern of antinuclear antibodies on HEp-20-10 cells

Diagnosing SS is complicated by the range of symptoms a patient may manifest, and the similarity between symptoms of SS and those of other conditions Also, patients who have symptoms of SS approach different specialities regarding their symptoms which make the diagnosis difficult Since the symptoms of this autoimmune disorder such as dry eyes and dry mouth are very common among people, and mostly observed from the age of 40 and above, it is often mistaken as age-related, thus ignored However, some medications can also cause symptoms that are similar to those of SS The combination of several tests, which can be done in a series, can eventually lead to the diagnosis of SS1634

SS is usually classified as either 'primary' or 'secondary' Primary Sjögren’s syndrome occurs by itself and secondary Sjögren’s syndrome occurs when another connective tissue disease is present

Blood tests can be done to determine if a patient has high levels of antibodies that are indicative of the condition, such as antinuclear antibody ANA and rheumatoid factor because SS frequently occurs secondary to rheumatoid arthritis, which are associated with autoimmune diseases Typical SS ANA patterns are SSA/Ro and SSB/La, of which SSB/La is far more specific; SSA/Ro is associated with numerous other autoimmune conditions, but are often present in SS However, SSA and SSB tests are frequently not positive in SS

The rose bengal test uses a stain that measures state and function of the lacrimal glands This test involves placing the non-toxic dye rose bengal on the eyes The dye’s distinctive colour helps in determining the state and functioning of tear film and the rate of tear evaporation Any distinctive colour change observed will be indicative of SS, but many related diagnostic tools will be used to confirm the condition of SS34

Schirmer's test measures the production of tears: a strip of filter paper is held inside the lower eyelid for five minutes, and its wetness is then measured with a ruler Producing less than 5 mm 020 in of liquid is usually indicative of SS This measurement analysis varies among people depending on other eye-related conditions and medications in use when the test is taken34 A slit-lamp examination can reveal dryness on the surface of the eye

Symptoms of dry mouth and dryness in the oral cavity are caused by the reduced production of saliva from the salivary glands parotid gland, submandibular gland, and sublingual gland To check the status of salivary glands and the production of saliva, a salivary flow-rate test is performed, in which the person is asked to spit as much as they can into a cup, and the resulting saliva sample is collected and weighed This test's results can determine whether the salivary glands are functioning adequately Not enough saliva produced could mean the person has SS34 An alternative test is non-stimulated whole saliva flow collection, in which the person spits into a test tube every minute for 15 minutes A resultant collection of less than 15 ml 0053 imp fl oz; 0051 US fl oz is considered a positive result35

A lip/salivary gland biopsy takes a tissue sample that can reveal lymphocytes clustered around salivary glands, and damage to these glands due to inflammation This test involves removing a sample of tissue from a person’s inner lip/salivary gland and examining it under a microscope In addition, a sialogram, a special X-ray test, is performed to see if any blockage is present in the salivary gland ducts ie parotid duct and the amount of saliva that flows into the mouth34

Also, a radiological procedure is available as a reliable and accurate test for SS A contrast agent is injected into the parotid duct, which opens from the cheek into the vestibule of the mouth opposite the neck of the upper second molar tooth Histopathology studies should show focal lymphocytic sialadenitis Objective evidence of salivary gland involvement is tested through ultrasound examinations, the level of unstimulated whole salivary flow, a parotid sialography or salivary scintigraphy, and autoantibodies against Ro SSA and/or La SSB antigens

SS can be excluded from people with past head and neck radiation therapy, acquired immunodeficiency syndrome AIDS, pre-existing lymphoma, sarcoidosis, graft-versus-host disease, and use of anticholinergic drugs


There is no prevention mechanism for SS due to its complexity as an autoimmune disorder However, lifestyle changes can reduce the risk factors of getting SS or reduce the severity of the condition with patients who have already been diagnosed Diet is strongly associated with inflammation that is mostly seen in many autoimmune related diseases including SS An experimental study concludes that SS patients show high sensitivity to gluten that directly relates to inflammation36 Moderate exercise is also found to be helpful in SS patients mainly reducing the effect of lung inflammation37


