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Seminars in Respiratory and Critical... Aug 2007Sjögren's syndrome (SS) is a chronic, slowly progressive, inflammatory, autoimmune disease characterized by (1) lymphocytic infiltration of the exocrine glands leading... (Review)
Review
Sjögren's syndrome (SS) is a chronic, slowly progressive, inflammatory, autoimmune disease characterized by (1) lymphocytic infiltration of the exocrine glands leading to diminished or absent glandular secretion, and (2) marked B-lymphocytic cell hyperreactivity manifested initially by a variety of serum autoantibodies, including those against the Ro(SSA) and La(SSB) ribonucleoproteins, ending in the development of B cell non-Hodgkin's lymphoma in a substantial number of patients. Most patients with SS present only with keratoconjunctivitis sicca and xerostomia. However, approximately 40% develop extraglandular manifestations that present in two ways: (1) the development of lymphoepithelial lesions in several extra-exocrine gland tissues (i.e., bronchi, renal tubules, or biliary ducts), and (2) vasculitis related to the deposition of immune complexes due to B cell hyperreactivity. Pulmonary manifestations develop in some patients and may present as (1) bronchitis sicca; (2) a wide spectrum of lymphoproliferative diseases, ranging from bronchus-associated lymphoid tissue (BALT) hyperplasia, lymphoid interstitial pneumonia, and B cell non-Hodgkin's lymphoma mainly of the extranodal marginal zone B-cell lymphoma of BALT-type or rarely of higher-grade malignancy; and (3) other interstitial pneumonias. Pleuritis can be seen in SS patients with associated systemic lupus erythematosus or rheumatoid arthritis.
Topics: Humans; Lung Diseases, Interstitial; Lymphoproliferative Disorders; Prognosis; Respiratory Function Tests; Respiratory Tract Diseases; Sjogren's Syndrome
PubMed: 17764063
DOI: 10.1055/s-2007-985667 -
QJM : Monthly Journal of the... Dec 2012Symptoms in keeping with autonomic dysfunction are commonly described by primary Sjögrens syndrome patients (pSS); whether objective abnormalities of autonomic function...
BACKGROUND
Symptoms in keeping with autonomic dysfunction are commonly described by primary Sjögrens syndrome patients (pSS); whether objective abnormalities of autonomic function occur is unclear. This study set out to explore dynamic cardiovascular autonomic responses in pSS and their relationship with symptoms and quality of life.
METHODS
Twenty-one people from the UK pSS registry, 21 community controls and 21 patients with the autoimmune liver disease primary biliary cirrhosis (PBC) (matched case-wise for age and sex) attended for assessment of autonomic responses to orthostasis and Valsalva manoeuvre (VM). pSS patients also completed EULAR Sjögrens Syndrome patient-reported index (ESSPRI), EULAR Sjögren's syndrome disease activity index (ESSDAI), fatigue impact scale and EURO-QOL 5-dimension (EQ-5D).
RESULTS
Compared with controls, pSS patients had significantly lower baseline systolic blood pressure (SBP) (114 ± 13 vs. 127 ± 20; P = 0.02), which dropped to a significantly lower value (98 ± 22 vs. 119 ± 24, P = 0.009). When area under the curve (AUC) was calculated for when the SBP was below baseline this was significantly greater in pSS compared to both control groups (pSS vs. control vs. PBC: 153 ± 236 vs. 92 ± 85 vs. 1.2 ± 0.3, P = 0.005). Peak phase IV SBP during the VM was significantly lower in pSS (P = 0.007) indicating early sympathetic failure. Increased heart rate associated with fatigue (P = 0.02; r(2) = 0.2) and EQ-5D. A shift in sympathetic-vagal balance associated with overall symptom burden (ESSPRI) (P = 0.04, r(2) = 0.3) and EULAR sicca score (P = 0.016; r(2) = 0.3), the latter also correlated with baroreceptor effectiveness (P = 0.03; r(2) = 0.2) and diastolic blood pressure variability (P = 0.003; r(2) = 0.4).
CONCLUSION
pSS patients have impaired blood pressure response to standing. Dysautonomia correlates with PSS-associated symptoms and quality of life.
