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CMAJ : Canadian Medical Association... Oct 2014
Review
Topics: Diagnosis, Differential; Humans; Risk Factors; Sjogren's Syndrome
PubMed: 24566651
DOI: 10.1503/cmaj.122037 -
Central nervous system disease in primary Sjogrens syndrome: the role of magnetic resonance imaging.Seminars in Arthritis and Rheumatism Dec 2004To examine the frequency of central nervous system (CNS) disease in primary Sjogrens syndrome (pSS) and indicate ways in which cerebral magnetic resonance imaging (MRI)... (Review)
Review
OBJECTIVES
To examine the frequency of central nervous system (CNS) disease in primary Sjogrens syndrome (pSS) and indicate ways in which cerebral magnetic resonance imaging (MRI) may help determine the significance of CNS involvement.
METHODS
The current review was based on a Medline (Pubmed) literature search through May 2003, focused on Sjogrens syndrome, other vasculitides, multiple sclerosis (MS), specific MRI techniques, and MRI findings with regard to the above-mentioned diseases. Additional literature was identified in the reference sections of articles listed in Medline.
RESULTS
Severe CNS manifestations reminiscent of MS have been described in pSS patients. Moreover, the prevalence of nonfocal neuropsychological abnormalities has been found to be elevated in some pSS patient populations. MRI studies suggest discrete cerebral tissue damage even in neurologically asymptomatic patients. However, small white matter lesions are nonspecific and may be related to age or cerebrovascular risk factors such as hypertension. A large controlled study, complementing established T2-weighted MRI with fluid-attenuated inversion recovery (FLAIR) to achieve high sensitivity in lesion detection, could indicate the disease specificity of white matter lesions in pSS. Newer MR techniques, such as spectroscopy and magnetization transfer imaging, applied, for example, in MS and systemic lupus erythematosus (SLE) to evaluate CNS tissue injury, could help determine the extent and mechanisms of macroscopic and microscopic CNS lesions in pSS.
CONCLUSIONS
Future controlled studies will be necessary to more precisely estimate the prevalence of CNS lesions in pSS, specifically of discrete white matter abnormalities. Newer MRI techniques have the potential to provide information on the severity and pathophysiological mechanisms of CNS tissue damage.
Topics: Brain; Humans; MEDLINE; Magnetic Resonance Imaging; Multiple Sclerosis; Sjogren's Syndrome
PubMed: 15609267
DOI: 10.1016/j.semarthrit.2004.07.005 -
Acta Oto-laryngologica 1976Sjögren's syndrome is a multisystemic connective tissue disease. The syndrome is associated with other major connective tissue disorders, such as rheumatoid arthritis,...
Sjögren's syndrome is a multisystemic connective tissue disease. The syndrome is associated with other major connective tissue disorders, such as rheumatoid arthritis, systemic lupus erythematosus, systemic sclerosis and others, Reticulo-endothelial malignances have repeatedly been reported in patients with Sjögren's syndrome. A clinical study of 20 patients with Sjögren's syndrome was performed in the Department of otolaryngology, Rikshospitalet, Oslo, Norway. A considerable complexity of symptoms was seen. One patient with malignant lymphoma and one with a thyroid carcinoma were encountered.
Topics: Adolescent; Adult; Aged; Child; Female; Humans; Male; Middle Aged; Prognosis; Sjogren's Syndrome
PubMed: 983688
DOI: 10.3109/00016487609120903 -
Deutsche Medizinische Wochenschrift... Apr 2016Sjögren's syndrome is a frequent autoimmune disease and is associated with a lymphocytic infiltration of the lachrymal and salivary glands and with sicca symptoms. Its... (Review)
Review
Sjögren's syndrome is a frequent autoimmune disease and is associated with a lymphocytic infiltration of the lachrymal and salivary glands and with sicca symptoms. Its diagnostic procedures consist of the quantification of tear and saliva production, identification of extraglandular manifestations, measurement of antibodies against SS-A and SS-B and of salivary gland biopsies. Dry eyes and dry mouth are treated only symptomatically, whereas extraglandular manifestations are treated similar to systemic lupus erythematosus.
