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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 -
Ocular Immunology and Inflammation May 2021The purpose of this review is to delve into the clinical and research understanding of the pathophysiology and presentation of Sjögren's-related keratoconjunctivitis... (Review)
Review
The purpose of this review is to delve into the clinical and research understanding of the pathophysiology and presentation of Sjögren's-related keratoconjunctivitis sicca in order address the diagnostic and management challenge that it represents, as well as to provide a basis for appreciating the pharmacotherapies designed to treat the ophthalmic symptoms of Sjögren's disease.
Topics: Dry Eye Syndromes; Humans; Keratoconjunctivitis Sicca; Meibomian Glands; Sjogren's Syndrome; Tears
PubMed: 33830848
DOI: 10.1080/09273948.2021.1903935 -
Seminars in Respiratory and Critical... Jun 2024Sjögren's disease (SjD) is a chronic, progressive autoimmune condition of exocrine and extraglandular tissues. It can present with isolated disease characterized by... (Review)
Review
Sjögren's disease (SjD) is a chronic, progressive autoimmune condition of exocrine and extraglandular tissues. It can present with isolated disease characterized by lymphocytic infiltration of salivary or lacrimal glands, but in approximately one-third of the patients, lymphocytic infiltration extends beyond exocrine glands to involve extraglandular organs such as the lungs. Pulmonary complications have been reported to occur between 9 and 27% of patients with SjD across studies. Respiratory manifestations occur on a spectrum of severity and include airways disease, interstitial lung disease, cystic lung disease, and lymphoma. Lung involvement can greatly affect patients' quality of life, has a major impact on the overall prognosis, and frequently leads to alteration in the treatment plans, highlighting the importance of maintaining a high index of clinical suspicion and taking appropriate steps to facilitate early recognition and intervention.
Topics: Humans; Sjogren's Syndrome; Lung Diseases; Quality of Life; Lung Diseases, Interstitial; Prognosis
PubMed: 38621712
DOI: 10.1055/s-0044-1785675 -
Modern Rheumatology Aug 2023Type I interferons (IFNs) have recently received a lot of attention with the elucidation of the pathogenesis of systemic lupus erythematosus (SLE). Type I IFNs are... (Review)
Review
Type I interferons (IFNs) have recently received a lot of attention with the elucidation of the pathogenesis of systemic lupus erythematosus (SLE). Type I IFNs are associated with many SLE symptoms and play a role in the pathogenesis of autoimmune diseases that may occur concurrently with SLE, such as Sjögren's syndrome, antiphospholipid syndrome, myositis, scleroderma, and interferonopathy. Type I IFNs could be the link between these diseases. However, direct measurement of type I IFN levels and the IFN gene signature is currently unavailable in clinical practice. This review discusses type I IFN signalling in SLE, investigates the role of type I IFN in the clinical manifestations and symptoms associated with SLE and other IFN-related diseases, and discusses the clinical tests that can be used to diagnose SLE and measure disease activity. In addition, the role of type I IFN-blocking therapies as potential treatments for SLE is discussed.
Topics: Humans; Lupus Erythematosus, Systemic; Interferon Type I; Antiphospholipid Syndrome; Signal Transduction; Sjogren's Syndrome
PubMed: 36440704
DOI: 10.1093/mr/roac140 -
Joint Bone Spine Jan 2023
Topics: Humans; Sjogren's Syndrome
PubMed: 36343878
DOI: 10.1016/j.jbspin.2022.105480 -
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 -
Brain and Behavior Aug 2020Sjögren's syndrome (SS) is a chronic inflammatory disease with an autoimmune background with possible complications from peripheral (PNS) and central nervous system...
OBJECTIVES
Sjögren's syndrome (SS) is a chronic inflammatory disease with an autoimmune background with possible complications from peripheral (PNS) and central nervous system (CNS). The aim of this study was to assess the prevalence and to describe the phenotype of peripheral neuropathies in patients with SS.
MATERIALS & METHODS
We studied fifty patients with primary Sjögren's syndrome for peripheral nervous system involvement. All patients underwent neurological and rheumatological examination followed by nerve conduction studies (NCS) of nine peripheral nerves.
RESULTS
Thirty-six patients (72%) fulfilled the criteria for the diagnosis of neuropathy. Carpal tunnel syndrome (54%) and axonal sensorimotor neuropathy (22%) were the most common. Neurological symptoms preceded the diagnosis of SS in eight patients.
CONCLUSIONS
Peripheral neuropathies are frequent in SS patients. Neurologists should be aware of possible autoimmune causes of neuropathies because clinical manifestations of neuropathy may precede the development of other symptoms of the autoimmune disease.
