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Rheumatic Diseases Clinics of North... May 2024The development of sicca in patients treated with immune checkpoint inhibitors (ICIs) is undoubtedly an underestimated complication, but one whose functional... (Review)
Review
The development of sicca in patients treated with immune checkpoint inhibitors (ICIs) is undoubtedly an underestimated complication, but one whose functional consequences and impact on quality of life are significant for patients. This update aims to review the frequency of this complication and different clinical pictures. The authors also propose a diagnostic and therapeutic approach to guide clinicians in daily practice.
Topics: Humans; Immune Checkpoint Inhibitors; Sjogren's Syndrome; Quality of Life
PubMed: 38670727
DOI: 10.1016/j.rdc.2024.02.004 -
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 -
Journal of Neuroimmunology Feb 2024This report details a rare case where a patient simultaneously suffered from Sjogren's syndrome (SS) and Meige's syndrome (MS). SS, an autoimmune disorder, and MS, a...
This report details a rare case where a patient simultaneously suffered from Sjogren's syndrome (SS) and Meige's syndrome (MS). SS, an autoimmune disorder, and MS, a rare neurological condition characterized by involuntary eyelid closure, presented in a 73-year-old male. The patient had been experiencing dry eye symptoms for the past 5 years, with the onset of eyelid spasms and tetanic eye closure occurring 3 years ago. Traditional treatments, including subthalamic nucleus deep brain stimulation, provided only temporary relief. Diagnostic evaluations, including blood tests and imaging, confirmed SS and MS coexistence. Treatment involved a combination of steroids, immunosuppressants, and immunoglobulin, leading to significant symptom relief. This case suggests a potential association between SS and the development of MS, highlighting the importance of immunomodulatory therapy in managing neurological symptoms. Further research is needed to explore the relationship between these two conditions and to develop more effective treatment strategies.
Topics: Male; Humans; Aged; Sjogren's Syndrome; Meige Syndrome; Blepharospasm; Treatment Outcome
PubMed: 38134494
DOI: 10.1016/j.jneuroim.2023.578264 -
La Revue de Medecine Interne Sep 2019
Topics: Humans; Sjogren's Syndrome
PubMed: 31627798
DOI: 10.1016/S0248-8663(19)30662-9 -
Expert Review of Clinical Immunology Feb 2021: Sjögren's syndrome is a unique systemic autoimmune disease, placed in the center of systemic autoimmunity and at the crossroads of autoimmunity and... (Review)
Review
: Sjögren's syndrome is a unique systemic autoimmune disease, placed in the center of systemic autoimmunity and at the crossroads of autoimmunity and lymphoproliferation. The diverse clinical picture of the disease, the inefficacy of current biologic treatments, and the co-existence with lymphoma conferring to the patients' morbidity and mortality force the scientific community to review disease pathogenesis and reveal the major implicated cellular and molecular elements.: Biomarkers for early diagnosis, prediction, stratification, monitoring, and targeted treatments can serve as a tool to interlink and switch from the clinical phenotyping of the disease into a more sophisticated classification based on the underlying critical molecular pathways and endotypes. Such a transition may define the establishment of the so-called precision medicine era in which patients' management will be based on grouping according to pathogenetically related biomarkers. In the current work, literature on Sjogren's syndrome covering several research fields including clinical, translational, and basic research has been reviewed.: The perspectives of clinical and translational research are anticipated to define phenotypic clustering of high-risk pSS patients and link the clinical picture of the disease with fundamental molecular mechanisms and molecules implicated in pathogenesis.
Topics: Autoimmunity; Biomarkers; Humans; Lymphoma; Lymphoproliferative Disorders; Precision Medicine; Sjogren's Syndrome
PubMed: 33478279
DOI: 10.1080/1744666X.2021.1879641 -
Blood Cells, Molecules & Diseases Jan 2023To present the rheumatological manifestations of chronic graft versus host disease (cGVHD) and describe how they differ from primary systemic connective tissue diseases. (Review)
Review
OBJECTIVES
To present the rheumatological manifestations of chronic graft versus host disease (cGVHD) and describe how they differ from primary systemic connective tissue diseases.
METHODS
Description of 7 patients with cGVHD with symptoms resembling Sjögren's syndrome and scleroderma, with a critical review of the literature.
RESULTS
7 patients treated at the hematology department, who developed cGVHD with present antinuclear antibodies, were referred to the rheumatology department for further evaluation. All patients presented symptoms of dry eye syndrome confirmed with ophthalmic tests. If the diagnosis of GVHD was not an exclusion criterion, Sjögren's syndrome criteria would be met by 4 of our patients - they presented not only with dryness but also with typical antibodies, inflammatory changes in salivary glands on ultrasound examination, and mononuclear cell infiltration in histopathological examination of labial salivary glands. Additionally, three patients presented with scleroderma-like syndromes, but with symptoms easy to differentiate from systemic sclerosis.
CONCLUSION
cGVHD may be difficult to distinguish from Sjögren's syndrome, but such distinction is important due to the different standards of treatment in cGVHD and primary connective tissue diseases.
Topics: Humans; Sjogren's Syndrome; Graft vs Host Disease
PubMed: 36371928
DOI: 10.1016/j.bcmd.2022.102709 -
Advances in Experimental Medicine and... 2023The salivary glands, exocrine glands in our body producing saliva, can be easily damaged by various factors. Radiation therapy and Sjogren's syndrome (a systemic...