Neither a cure for SS nor a specific treatment is known to permanently restore gland secretion Instead, treatment is generally symptomatic and supportive

Eye careedit

Moisture replacement therapies such as artificial tears may ease the symptoms of dry eyes Some patients with more severe problems use goggles to increase local humidity or have punctal plugs inserted to help retain tears on the ocular surface for a longer time

Additionally, cyclosporine Restasis is available by prescription to help treat chronic dry eye by suppressing the inflammation that disrupts tear secretion Prescription drugs are also available that help to stimulate salivary flow, such as cevimeline Evoxac and pilocarpine Salagen, a manufactured form of pilocarpine, can be used to help produce tears, as well as saliva in the mouth and intestines It is derived from the jaborandi plant

Vaginal drynessedit

In women with SS, vaginal dryness, vulvodynia and dyspareunia painful sexual intercourse are often reported; personal lubricants are recommended to help lessen irritation or pain that may result from dryness in the vaginal and vulva areas34


Nonsteroidal anti-inflammatory drugs NSAIDs may be used to treat musculoskeletal symptoms For individuals with severe complications, corticosteroids or immunosuppressive drugs may be prescribed, and sometimes IVIG intravenous immunoglobulin Also, disease-modifying antirheumatic drugs DMARDs such as methotrexate may be helpful Hydroxychloroquine Plaquenil is another option and is generally considered safer than methotrexate However, these prescribed drugs have a range of side effects such as nausea, loss of appetite, dizziness, hair loss, stomach aches/cramps, headache, liver toxicity, and increased risk of infections Also, people who take drugs to suppress the immune system are more likely to develop cancer later34


For systemic symptoms, including fatigue, joint pain, myositis and neuropathy, biologic immunosuppressant drugs such as rituximab and belimumab that work via B-cell pathology are often used and have less toxic profiles than traditional immunosuppressive regimens

Dental careedit

Preventive dental treatment is also necessary and often overlooked by the patient, as the lack of saliva associated with xerostomia creates an ideal environment for the proliferation of bacteria that cause cavities38 Treatments include at-home topical fluoride application to strengthen tooth enamel and frequent teeth cleanings by a dental hygienist Existing cavities must also be treated, as cavities that extend into the tooth can not be effectively treated through teeth cleaning alone, and are at a high risk of spreading into the pulp of the tooth, leading to the loss of vitality and need for extraction or root canal therapy This treatment regimen is the same as that used for all xerostomia patients, such as those undergoing head and neck radiation therapy which often damages the salivary glands, as they are more susceptible to radiation than other body tissues

Unfortunately, many patients, not realizing the need for dental treatment, do not see a dentist until most of their teeth are beyond the point of restoration It is not uncommon for a dentist to see a xerostomia patient with severe, untreatable cavities in almost every tooth In severe cases, the only viable treatment may be to extract all of the patient's teeth and treat with prosthetics such as dentures or implants


Published studies on the survival of SS patients are limited in varied respects, perhaps owing to the relatively small sample sizes, and secondary SS is associated with other autoimmune diseases However, results from a number of studies indicated, compared to other autoimmune diseases, SS is associated with a notably high incidence of malignant non-Hodgkin lymphoma NHL17 NHL is the cancer derived from white blood cells About 5% of patients with SS will develop some form of lymphoid malignancy39 Patients with severe cases are much more likely to develop lymphomas than patients with mild or moderate cases40 The most common lymphomas are salivary extranodal marginal zone B cell lymphomas MALT lymphomas in the salivary glands41 and diffuse large B-cell lymphoma40

Lymphomagenesis in primary SS patients is considered as a multistep process, with the first step being chronic stimulation of autoimmune B cells, especially B cells that produce rheumatoid factor at sites targeted by the disease4243 This increases the frequency of oncogenic mutation, leading to any dysfunction at checkpoints of autoimmune B-cell activation to transform into malignancy A study's finding has concluded the continuous stimulation of autoimmune B cells, leading to subtle germinal abnormalities in genes having specific consequences in B cells, which underlies the susceptibility to lymphoma44

