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Arthritis Research & Therapy Feb 2024Sjögren's disease is a heterogeneous autoimmune disorder that may be associated with systemic manifestations such as pulmonary or articular involvement. Systemic... (Review)
Review
Sjögren's disease is a heterogeneous autoimmune disorder that may be associated with systemic manifestations such as pulmonary or articular involvement. Systemic complications have prognostic implications and need to be identified and managed in a timely manner. Treatment should be tailored to the type and severity of organ involvement, ideally based on multidisciplinary evaluation.
Topics: Humans; Sjogren's Syndrome; Autoimmune Diseases
PubMed: 38331820
DOI: 10.1186/s13075-024-03262-4 -
International Journal of Molecular... Aug 2023Primary Sjögren's syndrome (pSS) is a connective tissue disease characterized by a wide spectrum of clinical features, extending from a benign glandular disease to an... (Review)
Review
Primary Sjögren's syndrome (pSS) is a connective tissue disease characterized by a wide spectrum of clinical features, extending from a benign glandular disease to an aggressive systemic disorder and/or lymphoma. The pathogenesis of Sjögren's syndrome (SS) is not completely understood, but it is assumed that pathogenesis of SS is multifactorial. The studies based on the animal models of SS provided significant insight in SS disease pathogenesis and management. The aim of this review is to summarize current studies on animal models with primary SS-like symptoms and discuss the impact of these studies on better understanding pathogenesis and management of Sjögren's syndrome. Databases PubMed, Web of Science, Scopus and Cochrane library were searched for summarizing studies on animal models in SS. Available data demonstrate that animal models are highly important for our understanding of SS disease.
Topics: Animals; Sjogren's Syndrome; Models, Animal; Aggression; Databases, Factual; Gene Library
PubMed: 37629175
DOI: 10.3390/ijms241612995 -
Medicina Clinica Sep 2020
Topics: Elasticity Imaging Techniques; Humans; Sjogren's Syndrome; Ultrasonography
PubMed: 32553423
DOI: 10.1016/j.medcli.2020.04.021 -
International Journal of Molecular... Nov 2020Fibrosis is presented in various physiologic and pathologic conditions of the salivary gland. Transforming growth factor beta (TGF-β) pathway has a pivotal role in the... (Review)
Review
Fibrosis is presented in various physiologic and pathologic conditions of the salivary gland. Transforming growth factor beta (TGF-β) pathway has a pivotal role in the pathogenesis of fibrosis in several organs, including the salivary glands. Among the TGF-β superfamily members, TGF-β1 and 2 are pro-fibrotic ligands, whereas TGF-β3 and some bone morphogenetic proteins (BMPs) are anti-fibrotic ligands. TGF-β1 is thought to be associated with the pro-fibrotic pathogenesis of sialadenitis, post-radiation salivary gland dysfunction, and Sjögren's syndrome. Potential therapeutic strategies that target multiple levels in the TGF-β pathway are under preclinical and clinical research for fibrosis. Despite the anti-fibrotic effect of BMPs, their in vivo delivery poses a challenge in terms of adequate clinical efficacy. In this article, we will review the relevance of TGF-β signaling in salivary gland fibrosis and advances of potential therapeutic options in the field.
Topics: Animals; Disease Susceptibility; Fibrosis; Humans; Radiation; Salivary Glands; Signal Transduction; Sjogren's Syndrome; Transforming Growth Factor beta
PubMed: 33266300
DOI: 10.3390/ijms21239138 -
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 -
Journal of Neurology Aug 2021This study addresses the challenging characterisation and differentiation of CIDP versus CIDP in association with Sjögren's syndrome to facilitate the process in...
BACKGROUND
This study addresses the challenging characterisation and differentiation of CIDP versus CIDP in association with Sjögren's syndrome to facilitate the process in clinical routine.
METHODS
Patients with both CIDP and Sjögren's syndrome and CIDP without Sjögren's syndrome were compared concerning relevant differences in clinical, laboratory and electrophysiological findings. 154 patients who fulfilled the diagnostic EFNS/PNS criteria for CIDP were included in the analysis. 54 of these patients additionally fulfilled the ACR/EULAR classification criteria for Sjögren's syndrome.