Topics: Diagnosis, Differential; Humans; Sjogren's Syndrome
PubMed: 27078243
DOI: 10.1055/s-0041-111436 -
Current Opinion in Rheumatology Sep 1995The literature published over the past year on Sjögren's syndrome is reviewed, including epidemiology, genetic, environmental, and clinical features. The criteria for... (Review)
Review
The literature published over the past year on Sjögren's syndrome is reviewed, including epidemiology, genetic, environmental, and clinical features. The criteria for the classification of Sjögren's syndrome remain controversial, potentially leading to confusion in clinical practice and in research publications. Dryness of the eyes and mouth can result from either interruption of the neurovascular innervation of the glands or from any infiltrative process that affects the ability of the glands to secrete. Recent studies have demonstrated that sicca symptoms also can result from autonomic neuropathy in patients with diabetes, multiple sclerosis, or systemic lupus erythematosus. It is suggested that the term Sjögren's syndrome be used to describe one subset of patients with sicca symptoms who exhibit particular major histocompatibility complex antigens, the presence of T cell lymphoid infiltrates on glandular biopsy, and specific autoantibodies in their sera. Even using these restrictive criteria for classification, no single environmental factor has been shown as necessary or sufficient for pathogenesis. Recent studies on Epstein-Barr virus have indicated a novel deleted virus in some Chinese Sjögren's syndrome patients. Other patients with sicca symptoms and autoimmune features may have infections with HIV or hepatitis C virus.
Topics: Animals; Disease Models, Animal; Environment; Humans; Major Histocompatibility Complex; Sjogren's Syndrome
PubMed: 8519614
DOI: 10.1097/00002281-199509000-00008 -
Expert Review of Clinical Immunology Oct 2022Neurologic manifestations in primary Sjogren's Syndrome (pSS) are characterized by a heterogeneity of clinical manifestations. In clinical practice, physicians are... (Review)
Review
INTRODUCTION
Neurologic manifestations in primary Sjogren's Syndrome (pSS) are characterized by a heterogeneity of clinical manifestations. In clinical practice, physicians are challenged with the absence of diagnostic criteria and the lack of clinical trials to support treatment. In this article, we will review the epidemiology, clinical and immunological characterization, diagnosis, and treatment of neurologic events in pSS.
AREAS COVERED
This narrative review provides an overview of the neurologic manifestations described in PSS, as well as complementary investigations and treatments reported. Articles were selected from PubMed searches conducted between December 2021 and February 2022.
EXPERT OPINION
Epidemiology and clinical features of neurologic manifestations are derived from different cohort studies. Our understanding of pathophysiology of neurologic manifestations in pSS has significantly increased in the past few years, especially regarding PNS. However, there are still many knowledge gaps on therapeutics. The few available data on therapy rely upon small case series, from experiences with other autoimmune diseases, such as systemic lupus erythematosus or expert opinion. There is an urgent need for well-designed clinical trials.
Topics: Humans; Lupus Erythematosus, Systemic; Sjogren's Syndrome
PubMed: 36001085
DOI: 10.1080/1744666X.2022.2117159 -
Modern Rheumatology Jan 2019Primary Sjögren's syndrome (pSS) is a chronic autoimmune disease that is estimated to affect 35 million people worldwide. Hallmarks of the disease are a loss of... (Review)
Review
Primary Sjögren's syndrome (pSS) is a chronic autoimmune disease that is estimated to affect 35 million people worldwide. Hallmarks of the disease are a loss of salivary and lacrimal gland function as well as lymphocytic infiltration, elevated proinflammatory cytokines, and circulating autoantibodies. Patients often experience significant fatigue and a decrease in their quality of life. Approximately 30-50% of pSS patients develop extra-glandular manifestations including malignant lymphoma. Although therapeutic approaches for pSS target both dryness and systemic manifestations, effective treatments are limited. However, new therapies targeting specific immune pathways associated with pSS are being developed. This review describes current and future targeted therapies against pSS.
