scholarly journals Cutting the “Gordian Knot” — Cardiac Involvement in Primary Sjögren Syndrome

2021 ◽  
pp. jrheum.201171
Author(s):  
George Markousis-Mavrogenis ◽  
Sophie I. Mavrogeni
Keyword(s):  
Myocardial Infarction ◽  
Cardiovascular Disease ◽  
Autoimmune Disease ◽  
Cardiac Involvement ◽  
Sjögren Syndrome ◽  
Systemic Autoimmune Disease ◽  
Sjogren Syndrome ◽  
Exocrine Glands ◽  
Primary Sjögren Syndrome ◽  
Increased Risk

Sjögren syndrome (SS) is a systemic autoimmune disease typically characterized by inflammatory involvement of the exocrine glands1. The association of SS with an increased risk of cardiovascular disease (CVD) including manifestations such as stroke and myocardial infarction has been demonstrated by numerous previous studies1.

scholarly journals Salivary 1H-NMR Metabolomics in Primary Sjögren Syndrome. Preliminary Results of a Pilot Case-Control Study

Proceedings ◽  
2019 ◽  
Vol 35 (1) ◽  
pp. 39
Author(s):  
Setti ◽  
Sandri ◽  
Tarentini ◽  
Panari ◽  
Mucci ◽  
...  
Keyword(s):  
Autoimmune Disease ◽  
1H Nmr ◽  
Case Control Study ◽  
Case Control ◽  
Sjögren Syndrome ◽  
Sjogren Syndrome ◽  
Exocrine Glands ◽  
Preliminary Results ◽  
Primary Sjögren Syndrome ◽  
Control Study

Primary Sjögren Syndrome (pSS) is a multisystem autoimmune disease which mainly involves exocrine glands, such as salivary and lacrimal. [...]

scholarly journals Primary Sjogren syndrome increases the risk of bisphosphonate-related osteonecrosis of the jaw

2021 ◽  
Vol 11 (1) ◽  
Author(s):  
Pei-I Kuo ◽  
Tzu-Min Lin ◽  
Yu-Sheng Chang ◽  
Tsung-Yun Hou ◽  
Hui-Ching Hsu ◽  
...  
Keyword(s):  
Propensity Score ◽  
Osteonecrosis Of The Jaw ◽  
Sjögren Syndrome ◽  
Cox Proportional Hazards Model ◽  
Matched Pair ◽  
Sjogren Syndrome ◽  
Research Database ◽  
Primary Sjögren Syndrome ◽  
Defined Daily Doses ◽  
Increased Risk

AbstractThe risk of bisphosphonate-related osteonecrosis of the jaw (BRONJ) in primary Sjogren syndrome (pSS) has rarely been explored. To explore the association between BRONJ and pSS, we conducted a population-based propensity-score-matched cohort study using Taiwan’s National Health Insurance Research Database, including pSS patients receiving antiosteoporotic therapy and patients without pSS receiving antiosteoporotic therapy. A 1:4 matched-pair cohort based on propensity score was created. The stratified Cox proportional hazards model compared the risk of BRONJ in the pSS and non-pSS groups. In the study, 23,280 pSS patients and 28,712,152 controls were enrolled. After matching, 348 patients with pSS receiving antiosteoporotic drugs and 50,145 without pSS receiving antiosteoporotic drugs were included for analysis. The risk of developing BRONJ was 1.96 times higher in pSS patients compared with non-pSS patients after adjustment for age, sex, and comorbidities. No dose–response effect was observed in the bisphosphonate-treated pSS cohorts, documented as the cumulative defined daily doses of either < 224 or ≥ 224 (hazard ratio [HR]: 2.407, 95% confidence interval [CI] 1.412–7.790; HR: 2.143, 95% CI 1.046–4.393, respectively) increased risk of developing osteonecrosis of the jaw. In conclusion, the risk of BRONJ is significantly higher in patients with pSS compared with the general population.

scholarly journals The Risk of Deep Venous Thrombosis and Pulmonary Embolism in Primary Sjögren Syndrome: A General Population-based Study

2017 ◽  
Vol 44 (8) ◽  
pp. 1184-1189 ◽  
Author(s):  
J. Antonio Aviña-Zubieta ◽  
Michael Jansz ◽  
Eric C. Sayre ◽  
Hyon K. Choi
Keyword(s):  
Pulmonary Embolism ◽  
General Population ◽  
Population Based ◽  
Sjögren Syndrome ◽  
First Year ◽  
Study Cohort ◽  
Sjogren Syndrome ◽  
Primary Sjögren Syndrome ◽  
Entry Time ◽  
Increased Risk