Apart from this notably higher incidence of malignant NHL, SS patients show only modest or clinically insignificant deterioration in specific organ-related function, which explains the only slight increases in mortality rates of SS patients in comparison with the remainder of the population17


Among the complications discussed above, women with anti-Ro/SSA and anti-La/SSA antibodies who become pregnant, have an increased rate of neonatal lupus erythematosus with congenital heart block requiring a pacemaker45 Type I cryoglobulinemia is a known complication of SS46


SS is the third most common rheumatic autoimmune disorder, behind rheumatoid arthritis RA and systemic lupus erythematosus SLE47 There are no geographical differences in the rates of SS48 SS has been reported in all areas of the world, although regional rates have not been well studied4849 Depending on the criteria for determining prevalence, studies estimate the prevalence of SS at 500,000 to 2 million people in the United States Moreover, other broader studies of prevalence of SS range widely with some reports of up to a prevalence of 3% of the population47 A few studies that have been conducted on the incidence of SS report that the incidence of the syndrome varies between 3 and 6 per 100,000 per year4750

Nine out of ten SS patients are reported to be women1349 In addition to prevalence in women, having a first-degree relative with an autoimmune disease and previous pregnancies have been identified as epidemiological risk factors51 Differences in prevalence due to race and ethnicity are unknown

Although SS occurs in all age groups, the average age of onset is between ages 40 and 60, although experts note that up to half of all cases may be left undiagnosed or unreported13475253 The prevalence of SS generally increases with age47

SS has been known to be reported in 30-50% of people with rheumatoid arthritis, as well as 10-25% with systemic lupus erythematosus13


Johann von Mikulicz-Radecki 1850–1905 is generally credited with the first description of SS In 1892, he described a 42-year-old man with enlargement of the parotid and lacrimal glands associated with a round-cell infiltrate and acinar atrophy3454 However, the criteria Mikulicz established for diagnosis, often led to misdiagnosis of Mikulicz’s syndrome Many conditions, such as tuberculosis, infections, sarcoidosis, and lymphoma present with similar conditions to those listed under Mikulicz’s syndrome34 Nevertheless, the term "Mikulicz’s syndrome" is still used occasionally to describe the appearance of lymphocytic infiltrates on salivary-gland biopsies34

In 1930, Henrik Sjögren 1899–1986, an ophthalmologist in Jönköping, Sweden, observed a patient with low secretions from the lacrimal and salivary glands55 Sjögren introduced the term keratoconjunctivitis sicca for the symptom of dry eyes keratoconjunctivitis In 1933, he published his doctoral thesis, describing 19 females, most of whom were postmenopausal and had arthritis, showing clinical and pathological manifestations of the syndrome54 Sjögren clarifies that keratoconjunctivitis sicca, resulting from water deficiency, had no relation to xerophthalmia, resulting from vitamin A deficiency54 Sjögren’s thesis was not well received as the Board of Examiners criticized some clinical aspects55

After extensive research and data collection, Sjögren published an essential paper in 1951, describing 80 patients with keratoconjunctivitis sicca, 50 of whom also had arthritis55 His subsequent follow-up conference trips pertaining to his paper led to an international interest in SS55 The term keratoconjunctivitis sicca was coined by Sjögren himself began to be identified as SS in literature,55 although it can now have more general usage


Singer-actress Carrie Ann Inaba is the National Awareness Ambassador and Spokesperson for the Sjogren's Syndrome Foundation

Research on multifactorial autoimmune diseases such as SS focuses on expanding the knowledge surrounding the disorder, improving diagnostic tools and finding ways to prevent, manage, and cure the disorder The United Kingdom Primary Sjögren's Syndrome Registry, a tissue biobank of samples taken for research, supported by the Medical Research Council, UK was established in 201056

As an autoimmune disease, susceptibility to SS is greatly influenced by the human leukocyte antigen57 DQA105:01, DQB102:01, and DRB103:01 alleles were identified as risk factors, while DQA102:01, DQA103:01 and DQB105:01 alleles were found to be protective factors for the disease58 The relationship between alleles and specific race was also established59 HLA-DQ2 and HLA-B8 are generally found in Caucasian patients, while HLA-DR5 is related to Greek and Israeli patients59 Multiple genome-wide association scans may be conducted in the future to identify key risk variants57