Topics: Humans; Immunologic Factors; Molecular Targeted Therapy; Quality of Life; Sjogren's Syndrome; Treatment Outcome
PubMed: 30424703
DOI: 10.1080/14397595.2018.1546268 -
Scientific Reports Dec 2021Primary Sjögren's syndrome (SS) is a systemic autoimmune disease characterized by lymphocytic infiltration and damage of exocrine salivary and lacrimal glands. The...
Primary Sjögren's syndrome (SS) is a systemic autoimmune disease characterized by lymphocytic infiltration and damage of exocrine salivary and lacrimal glands. The etiology of SS is complex with environmental triggers and genetic factors involved. By conducting an integrated multi-omics study, we confirmed a vast coordinated hypomethylation and overexpression effects in IFN-related genes, what is known as the IFN signature. Stratified and conditional analyses suggest a strong interaction between SS-associated HLA genetic variation and the presence of Anti-Ro/SSA autoantibodies in driving the IFN epigenetic signature and determining SS. We report a novel epigenetic signature characterized by increased DNA methylation levels in a large number of genes enriched in pathways such as collagen metabolism and extracellular matrix organization. We identified potential new genetic variants associated with SS that might mediate their risk by altering DNA methylation or gene expression patterns, as well as disease-interacting genetic variants that exhibit regulatory function only in the SS population. Our study sheds new light on the interaction between genetics, autoantibody profiles, DNA methylation and gene expression in SS, and contributes to elucidate the genetic architecture of gene regulation in an autoimmune population.
Topics: Autoantibodies; DNA Methylation; Epigenomics; Female; Gene Expression; Gene Expression Regulation; Genetic Variation; HLA Antigens; Humans; Interferons; Male; Sjogren's Syndrome
PubMed: 34857786
DOI: 10.1038/s41598-021-01324-0 -
Medicina Clinica Sep 2020
Topics: Elasticity Imaging Techniques; Humans; Sjogren's Syndrome; Ultrasonography
PubMed: 32553423
DOI: 10.1016/j.medcli.2020.04.021 -
Current Opinion in Pediatrics Apr 2022Childhood Sjogren's syndrome (cSS) is a rare, chronic autoimmune disease characterized by inflammation of the exocrine glands. cSS is underrecognized because of... (Review)
Review
PURPOSE OF REVIEW
Childhood Sjogren's syndrome (cSS) is a rare, chronic autoimmune disease characterized by inflammation of the exocrine glands. cSS is underrecognized because of differences in clinical presentation compared with adults. Until recently, publications describing clinical manifestations in cSS were limited to case reports and case series with small numbers of patients. Diagnostic studies to assess glandular symptoms in adults, are less commonly obtained in children.
RECENT FINDINGS
Recent cohort studies describe presenting diagnostic clinical features in large populations of cSS and demonstrate how current classification criteria, used in adults, are not applicable to children. Recurrent parotitis is the consistent predominant manifestation that is inversely correlated with age. Novel salivary biomarkers and salivary gland ultrasonography are important objective measure, which may improve diagnosis and disease monitoring. Standardized treatment recommendations are needed.
SUMMARY
Findings from large cohort studies provide a framework for the future development of diagnostic criteria for cSS. Such criteria should incorporate objective measures that are easily obtained in children. Future research to improve understanding of the application of novel biomarkers and imaging and developing consensus on treatment recommendations is needed.
Topics: Adult; Autoimmune Diseases; Biomarkers; Child; Chronic Disease; Humans; Parotitis; Rare Diseases; Salivary Glands; Sjogren's Syndrome
PubMed: 34879027
DOI: 10.1097/MOP.0000000000001093