Objective.To estimate the future risk and time trends of venous thromboembolism (VTE) in individuals with newly diagnosed primary Sjögren syndrome (pSS) in the general population.Methods.Using a population database that includes all residents of British Columbia, Canada, we created a study cohort of all patients with incident SS and up to 10 controls from the general population matched for age, sex, and entry time. We compared incidence rates (IR) of pulmonary embolism (PE), deep vein thrombosis (DVT), and VTE between the 2 groups according to SS disease duration. We calculated HR, adjusting for confounders.Results.Among 1175 incident pSS cases (mean age 56.7 yrs, 87.6% women), the IR of PE, DVT, and VTE were 3.9, 2.8, and 5.2 per 1000 person-years (PY), respectively; the corresponding rates in the comparison cohort were 0.9, 0.8, and 1.4 per 1000 PY. Compared with non-SS individuals, the multivariable HR for PE, DVT, and VTE among SS cases were 4.07 (95% CI 2.04–8.09), 2.80 (95% CI 1.27–6.17), and 2.92 (95% CI 1.66–5.16), respectively. The HR matched for age, sex, and entry time for VTE, PE, and DVT were highest during the first year after SS diagnosis (8.29, 95% CI 2.57–26.77; 4.72, 95% CI 1.13–19.73; and 7.34, 95% CI 2.80–19.25, respectively).Conclusion.These findings provide population-based evidence that patients with pSS have a substantially increased risk of VTE, especially within the first year after SS diagnosis. Further research into the involvement of monitoring and prevention of VTE in SS may be warranted.

scholarly journals Cerebral Venous Thrombosis Revealing Primary Sjögren Syndrome: Report of 2 Cases

2013 ◽  
Vol 2013 ◽  
pp. 1-4 ◽  
Author(s):  
A. Mercurio ◽  
M. Altieri ◽  
V. M. Saraceni ◽  
T. Paolucci ◽  
G. L. Lenzi
Keyword(s):  
Nervous System ◽  
Venous Thrombosis ◽  
Cerebral Venous Thrombosis ◽  
Lymphocytic Infiltration ◽  
Sjögren Syndrome ◽  
Sjogren Syndrome ◽  
Exocrine Glands ◽  
Neurologic Complications ◽  
Primary Sjögren Syndrome ◽  
Syndrome Report

Sjögren syndrome (SS) is an autoimmune disease of the exocrine glands, characterized by focal lymphocytic infiltration and destruction of these glands. Neurologic complications are quite common, mainly involving the peripheral nervous system (PNS). The most common central nervous system (CNS) manifestations are myelopathy and microcirculation vasculitis. However, specific diagnostic criteria for CNS SS are still lacking. We report two cases of primary SS in which the revealing symptom was cerebral venous thrombosis (CVT) in the absence of genetic or acquired thrombophilias.

scholarly journals Lung complications of Sjogren syndrome

2020 ◽  
Vol 29 (157) ◽  
pp. 200021
Author(s):  
Fabrizio Luppi ◽  
Marco Sebastiani ◽  
Nicola Sverzellati ◽  
Alberto Cavazza ◽  
Carlo Salvarani ◽  
...  
Keyword(s):  
Lymphocytic Infiltration ◽  
Airway Disease ◽  
Pulmonary Involvement ◽  
Sjögren Syndrome ◽  
Systemic Autoimmune Disease ◽  
Sjogren Syndrome ◽  
Recent Trial ◽  
Risk Of Death ◽  
Primary Sjögren Syndrome ◽  
Related Quality

Primary Sjogren syndrome (pSS) is a systemic autoimmune disease characterised by lymphocytic infiltration of exocrine glands and by a number of systemic manifestations, including those regarding the lung. Pulmonary involvement in pSS includes interstitial lung disease (ILD) and airway disease, together with lymphoproliferative disorders.Patients with pSS-ILD report impaired health-related quality of life and a higher risk of death, suggesting the importance of early diagnosis and treatment of this type of pulmonary involvement. In contrast, airway disease usually has little effect on respiratory function and is rarely the cause of death in these patients.More rare disorders can be also identified, such as pleural effusion, cysts or bullae.Up to date, available data do not allow us to establish an evidence-based treatment strategy in pSS-ILD. No data are available regarding which patients should be treated, the timing to start therapy and better therapeutic options. The lack of knowledge about the natural history and prognosis of pSS-ILD is the main limitation to the development of clinical trials or shared recommendations on this topic. However, a recent trial showed the efficacy of the antifibrotic drug nintedanib in slowing progression of various ILDs, including those in pSS patients.