Viruses that can trigger the immune response of the syndrome include human T-lymphotropic virus type 1 HTLV-1, Epstein-Barr virus EBV, human immunodeficiency virus HIV and hepatitis C virus HCV59 The UK Primary Sjögren's Syndrome Registry supports clinical trials and genetic studies of SS and is open to patients wishing to participate in research studies and researchers studying the disease56

Some research showed that the lack of vitamin A and vitamin D are associated with this disease59 Vitamin D deficiency was found to be related to neurological manifestations and the presence of lymphoma among patients On the other hand, vitamin A levels were inversely associated with extra-glandular manifestations of the disease59

Saliva is a potential diagnostic tool of SS because the salivary component is changed after onset of the disease60 With the new miniaturization technology, called ‘lab on a chip’, the diagnosis can be more convenient60

With regard to therapeutics, multiple monoclonal antibodies were under investigation in 200761 The most promising seemed to be the anti-CD20 rituximab and the anti-CD22 epratuzumab, while the anti-TNF-α and IFN-α seemed less effective61

In 2014, the Sjögren's Syndrome Foundation announced a five-year goal to cut the time to diagnoses in half62

Notable casesedit

  • Shannon Boxx US Olympic soccer player has both SS and lupus63
  • Carrie Ann Inaba singer-actress is the National Awareness Ambassador & Spokesperson for the Sjogren Syndrome Foundation64
  • Venus Williams world champion tennis player has been diagnosed with SS and said she had struggled with fatigue for years65

See alsoedit

  • Benign lymphoepithelial lesion
  • Burning mouth syndrome
  • Invisible disability
  • Keratoconjunctivitis sicca
  • List of cutaneous conditions
  • Parotitis
  • Xerostomia