The salivary glands, exocrine glands in our body producing saliva, can be easily damaged by various factors. Radiation therapy and Sjogren's syndrome (a systemic autoimmune disease) are the two main causes of salivary gland damage, leading to a severe reduction in patients' quality of life. Gene transfer to the salivary glands has been considered a promising approach to treating the dysfunction. Gene therapy has long been applied to cure multiple diseases, including cancers, and hereditary and infectious diseases, which are proven to be safe and effective for the well-being of patients. The application of this treatment on salivary gland injuries has been studied for decades, yet its clinical progress is delayed. This chapter provides a coup d'oeil into gene transfer methods and various gene/vector types for salivary glands to help the new scientists and update established scientists on the progress that has been made during the past decades for the treatment of salivary gland disorders.
Topics: Humans; Quality of Life; Salivary Glands; Sjogren's Syndrome; Saliva; Genetic Therapy
PubMed: 36826746
DOI: 10.1007/5584_2023_766 -
Journal of Neuroimaging : Official... Nov 2021The phenotype of Sjögren's syndrome-associated neuropathy has been better characterized in recent years. However, Sjögren's syndrome-associated neuropathy remains an...
BACKGROUND AND PURPOSE
The phenotype of Sjögren's syndrome-associated neuropathy has been better characterized in recent years. However, Sjögren's syndrome-associated neuropathy remains an underdiagnosed entity with only few insights considering the pathomechanisms of nerve damage. Nerve ultrasound has proven to be a useful and efficient tool in detecting nerve damage of autoimmune origin. We, therefore, aimed to evaluate this method for Sjögren's syndrome-associated neuropathy.
METHODS
Patients with Sjögren's syndrome and clinical signs of neuropathy underwent sonographic examination of both median and ulnar nerves. Nerve thickening was classified for cross-sectional areas of >12 mm² at the median nerve and for >10 mm² at the ulnar nerve. Fascicle thickening was documented for cross-sectional areas ≥5 mm² at the median and ≥3 mm² at the ulnar nerve.
RESULTS
Forty-three patients were included in the analysis (median age 60 years [interquartile range 53-73 years], female rate 60%). 31/43 patients (72%) showed abnormalities on nerve ultrasound, while nerve thickening was found more frequently than fascicle thickening (90% vs. 52% of patients with sonographic abnormalities, respectively). Abnormal findings were observed more frequently at the median nerve and in proximal localization. Abnormal findings on nerve conduction studies were evident in 36/43 patients (84%). Nerve conduction studies revealed a tendency of demyelinating nerve damage patterns being associated with abnormal findings on nerve ultrasound.
CONCLUSIONS
In addition to nerve conduction studies, nerve ultrasound may have a supporting role in the diagnosis of Sjögren's syndrome-associated neuropathy. Also, our data support an immune-mediated inflammatory demyelinating pathogenesis of Sjögren's syndrome-associated neuropathy.
Topics: Female; Humans; Neurologic Examination; Peripheral Nervous System Diseases; Sjogren's Syndrome; Ultrasonography
PubMed: 34270142
DOI: 10.1111/jon.12907 -
Nature Reviews. Rheumatology Dec 2019
Review
Topics: Antirheumatic Agents; Autoimmunity; Humans; Immunologic Factors; Sjogren's Syndrome
PubMed: 31605032
DOI: 10.1038/s41584-019-0325-4 -
European Archives of... Jul 2023This scoping review aims to synthesize all of the currently available information on how xerostomia correlates with vocal function and the mechanisms that underpin it. (Review)
Review
PURPOSE
This scoping review aims to synthesize all of the currently available information on how xerostomia correlates with vocal function and the mechanisms that underpin it.
METHODS
Our scoping review used PubMed, Scopus, Embase, and Web of Science databases to review articles published between January 1999 and July 2022 in accordance with the PRISMA-ScR guidelines. In addition to the academic databases, we also conducted a manual search of Google Scholar. Further investigation was conducted on studies that examined the relationship between xerostomia and vocal function.
RESULTS
Of the 682 initially identified articles, 21 met our inclusion criteria. Among the included studies, two articles (n = 2) revealed the mechanistic relationship between xerostomia and vocal function. Most studies (n = 12) focused on xerostomia secondary to other underlying conditions or treatments, among which radiotherapy and Sjögren's syndrome were commonly investigated. Seven studies (n = 7) provided details about common vocal parameters measured in studies of xerostomia and the voice.
CONCLUSION
The literature currently lacks publications regarding the relationship between xerostomia and vocal function. Most of the studies included in this review were about xerostomia secondary to other conditions or medical treatments. Therefore, the impacts on the voice that were observed were very multifaceted and the role of xerostomia alone in phonation could not be ascertained. Nevertheless, it is clear that dryness in the mouth plays some role in vocal function and further research should focus on clarifying and finding the underlying mechanism behind this relationship by incorporating high-speed imaging and cepstral peak prominence analyses.
Topics: Humans; Voice Quality; Xerostomia; Sjogren's Syndrome; Phonation; Voice
PubMed: 36995371
DOI: 10.1007/s00405-023-07941-x