RESULTS
The frequency of female patients was higher in patients with CIDP and Sjögren's syndrome (52%) versus CIDP patients without Sjögren's syndrome (28%). Furthermore, the occurrence of cranial nerve impairment was significantly higher in patients with Sjögren's syndrome (39% versus 14%). There were no significant group differences in the evaluation of initial symptoms, severity of disability judged by INCAT disability scale score, presence or distribution of sensory deficits, limb weakness and the presence of ataxia, pain or dysautonomia, CSF laboratory or electrophysiological findings.
CONCLUSIONS
In conclusion, our data indicate that cranial nerve impairment and female gender might represent red flags for an additional Sjögren's syndrome in patients with CIDP. The patterns of clinical disabilities and electrophysiological findings due to peripheral nerve damage are similar in both CIDP entities.
Topics: Ataxia; Female; Humans; Muscle Weakness; Polyradiculoneuropathy, Chronic Inflammatory Demyelinating; Sjogren's Syndrome
PubMed: 33611611
DOI: 10.1007/s00415-021-10459-z -
Joint Bone Spine Mar 2023
Topics: Humans; Sjogren's Syndrome; Antibodies, Antinuclear; Biopsy
PubMed: 36464213
DOI: 10.1016/j.jbspin.2022.105502 -
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 -
Clinics in Chest Medicine Sep 2019Sjögren syndrome (SS) is a progressive autoimmune disease characterized by dryness, predominantly of the eyes and mouth, caused by chronic lymphocytic infiltration of... (Review)
Review
Sjögren syndrome (SS) is a progressive autoimmune disease characterized by dryness, predominantly of the eyes and mouth, caused by chronic lymphocytic infiltration of the lacrimal and salivary glands. Extraglandular inflammation can lead to systemic manifestations, many of which involve the lungs. Studies in which lung involvement is defined as requiring the presence of respiratory symptoms and either radiograph or pulmonary function test abnormalities quote prevalence estimates of 9% to 22%. The most common lung diseases that occur in relation to SS are airways disease and interstitial lung disease. Evidence-based guidelines to inform treatment recommendations for lung involvement are largely lacking.
Topics: Humans; Lung; Lung Diseases; Sjogren's Syndrome
PubMed: 31376889
DOI: 10.1016/j.ccm.2019.05.002 -
Advances in Experimental Medicine and... 2021Sjogren's syndrome is an autoimmune connective tissue disease targeting the exocrine glands and frequently affecting the respiratory system. The pulmonary disease is the...
Sjogren's syndrome is an autoimmune connective tissue disease targeting the exocrine glands and frequently affecting the respiratory system. The pulmonary disease is the most important extra-glandular manifestation as it carries most of the morbidity and mortality. Typically, it affects the small airways ranging from mild to severe respiratory symptoms. The upper airways are also commonly involved, predisposing sinusitis to occur more frequently than in the normal population. Lymphocytic interstitial pneumonia was initially thought to be the prevailing parenchymal disease; however, multiple cohorts report non-interstitial pneumonia to be the most frequent subtype of interstitial lung disease. In the review of high-resolution computed tomography scans, cystic lesions are commonly found and associate with both the small airways and parenchymal disease. Under their presence, amyloidosis or lymphomas should be considered in the differential. Overall, Sjogren's syndrome has a higher risk for lymphoma, and in lungs this condition should be thought of, especially when the images reveal pulmonary nodularity, lymphocytic interstitial pneumonia and lymphadenopathy. Although, pulmonary artery hypertension was traditionally and exceptionally linked with Sjogren's syndrome, together with systemic lupus erythematosus, they are now acknowledged to be the most common pulmonary vascular disease in east Asian populations, even over patients with systemic sclerosis. Although there are no controlled prospective trials to treat pulmonary disease in Sjogren's syndrome, the mainstay treatment modality still falls on glucocorticoid therapy (systemic and inhaled), combined with immune modulators or alone. Most of the evidence sustains successful outcomes based on reported cases or case series.
Topics: Humans; Lung; Lung Diseases, Interstitial; Lupus Erythematosus, Systemic; Prospective Studies; Sjogren's Syndrome
PubMed: 33788195
DOI: 10.1007/978-3-030-63046-1_12