  • The original text from this article was obtained from a public domain resource at NIH
  1. ^ Elsevier, Dorland's Illustrated Medical Dictionary, Elsevier 
  2. ^ a b c d e f g h i j Brito-Zerón, P; Baldini, C; Bootsma, H; Bowman, SJ; Jonsson, R; Mariette, X; Sivils, K; Theander, E; Tzioufas, A; Ramos-Casals, M 7 July 2016 "Sjögren syndrome" Nature Reviews Disease Primers 2: 16047 PMID 27383445 doi:101038/nrdp201647 
  3. ^ a b c d Ng, Wan-Fai 2016 Sjogren's Syndrome Oxford University Press pp 10–11 ISBN 9780198736950 
  4. ^ a b c d e f g "What Is Sjögren’s Syndrome Fast Facts" NIAMS November 2014 Retrieved 15 July 2016 
  5. ^ Ferri, Fred F 2010 Ferri's differential diagnosis : a practical guide to the differential diagnosis of symptoms, signs, and clinical disorders 2nd ed ed Philadelphia, PA: Elsevier/Mosby p Chapter S ISBN 0323076998 CS1 maint: Extra text link
  6. ^ a b Singh, AG; Singh, S; Matteson, EL March 2016 "Rate, risk factors and causes of mortality in patients with Sjögren's syndrome: a systematic review and meta-analysis of cohort studies" Rheumatology Oxford, England 55 3: 450–60 PMID 26412810 doi:101093/rheumatology/kev354 
  7. ^ a b c d John H, Klippel 2008 Primer on the rheumatic diseases 13th ed New York, NY: Springer p 389 ISBN 9780387685663 Retrieved 15 July 2016 
  8. ^ Fox, R I, Stern, M & Michelson, P Update in Sjögren syndrome Curr Opin Rheumatol 12, 391-398 2000
  9. ^ Lundin KE, Wijmenga C Sep 2015 "Coeliac disease and autoimmune disease–genetic overlap and screening" Nat Rev Gastroenterol Hepatol Review 12 9: 507–15 PMID 26303674 doi:101038/nrgastro2015136 
  10. ^ Denham JM, Hill ID Aug 2013 "Celiac disease and autoimmunity: review and controversies" Curr Allergy Asthma Rep Review 13 4: 347–53 PMC 3725235  PMID 23681421 doi:101007/s11882-013-0352-1 
  11. ^ a b Gabriel SE, Michaud K 2009 "Epidemiological studies in incidence, prevalence, mortality, and comorbidity of the rheumatic diseases" Arthritis Res Ther Review 11 3: 229 PMC 2714099  PMID 19519924 doi:101186/ar2669 
  12. ^ Papageorgiou A, Voulgarelis M, Tzioufas AG Jul 2015 "Clinical picture, outcome and predictive factors of lymphoma in Sjӧgren syndrome" Autoimmun Rev Review 14 7: 641–9 PMID 25808075 doi:101016/jautrev201503004 
  13. ^ a b c d e Borchers A T, Naguwa S M, Keen C L, Gershwin M E 2003 "Immunopathogenesis of Sjögren's syndrome" Clin Rev Allergy Immunol 25: 89–104 doi:101385/criai:25:1:89 CS1 maint: Multiple names: authors list link
  14. ^ a b c d Voulgarelis M, Tzioufas A G 2010 "Pathogenetic mechanisms in the initiation and perpetuation of Sjögren's syndrome" Nature Reviews Rheumatology 6: 529–537 doi:101038/nrrheum2010118 
  15. ^ a b c d e f Delaleu N, Jonsson R, Koller M M 2005 "Sjögren's syndrome" Eur J Oral Sci 113: 101–113 doi:101111/j1600-0722200400183x CS1 maint: Multiple names: authors list link
  16. ^ a b Whitacre C C 2001 "Sex differences in autoimmune disease" Nat Immunol 2: 777–780 doi:101038/ni0901-777 
  17. ^ a b c d e f Voulgarelis M, Tzioufas A G 2010 "Pathogenetic mechanisms in the initiation and perpetuation of SjÖgren's syndrome" Nature Reviews Rheumatology 6: 529–537 doi:101038/nrrheum2010118 
  18. ^ Harboe, Erna; Tjensvoll, Anne Bolette; Vefring, Hege K; Gøransson, Lasse G; Kvaløy, Jan Terje; Omdal, Roald 2009 "Fatigue in primary Sjögren's syndrome – A link to sickness behaviour in animals" Brain, Behavior, and Immunity 23 8: 1104–8 PMID 19560535 doi:101016/jbbi200906151 