Prognostic markers of lymphoma development in primary Sjögren syndrome

Lupus ◽  
2019 ◽  
Vol 28 (8) ◽  
pp. 923-936 ◽  
Author(s):  
S Retamozo ◽  
P Brito-Zerón ◽  
M Ramos-Casals
Keyword(s):  
Risk Factors ◽  
Lupus Erythematosus ◽  
Activity Index ◽  
Sjögren Syndrome ◽  
Systemic Autoimmune Disease ◽  
Low Grade ◽  
Sjogren Syndrome ◽  
Close Link ◽  
Systemic Involvement ◽  
Primary Sjögren Syndrome

Sjögren syndrome is a systemic autoimmune disease that principally affects women between the fourth and sixth decades of life who present with sicca symptomatology caused by dryness of the main mucosal surfaces. The clinical spectrum of Sjögren syndrome extends from dryness to systemic involvement. Since 1978, Sjögren syndrome has been closely associated with an enhanced risk of lymphoma, one of the most severe complications a patient may develop. Primary Sjögren syndrome patients have a 10–44-fold greater risk of lymphoma than healthy individuals, higher than that reported for systemic lupus erythematosus and rheumatoid arthritis. The close link between lymphoma and Sjögren syndrome is clearly exemplified by the very specific type of lymphoma arising in Sjögren syndrome patients, mainly low-grade B-cell lymphomas (predominantly a marginal zone histological type) with primary extranodal involvement of the major salivary glands (overwhelmingly parotid), with a primordial role of cryoglobulinemic-related markers (both clinical and immunological). The most recent studies support a higher number of risk factors detected in an individual leads to a higher lymphoma risk. A close follow-up of high-risk groups with longitudinal assessments of all known risk factors, including cryoglobulin-related markers and EULAR Sjögren's syndrome disease activity index measurement in particular, is mandatory.

P083 Primary Goujerot- Sjögren Syndrome: a pediatric case report

Rheumatology ◽  
2021 ◽  
Vol 60 (Supplement_5) ◽  
Author(s):  
Djohra Hadef ◽  
Samy Slimani ◽  
Yamina Ouchen
Keyword(s):  
Autoimmune Disease ◽  
Clinical Signs ◽  
Lymphocytic Infiltration ◽  
Initial Therapy ◽  
Sjögren Syndrome ◽  
Lower Limbs ◽  
Systemic Autoimmune Disease ◽  
Sjogren Syndrome ◽  
Organ Systems ◽  
One Year

Abstract Background Gourgerot-Sjögren syndrome (GSS) is a systemic autoimmune disease characterized by lymphocytic infiltration of the exocrine glands leading to dryness of the mucous membranes. It may also involve other organs and organ systems. GSS can be primary (pGSS) or secondary due to other autoimmune diseases. pSGS mainly affects women over 40 years old, but can occur at any age. GSS is rare in children and is often secondary to other diseases. We report the case of a 12-year-old girl with primary Gougerot-Sjögren Syndrome Methods and results A 12-year-old girl from Batna (Algeria) presented to her pediatrician in 2016 for a rash with arthralgia. She had no relevant past medical history and is the child of a non-consanguineous marriage. Clinical examination found a well-nourished child with a purpuric rash on her lower limbs. She complained of arthralgia without clinical signs of arthritis. The child also complained of a sensation of a foreign body and burning in both eyes. The eye exam was normal except for a positive Schirmer's test. There were no signs of parotitis. Laboratory tests showed an erythrocyte sedimentation rate (ESR) of 84 mm the first h and a negative C-reactive protein (CRP). She was also Leukopenic. Serological testing resulted in a positive Anti SS-A and Anti SS-B. The abdominal pelvic ultrasound was normal. The patient was diagnosed with primary Gougerot-Sjögren Syndrome. Artificial tears and hydroxychloroquine were started as initial therapy. One year later we added an immunosuppressant (Methotrexate) due to the persistence of clinical signs. Conclusion Our case is particular in that primary Gougerot-Sjögren Syndrome is rare in children. Routine follow- up with this patient is important to determine whether it is indeed pGSS with pediatric onset or GSS secondary to another autoimmune disease.

scholarly journals Patients with chronic periodontitis present increased risk for primary Sjögren syndrome: a nationwide population-based cohort study