Topics: Adult; Aged; Area Under Curve; Autonomic Nervous System; Blood Pressure; Case-Control Studies; Dizziness; Fatigue; Female; Humans; Hypotension, Orthostatic; Male; Middle Aged; Quality of Life; Sjogren's Syndrome; Valsalva Maneuver
PubMed: 22976617
DOI: 10.1093/qjmed/hcs172 -
Journal of Clinical Laboratory Analysis Feb 2021Primary Sjögren's syndrome (pSS) is an autoimmune disease characterized by a lymphocytic infiltrate in salivary glands driving to epithelial damage. The pSS patients...
BACKGROUND
Primary Sjögren's syndrome (pSS) is an autoimmune disease characterized by a lymphocytic infiltrate in salivary glands driving to epithelial damage. The pSS patients present heterogenic clinical and serological characteristics. This heterogenicity could be due to the cytokine microenvironment. Cytokine levels have been analyzed and reported individually, showing controversial results; for that reason, we considered essential to evaluate a cluster of cytokines and relate them with antibody levels and clinical characteristics to find pSS subgroups.
METHODS
Ninety-nine pSS patients, diagnosed by the 2016 ACR/EULAR classification criteria, and 76 control subjects (CS) were included. Cytokine quantification was performed by Multiplex assay. Principal component analysis (PCA) was realized, and the K-mean test was used to identify clusters/groups. Groups were analyzed by the Kruskal-Wallis test and the Bonferroni test.
RESULTS
Higher IFN-γ, IL-17F, IL-21, IL-23, IL-4, and IL-31 levels were observed in pSS patients in comparison with control subjects. PCA analysis showed three groups. The severe group was characterized by higher cytokine concentrations as well as an increase in clinical parameters such as antibody levels, damage index score, and others. The moderate group presented intermediate severity; meanwhile, the mild group presented the lowest severity.
CONCLUSION
Cluster analysis revealed three groups that were different in cytokine levels and clinical parameters in which the mild group was defined by lower severity, the moderate group with intermediate severity, and the severe group with higher severity. This analysis could help subclassify the primary Sjögren syndrome patients for a better understanding of the clinical phenotype that impacts the treatment approach.
Topics: Adult; Aged; Case-Control Studies; Cytokines; Female; Humans; Male; Middle Aged; Principal Component Analysis; Severity of Illness Index; Sjogren's Syndrome
PubMed: 33070375
DOI: 10.1002/jcla.23629 -
Neurosciences (Riyadh, Saudi Arabia) Jul 2019To use the Montreal Cognitive Assessment (MoCA) test to assess the subclinical cognitive impairment in patients with Primary Sjogren`s Syndrome (PSS) and assess the...
OBJECTIVE
To use the Montreal Cognitive Assessment (MoCA) test to assess the subclinical cognitive impairment in patients with Primary Sjogren`s Syndrome (PSS) and assess the correlation of MoCA results with magnetic resonance imaging (MRI) findings in these patients.
METHODS
The MoCA test was prospectively administered to 32 consecutive patients (31 females, 1 male) diagnosed with PSS and 30 healthy controls (29 females, 1 male) at Antalya Education and Research Hospital between June 2014 and October 2015. Twenty PSS patients underwent a brain MRI (T1, T2, and T2- FLAIR-weighted sequences).
RESULTS
The mean age was 45.84 (range 24-63) in the PSS group, and the mean duration of disease was 3.5 years (4 months - 18 years). There were 22 patients (68.80%) with 5-8 years of education and 10 patients (31.30%) with more than 8 years of education. The mean age was 42.8 (28-64) in the control group. There were 20 controls (66.70%) with 5-8 years of education and 10 controls (33.3%) with more than 8 years of education. The delayed recall rate of the patient group with 5-8 years of education was significantly lower than that of the control group, and the recall rate with multiple choice cues for the same patient group was significantly higher than that of the control group (p less than 0.05). There was no correlation between the number of lesions and total MoCA score or subgroups.
CONCLUSION
We suggest that the MoCA test is a single-page, easy-to-administer test, can be used to assess cognition in patients with PSS especially in large groups.
Topics: Adult; Cognition; Female; Humans; Magnetic Resonance Imaging; Male; Middle Aged; Neuropsychological Tests; Sjogren's Syndrome
PubMed: 31380819
DOI: 10.17712/nsj.2019.3.20180027 -
The Indian Journal of Tuberculosis Jan 2022To present a case of Sjogren syndrome with pulmonary manifestations in an adult female and discuss its assessment and management.
OBJECTIVE
To present a case of Sjogren syndrome with pulmonary manifestations in an adult female and discuss its assessment and management.
DESIGN
Case Report.