  19. ^ Perrier, S; Coussediere, C; Dubost, JJ; Albuisson, E; Sauvezie, B 1998 "IL-1 receptor antagonist IL-1RA gene polymorphism in Sjögren's syndrome and rheumatoid arthritis" Clinical immunology and immunopathology 87 3: 309–13 PMID 9646842 doi:101006/clin19984520 
  20. ^ Reveille JD: The molecular genetics of systemic lupus erythematosus and Sjögren's syndrome Curr Opin Rheumatol 1992, 4:644-656
  21. ^ Lessard, C J et al Identification of multiple Sjögren’s syndrome susceptibility loci abstract OP0020 AnnRheum Dis 72 Suppl 3, 54 2013
  22. ^ Kang HI, Fei HM, Saito I, Sawada S, Chen SL, Yi D, Chan E, Peebles C, Bugawan TL, Erlich HA; et al ": Comparison of HLA class II genes in Caucasoid, Chinese, and Japanese patients with primary Sjögren's syndrome" J Immunol 1993 150: 3615–3623 CS1 maint: Explicit use of et al linkCS1 maint: Multiple names: authors list link
  23. ^ Bolstad AI, Wassmuth R, Haga HJ, Jonsson R "HLA markers and clinical characteristics in Caucasians with primary Sjögren's syndrome" J Rheumatol 2001 28: 1554–1562 CS1 maint: Multiple names: authors list link
  24. ^ Fei HM, Kang H, Scharf S, Erlich H, Peebles C, Fox R "Specific HLA-DQA and HLA-DRB1 alleles confer susceptibility to Sjögren's syndrome and autoantibody production" J Clin Lab Anal 1991 5: 382–391 CS1 maint: Multiple names: authors list link
  25. ^ Lu Q 2013 "The critical importance of epigenetics in autoimmunity" J Autoimmun 41: 1–5 doi:101016/jjaut201301010 
  26. ^ Takeda, K, Kaisho, T & Akira, S Toll-like receptors" Annu Rev Immunol 2003; 21, 335–376
  27. ^ Pflugfelder S C; et al 1993 "Epstein–Barr virus and the lacrimal gland pathology of Sjögren's syndrome" Am J Pathol 143: 49–64 CS1 maint: Explicit use of et al link
  28. ^ Manoussakis M N; et al 2007 "Rates of infiltration by macrophages and dendritic cells and expression of interleukin-18 and interleukin-12 in the chronic inflammatory lesions of Sjögren's syndrome: correlation with certain features of immune hyperactivity and factors associated with high risk of lymphoma development" Arthritis Rheum 56: 3977–3988 doi:101002/art23073 CS1 maint: Explicit use of et al link
  29. ^ Ohlsson M; et al 2002 "CD40, CD154, Bax and Bcl-2 expression in Sjögren's syndrome salivary glands: a putative anti-apoptotic role during its effector phases" Scand J Immunol 56: 561–571 doi:101046/j1365-3083200201168x CS1 maint: Explicit use of et al link
  30. ^ Ohlsson M; et al 2001 "Fas-induced apoptosis is a rare event in Sjo ̈ gren's syndrome" Lab Invest 81: 95–105 doi:101038/labinvest3780215 CS1 maint: Explicit use of et al link
  31. ^ Cutolo M, Sulli A, Capellino S, Villaggio B, Montagna P, Seriolo B, Straub RH 2004 "Sex hormones influence on the immune system: basic and clinical aspects in autoimmunity" Lupus 13 9: 635–8 PMID 15485092 doi:101191/0961203304lu1094oa 
  32. ^ Mavragani CP, Fragoulis GE, Moutsopoulos HM Dec 2012 "Endocrine alterations in primary Sjogren's syndrome: an overview" J Autoimmun Review 39 4: 354–8 PMID 22695186 doi:101016/jjaut201205011 
  33. ^ Sullivan DA, Wickham LA, Rocha EM, Krenzer KL, Sullivan BD, Steagall R, Cermak JM, Dana MR, Ullman MD, Sato EH, Gao J, Rocha FJ, Ono M, Silveira LA, Lambert RW, Kelleher RS, Tolls DB, Toda I 1999 "Androgens and dry eye in Sjögren's syndrome" Ann N Y Acad Sci 876: 312–24 PMID 10415627 doi:101111/j1749-66321999tb07656x 
  34. ^ a b c d e f g h i j Fox R I 2005 "Sjögren's syndrome" Lancet 366: 321–331 doi:101016/s0140-67360566990-5 
  35. ^ Dr J Parks, Ancaster ON Canada
  36. ^ Liden M; et al 2007 "Gluten sensitivity in patients with primary Sjogren's syndrome" Scand J Gastroenterol 42: 962–962 PMID 17613926 doi:101080/00365520701195345 CS1 maint: Explicit use of et al link
