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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 -
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 -
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 -
Oral Diseases Mar 2017IgG4-related disease (IgG4-RD) has emerged as a new entity in the last decade. It comprises numerous conditions previously thought to be unrelated. Macroscopically,... (Review)
Review
IgG4-related disease (IgG4-RD) has emerged as a new entity in the last decade. It comprises numerous conditions previously thought to be unrelated. Macroscopically, these diseases cause diffuse organ swelling and formation of pseudotumorous masses. Histopathologically, they are characterized by a lymphoplasmacytic infiltrate with increased IgG4+ plasma cells and storiform fibrosis. Despite rapid progress within the last years, our knowledge on these conditions is still fragmented. To date, more than forty organs have been reported to be included in IgG4-RD, and salivary gland involvement is amongst the most common organs affected [IgG4-related sialadenitis (IgG4-RS)]. Interestingly, IgG4-RS shares commonalities with Sjögren's syndrome (SS), like glandular enlargement, sicca symptoms, arthralgias, hypergammaglobulinemia, hypocomplementemia, and circulating antinuclear antibodies. Nonetheless, they differ in that the incidence of anti-Ro and anti-La reactivity is not frequently found in patients with IgG4-RS, their salivary glands are infiltrated by a large number of IgG4+ plasma cells and IgG4-RS symptoms respond promptly to steroids. The aim of this review was to describe the clinical, serological, histopathological and pathophysiological aspects of IgG4-RS in the context of IgG4-RD and highlight the differences between IgG4-RS and SS.
Topics: Diagnosis, Differential; Humans; Immunoglobulin G; Salivary Glands; Sialadenitis; Sjogren's Syndrome
PubMed: 27318181
DOI: 10.1111/odi.12526 -
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 -
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 -
Home Healthcare Now Oct 2015There are approximately 4 million Americans diagnosed with Sjogren's Syndrome. This article discusses the epidemiology, pathophysiology, diagnostics, and implications... (Review)
Review
There are approximately 4 million Americans diagnosed with Sjogren's Syndrome. This article discusses the epidemiology, pathophysiology, diagnostics, and implications for home care clinicians who may encounter patients with this syndrome. Chronic pain is discussed as well as interventions to manage symptoms such fatigue, dry eyes mouth and skin.
Topics: Eye; Humans; Mouth; Prevalence; Sjogren's Syndrome; United States
PubMed: 26418108
DOI: 10.1097/NHH.0000000000000295 -
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