SETTING
Tertiary care hospital.
PATIENT
One.
RESULTS
A 50 yrs female admitted with complaints of dryness of eyes with decreased salivation causing difficulty in swallowing since last 3 years, with persistent dry cough since 10-15 days and progressive dyspnea since 4-5 days. Anti-nuclear antibody (ANA) profile revealed Anti- Ro/SS-A and Anti- La/SS-B Positive. Also, sub-lingual excisional biopsy was done which was consistent with findings of Sjogren's syndrome. Patient showed significant improvement after starting oral glucocorticoids, systemic anti inflammatory agents (Tab. HCQS), artificial tear drops, oral iron supplements and other supportive treatment.
CONCLUSION
Sjögren syndrome (SS) is a chronic inflammatory disorder characterized by diminished lacrimal and salivary gland function and associated with lymphocytic infiltration of exocrine glands, and can affect extraglandular organ systems including the skin, lung, heart, kidney, neural, and hematopoietic systems. We present a case of Sjogren syndrome in an adult female presenting with xerostomia and dyspnea and was diagnosed upon detection of anti-Ro and anti-La antibodies and confirmed by histopathological examination of lip biopsy. Patient was started on oral steroids and other supportive treatment, General condition improved significantly and is doing very well on regular follow-up.
Topics: Adult; Antibodies, Antinuclear; Biopsy; Female; Humans; Lung; Sjogren's Syndrome
PubMed: 35074142
DOI: 10.1016/j.ijtb.2020.12.004 -
Scientific Reports May 2020In primary Sjögren's syndrome (pSS) the exocrine glands become infiltrated with lymphocytes instigating severe damage to the salivary and lacrimal glands causing dry... (Clinical Trial)
Clinical Trial
In primary Sjögren's syndrome (pSS) the exocrine glands become infiltrated with lymphocytes instigating severe damage to the salivary and lacrimal glands causing dry eyes and dry mouth. Previous investigations have suggested that dysregulated localized and systemic inflammation contributes to the development and pathogenesis of pSS. A miR microarray performed in primary human conjunctival epithelial cells (PECs) demonstrated significant differences in miR expression at the ocular surface between pSS patients and healthy controls. MicroRNA-744-5p (miR-744-5p) was identified as being of particular interest, as its top predicted target is Pellino3 (PELI3), a known negative regulator of inflammation. Validation studies confirmed that miR-744-5p expression is significantly increased in PECs from pSS patients, whilst PELI3 was significantly reduced. We validated the miR-744 binding site in the 3' untranslated region (UTR) of PELI3 and demonstrated that increasing PELI3 levels with a miR-744-5p antagomir in an inflammatory environment resulted in reduced levels of IFN dependent chemokines Rantes (CCL5) and CXCL10. These results reveal a novel role for miR-744-5p in mediating ocular inflammation via Pellino3 expression in pSS patients and suggest that miR-744-5p may be a potential therapeutic target for the management of severe dry eye disease and ocular inflammation in pSS patients.
Topics: Adult; Aged; Chemokine CCL5; Chemokine CXCL10; Dry Eye Syndromes; Female; Humans; Inflammation; Lacrimal Apparatus; Male; MicroRNAs; Middle Aged; Sjogren's Syndrome
PubMed: 32366870
DOI: 10.1038/s41598-020-64422-5 -
Clinical Immunology (Orlando, Fla.) Sep 2017
Topics: Animals; Biomedical Research; Humans; Research; Sjogren's Syndrome
PubMed: 28673862
DOI: 10.1016/j.clim.2017.06.011 -
Journal of Immunology Research 2019
Topics: Animals; Disease Models, Animal; Humans; Sjogren's Syndrome
PubMed: 31236421
DOI: 10.1155/2019/8101503 -
Advances in Experimental Medicine and... 1998
Review
Topics: Animals; Apoptosis; Autoimmune Diseases; Cytokines; Humans; Lacrimal Apparatus; Mice; Mice, Knockout; Salivary Glands; Sjogren's Syndrome; Transforming Growth Factor beta
PubMed: 9634993
DOI: 10.1007/978-1-4615-5359-5_135 -
Praxis Nov 2012
Review
Topics: Combined Modality Therapy; Cooperative Behavior; Diagnosis, Differential; Humans; Interdisciplinary Communication; Patient Care Team; Prognosis; Sjogren's Syndrome
PubMed: 23184549
DOI: 10.1024/1661-8157/a001133