  37. ^ Strombeck BE, et al, Effects of exercise on aerobic capacity and fatigue in women with primary Sjögren's syndrome, Rheumatology, 46:868–871, 2007 PMID 17308315 Accessed 2016-11-12
  38. ^ Xin W, Leung KC, Lo EC, Mok MY, Leung MH, A randomized, double-blind, placebo-controlled clinical trial of fluoride varnish in preventing dental caries of Sjögren's syndrome patients, BMC Oral Health, 161:102, Sep 23, 2016 PMID 27664129 doi:101186/s12903-016-0296-7 Accessed 2016-11-12
  39. ^ Tzioufas, Athanasios G; Voulgarelis, Michael 2007 "Update on Sjögren's syndrome autoimmune epithelitis: from classification to increased neoplasias" Best Pract Res Clin Rheumatol 21 6: 989–1010 PMID 18068857 doi:101016/jberh200709001 
  40. ^ a b Smedby, K E; Baecklund, E; Askling, J 2006 "Malignant lymphomas in autoimmunity and inflammation: a review of risks, risk factors, and lymphoma characteristics" Cancer Epidemiol Biomarkers Prev 15 11: 2069–77 PMID 17119030 doi:101158/1055-9965EPI-06-0300 
  41. ^ Voulgarelis, Michael; Skopouli, Fotini N 2007 "Clinical, immunologic, and molecular factors predicting lymphoma development in Sjogren's syndrome patients" Clin Rev Allergy Immunol 32 3: 265–74 PMID 17992593 doi:101007/s12016-007-8001-x 
  42. ^ Martin T; et al 2000 "Salivary gland lymphomas in patients with Sjögren's syndrome may frequently develop from rheumatoid factor B cells" Arthritis Rheum 43: 908–916 doi:101002/1529-013120000443:4<908::aid-anr24>30co;2-k CS1 maint: Explicit use of et al link
  43. ^ Bende R J; et al 2005 "Among B cell non-Hodgkin's lymphomas, MALT lymphomas express a unique antibody repertoire with frequent rheumatoid factor reactivity" J Exp Med 201: 1229–1241 doi:101084/jem20050068 CS1 maint: Explicit use of et al link
  44. ^ Nocturne, G et al Germinal and somatic genetic variants of TNFAIP3 promote lymphomagenesis process complicating primary Sjögren’s syndrome abstract OP0023" Ann Rheum Dis 72 Suppl 3, 55 2013
  45. ^ Manthorpe, R; Svensson, A; Wirestrand, LE November 2004 "Late neonatal lupus erythematosus onset in a child born of a mother with primary Sjögren's syndrome" Ann Rheum Dis 63 11: 1496–7 PMC 1754813  PMID 15479901 doi:101136/ard2003014944 
  46. ^ Ramos-Casals, Manel; Cervera, Ricard; Yagüe, Jordi; García-Carrasco, Mario; Trejo, Olga; Jiménez, Sonia; Morlà, Rosa M; Font, Josep; Ingelmo, Miguel Dec 1998 "Cryoglobulinemia in primary Sjögren's syndrome: prevalence and clinical characteristics in a series of 115 patients" Semin Arthritis Rheum 28 3: 200–5 PMID 9872481 doi:101016/S0049-01729880037-1 
  47. ^ a b c d e Fox R I, Stern M, Michelson P 2000 "Update in Sjögren syndrome" Curr Opin Rheumatol 12: 391–398 CS1 maint: Multiple names: authors list link
  48. ^ a b Mavragani C P, Moutsopoulos H M 2010 "The geoepidemiology of Sjogren's syndrome" Autoimmunity Reviews 9: A305–A310 PMID 19903539 doi:101016/jautrev200911004 
  49. ^ a b Jonsson R, Vogelsang P, Volchenkov R, Espinosa A 2011 "The complexity of Sjogren's syndrome: Novel aspects on pathogenesis" Immunol Lett 141: 1–9 PMID 21777618 doi:101016/jimlet201106007 CS1 maint: Multiple names: authors list link
  50. ^ Alamanos Y; et al 2006 "Epidemiology of primary Sjögren's syndrome in north-west Greece, 1982-2003" Rheumatology Oxford 45: 187–191 PMID 16332955 doi:101093/rheumatology/kei107 CS1 maint: Explicit use of et al link
  51. ^ Priori R; et al 2007 "Risk factors for Sjögren's syndrome: a case-control study" Clin Exp Rheumatol 25: 378 CS1 maint: Explicit use of et al link