PeerJ ◽  
2018 ◽  
Vol 6 ◽  
pp. e5109 ◽  
Author(s):  
Tai-Chen Lin ◽  
Chien-Fang Tseng ◽  
Yu-Hsun Wang ◽  
Hui-Chieh Yu ◽  
Yu-Chao Chang
Keyword(s):  
Cohort Study ◽  
General Population ◽  
Chronic Periodontitis ◽  
Population Based ◽  
Sjögren Syndrome ◽  
Female Group ◽  
Sjogren Syndrome ◽  
Cox Regression Analysis ◽  
Primary Sjögren Syndrome ◽  
Increased Risk

Many reports have mentioned the association between chronic periodontitis (CP) and primary Sjögren syndrome (pSS). However, no cohort study has been performed for the risk of pSS in patients with CP. In this study, we evaluated the risk of pSS from CP exposure in a nationwide population-based cohort study in Taiwan. We studied the claims data of Taiwanese population from 2001 to 2012. We identified 76,765 patients with CP from the National Health Insurance Database in Taiwan. We also selected 76,765 controls that were randomly frequency matched by age, sex, and index year from the general population. We analyzed the risk of pSS by using Cox proportional hazards regression models including sex, age, and comorbidities. In this study, 76,765 patients with CP (mean age: 40.8 years) and 76,765 controls (mean age: 41.0 years) were followed-up for 8.54 and 8.49 years, respectively. A total of 869 cases of pSS were identified in CP cohort and 483 cases in non-CP cohort. Multivariate Cox regression analysis indicated that the incidence rate of pSS was significantly higher in CP cohort than those who in non-CP cohort (adjusted HR: 1.79, 95% CI [1.60–2.00]). Taken together, this nationwide retrospective cohort study demonstrated that the risk of pSS was significantly higher in patients with CP than in the general population. The association between CP and pSS was significant in the female group.

scholarly journals Mechanisms of Disease in Sjögren Syndrome—New Developments and Directions

2020 ◽  
Vol 21 (2) ◽  
pp. 650
Author(s):  
Cintia S. de Paiva ◽  
Stephen C. Pflugfelder
Keyword(s):  
Autoimmune Disease ◽  
Sjögren Syndrome ◽  
Sjogren Syndrome ◽  
Exocrine Glands ◽  
Lacrimal Glands ◽  
New Developments

Sjögren Syndrome (SS) is an autoimmune disease that affects the exocrine glands, mainly salivary and lacrimal glands [...]

Neurophysiological Features of Peripheral Nervous System Involvement and Immunological Profile of Patients with Primary Sjögren Syndrome

2020 ◽  
Vol 47 (11) ◽  
pp. 1661-1667 ◽  
Author(s):  
Joanna Perzyńska-Mazan ◽  
Maria Maślińska ◽  
Robert Gasik
Keyword(s):  
Peripheral Neuropathy ◽  
Axonal Neuropathy ◽  
Sjögren Syndrome ◽  
Sjogren Syndrome ◽  
Primary Sjögren Syndrome ◽  
Immunological Tests ◽  
Increased Risk ◽  
Independent Association ◽  
Electrophysiological Examination ◽  
The Relationship

ObjectiveThe aim of this study was to evaluate the prevalence, type of neuropathy, and the relationship between the presence of autoantibodies and neuropathy development in patients with primary Sjögren syndrome (pSS).MethodsSixty-one patients with pSS underwent a complete neurological and electrophysiological examination as well as immunological tests including rheumatoid factor (RF) and autoantibodies such as antinuclear antibodies (ANA), anti-Ro/SSa, and anti-La/SSB antibodies.ResultsThe axonal loss or demyelination were found in 39 patients (63.9%). Twenty-nine (47.5%) subjects fulfilled both clinical and electrophysiological criteria of peripheral neuropathy of predominantly axonal type. Seropositivity to both anti-Ro and anti-La antibodies was more frequently found in patients with normal nerve conduction study. Seropositivity to anti-Ro alone was present in the majority of patients with axonal neuropathy (P < 0.05). The presence of RF was associated with several electrodiagnostic signs of demyelination (P < 0.01). The ANA titer showed no independent association with neuropathy.ConclusionPeripheral neuropathy is a frequent complication in patients with pSS. Seropositivity limited to anti-Ro is associated with increased risk of axonal neuropathy in comparison to seropositivity to both anti-Ro and anti-La antibodies. Seropositivity to RF may contribute to demyelination.

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