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Zeitschrift Fur Rheumatologie Jun 2009Sjögren's syndrome is a systemic autoimmune disease with a predominant involvement of exocrine glands leading to sicca symptoms. Extraglandular involvement occurs in... (Review)
Review
Sjögren's syndrome is a systemic autoimmune disease with a predominant involvement of exocrine glands leading to sicca symptoms. Extraglandular involvement occurs in about 40% of patients with skin, musculoskeletal, neurological and organ manifestations. Systemic vasculitic manifestations of Sjögren's syndrome can be assumed in approximately 5%-10% of patients. Leukocytoclastic or cryoglobulinemic vasculitis represent classic vasculitic manifestations of Sjögren's syndrome. In the pathogenesis of vasculitis, B-cell-driven autoimmune processes play a major role by producing autoantibodies against the Ro/SS-A and La/SS-B antigens and cryoglobulins. In patients with Sjögren's syndrome, manifestation of vasculitis, non-Hodgkin's lymphoma and glomerulonephritis, as well as positive cryoglobulins and decreased levels of complement factors, are considered negative prognostic markers. Various immunosuppressive strategies, usually in co-medication with glucocorticoids, are used for the treatment of vasculitis in Sjögren's syndrome. For refractory and severe manifestations, a B-cell-targeted therapy with Rituximab should be also considered.
Topics: Humans; Immunosuppressive Agents; Sjogren's Syndrome; Vasculitis
PubMed: 19357858
DOI: 10.1007/s00393-008-0400-8 -
Clinical and Experimental Rheumatology 2018Sjögren's syndrome is a complex and potentially disabling slow progressive, systemic disorder. During the last twelve months several original and important... (Review)
Review
Sjögren's syndrome is a complex and potentially disabling slow progressive, systemic disorder. During the last twelve months several original and important contributions have been published on the pathogenesis, diagnosis and therapy of the disease. This review, following the others of this series is aimed at summarising some of the most significant studies that have been recently published. Regarding the pathogenesis, we will specifically focus on novel insights on miRNA, gut microbiota, adaptive and innate autoimmunity and animal models. Concerning novelties in pSS diagnosis, we will focus on salivary gland ultrasonography and histology. Finally, we will conclude with an update of the clinical manifestations of the disease and with an overview of the future therapies.
Topics: Animals; Disease Models, Animal; Disease Progression; Female; Humans; Male; Predictive Value of Tests; Sex Factors; Sjogren's Syndrome; Treatment Outcome
PubMed: 30156536
DOI: No ID Found -
Clinical and Experimental Rheumatology 2019Primary Sjögren's syndrome (pSS) is a complex and heterogeneous disorder characterised by a wide spectrum of glandular and extra-glandular features. Novel insights into... (Review)
Review
Primary Sjögren's syndrome (pSS) is a complex and heterogeneous disorder characterised by a wide spectrum of glandular and extra-glandular features. Novel insights into disease pathogenesis and the discovery of novel biomarkers are allowing us to characterise the disease not only phenotypically on the basis of clinical presentation, but also on the basis of the endotype. Ultimately, a better stratification of patients may pave new avenues for novel targeted therapies, opening new possibilities for the application of personalised medicine in pSS.
Topics: Biomarkers; Humans; Prognosis; Proteomics; Sjogren's Syndrome
PubMed: 31464675
DOI: No ID Found -
Journal of Autoimmunity Jun 2014Primary Sjögren's syndrome (pSS) is one of the most frequent autoimmune systemic diseases, mainly characterized by ocular and oral dryness due to the progressive... (Review)
Review
Primary Sjögren's syndrome (pSS) is one of the most frequent autoimmune systemic diseases, mainly characterized by ocular and oral dryness due to the progressive destruction of lachrymal and salivary glands by an inflammatory process. A noteworthy proportion of patients also features extraglandular manifestations, sometimes severe and life-threatening. Until now, its management relies mostly on symptomatic interventions, long-term monitoring, and, in patients with severe systemic complications, immunosuppressive drugs can be provided. However, recent years have seen great progresses in the understanding of the pathological processes of the disease. The central role of regulatory lymphocytes, the implication of the type 1 interferon pathway in some patients or the importance of epigenetics have been highlighted. New classification criteria have been recently published and have shed in light an international attempt for a better recognition of the patients, probably thanks to the development of new diagnostic procedures such as salivary gland ultrasonography. To facilitate the detection of treatment efficacy in clinical trials and to help in determining which subgroups of patients would have benefits from intensive therapies, a better definition of activity scores and the availability of new prognostic markers are urgent. Thereby, the development of future therapies should be based on specific molecular signatures that will enable a personalized management of each patient. This review focuses on the most striking advances in the fields of pathophysiology, diagnosis and treatment of pSS, which generate a great hope for pSS patients.