  52. ^ Haugen A J; et al 2008 "Estimation of the prevalence of primary Sjögren's syndrome in two age-different community-based populations using two sets of classification criteria: the Hordaland Health Study Scand" J Rheumatol 37: 30–34 PMID 18189192 doi:101080/03009740701678712 CS1 maint: Explicit use of et al link
  53. ^ García-Carrasco M; et al 2002 "Primary Sjögren syndrome: clinical and immunologic disease patterns in a cohort of 400 patients" Medicine 81: 270–280 doi:101097/00005792-200207000-00003 CS1 maint: Explicit use of et al link
  54. ^ a b c Parke A L, Buchanan W W 1998 "Sjögren's syndrome: History, clinical and pathological features" Inflammopharmacology 6: 271–287 doi:101007/s10787-998-0012-6 
  55. ^ a b c d e Murube, J Henrik Sjögren, 1899-1986 The ocular surface 8, 2-2 2010
  56. ^ a b Ng, W-F; Bowman, S J; Griffiths, B; Ukpssr Study, Group January 2011 "United Kingdom Primary Sjogren's Syndrome Registry--a united effort to tackle an orphan rheumatic disease" Rheumatology Oxford 50 1: 32–9 PMID 20693261 doi:101093/rheumatology/keq240 
  57. ^ a b Ice, John A; Li, He; Adrianto, Indra; Lin, Paul Chee; Kelly, Jennifer A; Montgomery, Courtney G; Lessard, Christopher J; Moser, Kathy L August 2012 "Genetics of Sjögren's syndrome in the genome-wide association era" J Autoimmun 39 1–2: 57–63 PMC 3518871  PMID 22289719 doi:101016/jjaut201201008 
  58. ^ Cruz-Tapias, Paola; Rojas-Villarraga, Adriana; Maier-Moore, Shannon; Anaya, Juan-Manuel February 2012 "HLA and Sjögren's syndrome susceptibility A meta-analysis of worldwide studies" Autoimmun Rev 11 4: 281–7 PMID 22001416 doi:101016/jautrev201110002 
  59. ^ a b c d e Peri, Yogev; Agmon-Levin, Nancy; Theodor, Emanuel; Shoenfeld, Yehuda February 2012 "Sjögren's syndrome, the old and the new" Best Pract Res Clin Rheumatol 26 1: 105–17 PMID 22424197 doi:101016/jberh201201012 
  60. ^ a b Liu, Jingyi; Duan, Yixiang July 2012 "Saliva: a potential media for disease diagnostics and monitoring" Oral Oncol 48 7: 569–77 PMID 22349278 doi:101016/joraloncology201201021 
  61. ^ a b Meijer, Jiska M; Pijpe, Justin; Bootsma, Hendrika; Vissink, Arjan; Kallenberg, Cees G M June 2007 "The future of biologic agents in the treatment of SS" Clin Rev Allergy Immunol 32 3: 292–7 PMC 2071970  PMID 17992596 doi:101007/s12016-007-8005-6 
  62. ^ ""Breakthrough Goal" SSF Launches 5-Year Breakthrough Goal/"To shorten the time to diagnose Sjögren’s by 50% in 5 years!"" Sjogren's Syndrome Foundation August 2016 
  63. ^ "Olympic soccer player Shannon Boxx's battle with lupus" CNN 2012 Retrieved 18 February 2014 
  64. ^ Sjogren's Syndrome Foundation Thanks Carrie Ann Inaba for Helping Raise Awareness
  65. ^ "Williams Says She Struggled With Fatigue for Years" NY Times 2011 Retrieved 18 February 2014 

External linksedit

  • ICD-10: M350
  • ICD-9-CM: 7102
  • OMIM: 270150
  • MeSH: D012859
  • DiseasesDB: 12155
External resources
  • MedlinePlus: 000456
  • eMedicine: med/2136 emerg/537 derm/846 ped/2811 oph/477 oph/695
  • Patient UK: Sjögren's syndrome

  • Sjögren's Syndrome Foundation website
  • Sjögren's Syndrome at NHS Choices
  • Questions and Answers about Sjögren’s Syndrome - US National Institute of Arthritis and Musculoskeletal and Skin Diseases

sjogren's syndrome, sjogren's syndrome autoimmune disease, sjögren's syndrome, sjögren's syndrome and hives, sjögren's syndrome diagnosis, sjögren's syndrome foundation, sjögren's syndrome prognosis, sjögren's syndrome pronunciation, sjögren's syndrome symptoms, sjögren's syndrome treatment

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