Topics: Humans; Neurologic Examination; Neurology; Peripheral Nervous System Diseases; Sjogren's Syndrome
PubMed: 32583978
DOI: 10.1002/brb3.1665 -
Clinical and Experimental Rheumatology 2021
Topics: Humans; Sjogren's Syndrome
PubMed: 34251305
DOI: 10.55563/clinexprheumatol/8vip9k -
Clinical and Experimental Rheumatology 2021To describe the clinical spectrum of Sjögren's syndrome (SS) patients with combined seronegativity.
OBJECTIVES
To describe the clinical spectrum of Sjögren's syndrome (SS) patients with combined seronegativity.
METHODS
From a multicentre study population of consecutive SS patients fulfilling the 2016 ACR-EULAR classification criteria, patients with triple seronegativity [anti-Ro/SSA(-), anti-La/SSB(-), RF(-) and ANA(+)] and quadruple seronegativity [anti-Ro/SSA(-), anti-La/SSB(-), RF(-) and ANA(-)] were identified retrospectively. Both groups were matched in an 1:1 ratio with 2 distinct control SS groups: i) classic anti-Ro/SSA seropositive patients [SS(+)] and ii) classic anti-Ro/SSA seropositive patients with negative rheumatoid factor [SS(+)/RF(-)] to explore their effect on disease expression. Clinical, laboratory and, histologic features were compared. A comparison between triple and quadruple seronegative SS patients was also performed.
REESULTS
One hundred thirty-five SS patients (8.6%) were identified as triple seronegative patients and 72 (4.5%) as quadruple. Triple seronegative patients had lower frequency of peripheral nervous involvement (0% vs. 7.2% p=0.002) compared to SS(+) controls and lower frequency of interstitial renal disease and higher prevalence of dry mouth than SS(+)/RF(-) controls. Quadruple seronegative patients presented less frequently with persistent lymphadenopathy (1.5% vs. 16.9 p=0.004) and lymphoma (0% vs. 9.8% p=0.006) compared to SS(+) controls and with lower prevalence of persistent lymphadenopathy (1.5% vs. 15.3% p=0.008) and higher frequency of dry eyes (98.6% vs. 87.5% p=0.01) and autoimmune thyroiditis (44.1% vs. 17.1% p=0.02) compared to SS(+)/RF(-) SS controls. Study groups comparative analysis revealed that triple seronegative patients had higher frequency of persistent lymphadenopathy and lymphoma, higher focus score and later age of SS diagnosis compared to quadruple seronegative patients.
CONCLUSIONS
Combined seronegativity accounts for almost 9% of total SS population and is associated with a milder clinical phenotype, partly attributed to the absence of rheumatoid factor.
Topics: Humans; Lymphadenopathy; Retrospective Studies; Rheumatoid Factor; Sjogren's Syndrome
PubMed: 34665703
DOI: 10.55563/clinexprheumatol/47a4kr -
European Journal of Ophthalmology Nov 2022Sjögren's syndrome is an autoimmune disease, and its important feature is the lymphocyte infiltration of exocrine glands, including lacrimal glands. It contributes to... (Review)
Review
BACKGROUND
Sjögren's syndrome is an autoimmune disease, and its important feature is the lymphocyte infiltration of exocrine glands, including lacrimal glands. It contributes to defects of their activity and causes that one of the main manifestation of Sjögren's syndrome is dry eye. Unfortunately, the discrimination between dry eye related and non-related to Sjögren's syndrome is difficult at the initial stages of diseases. In addition, the available agents for the treatment of Sjögren's syndrome-related dry eye have limited efficacy.
AIM
The purpose of this study was to describe and emphasize differences between Sjögren's Syndrome-related dry eye and non-Sjögren's Syndrome-related dry eye together with the determination of novel therapeutic options for Sjögren's Syndrome-related dry eye.
METHOD
A review of the relevant papers describing characteristics of Sjögren's Syndrome-related dry eye and its therapy was conducted. This article is based on both pre-clinical and clinical evidences.
RESULTS
On the basis of our analysis, we indicated differences between Sjögren's Syndrome-related dry eye and non-Sjögren's Syndrome-related dry eye. Moreover, there are some novel markers that could be used in the diagnosis of Sjögren's Syndrome-related dry eye. In addition, expect artificial tear, other agents e.g. hydroxychloroquine can be effective in therapy of disease.
CONCLUSIONS
Sjögren's Syndrome-related dry eye is a disorder, whose diagnosis may be difficult and mistaken for non-Sjögren's Syndrome-related dry eye. However, Sjögren's Syndrome-related dry eye has some specific features. In addition, the development of newer and safer therapeutic agents for Sjögren's syndrome-related dry eye is needed, and therefore further clinical, randomized studies are necessary.
Topics: Dry Eye Syndromes; Humans; Hydroxychloroquine; Lubricant Eye Drops; Sjogren's Syndrome; Tears
PubMed: 35354331
DOI: 10.1177/11206721221091375