Topics: Humans; Sjogren's Syndrome
PubMed: 24612946
DOI: 10.1016/j.jaut.2014.02.006 -
Frontiers in Immunology 2022Sjögrens syndrome (SS) is caused by autoantibodies that attack proprioceptive salivary and lacrimal gland tissues. Damage to the glands leads to dry mouth and eyes and... (Review)
Review
Sjögrens syndrome (SS) is caused by autoantibodies that attack proprioceptive salivary and lacrimal gland tissues. Damage to the glands leads to dry mouth and eyes and affects multiple systems and organs. In severe cases, SS is life-threatening because it can lead to interstitial lung disease, renal insufficiency, and lymphoma. Histological examination of the labial minor salivary glands of patients with SS reveals focal lymphocyte aggregation of T and B cells. More studies have been conducted on the role of B cells in the pathogenesis of SS, whereas the role of T cells has only recently attracted the attention of researchers. This review focusses on the role of various populations of T cells in the pathogenesis of SS and the progress made in research to therapeutically targeting T cells for the treatment of patients with SS.
Topics: Humans; Sjogren's Syndrome; T-Lymphocytes; Salivary Glands, Minor; Lacrimal Apparatus; Autoantibodies
PubMed: 36389806
DOI: 10.3389/fimmu.2022.995895 -
Advances in Rheumatology (London,... Oct 2022Sjogren's syndrome (SS) is an autoimmune disease characterized by lymphocytic infiltration of the exocrine glands and other organs, associated with sicca syndrome but... (Meta-Analysis)
Meta-Analysis
Recommendations for evaluation and diagnosis of extra-glandular manifestations of primary Sjögren syndrome: results of an epidemiologic systematic review/meta-analysis and a consensus guideline from the Brazilian society of rheumatology (hepatic, gastrointestinal and pancreatic).
Sjogren's syndrome (SS) is an autoimmune disease characterized by lymphocytic infiltration of the exocrine glands and other organs, associated with sicca syndrome but also with systemic involvement with varying degrees of severity. Despite their importance, some systemic manifestations, mainly liver, gastrointestinal, and pancreatic are not routinely evaluated. To address these manifestations, the Sjögren's Syndrome Committee of the Brazilian Society of Rheumatology conducted a broad systematic review of the literature on studies investigating prevalence and diagnosis of these symptoms in Sjogren´s patients and made recommendations based on the findings. Agreement between the experts was achieved using the Delphi method. This is the second part of this guideline, providing 6 recommendations for liver, gastrointestinal, and pancreatic care of SS patients.
Topics: Humans; Brazil; Consensus; Liver; Rheumatology; Sjogren's Syndrome
PubMed: 36217186
DOI: 10.1186/s42358-022-00267-y -
Clinical and Experimental Rheumatology Dec 2023Sjögren's syndrome (SS) is a complex and heterogeneous disease that typically affects middle-aged women. However, while it is rare, the disease may occur in male... (Review)
Review
Sjögren's syndrome (SS) is a complex and heterogeneous disease that typically affects middle-aged women. However, while it is rare, the disease may occur in male patients and in females during their childhood/adolescence or in the elderly. Contrasting data have been reported on these three subgroups clinical features and long-term outcomes. Notably, recent studies have pinpointed the severity of the disease in male patients and in both the early and the late-onset subgroups.The aim of this review is, therefore, to summarise the available evidence from the recent literature on these phenotypes. The focus will be on the clinical and laboratory features, and on the lymphoma risk observed in the three subgroups distinct phenotypes: of male patients as well as young-onset SS and elderly-onset SS. Ultimately, an accurate phenotypic stratification may represent the first step towards individualised medical approaches.
Topics: Aged; Middle Aged; Adolescent; Humans; Male; Female; Child; Sjogren's Syndrome; Age of Onset; Lymphoma; Phenotype
PubMed: 38149517
DOI: 10.55563/clinexprheumatol/lygrzv -
British Medical Journal Oct 1973
Topics: Arthritis, Rheumatoid; Female; Humans; Lupus Erythematosus, Systemic; Male; Middle Aged; Sjogren's Syndrome
PubMed: 4745345
DOI: No ID Found -
Illinois Dental Journal Oct 1992
Topics: Diagnosis, Differential; Humans; Sjogren's Syndrome; Xerostomia
PubMed: 1286865
DOI: No ID Found -
The Journal of Laryngology and Otology Nov 1986
Topics: Aged; Animals; Autoimmune Diseases; Female; Humans; Lymphoma; Male; Mice; Middle Aged; Parotid Neoplasms; Sjogren's Syndrome
PubMed: 3794535
DOI: 10.1017/s002221510010101x