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Journal of Clinical Rheumatology :... Aug 2023The aim of this study was to study clinical and biological differences between men and women with primary Sjögren syndrome (pSS) in China and perform a literature... (Review)
Review
OBJECTIVES
The aim of this study was to study clinical and biological differences between men and women with primary Sjögren syndrome (pSS) in China and perform a literature review to confirm if the clinical phenotypes are affected by sex in patients with pSS.
METHODS
Data from 961 patients with pSS treated at a tertiary hospital in China between January 2013 and March 2022 were analyzed based on medical records. Clinical characteristics, including disease manifestations and serological parameters of the disease, were compared between men and women with pSS using the Mann-Whitney U test and χ 2 test.
RESULTS
This study included 140 (14.6%) men and 821 (85.4%) women with pSS. Women with pSS demonstrated a higher prevalence of dry mouth, dry eyes, arthralgia, and dental caries ( p < 0.05); higher erythrocyte sedimentation rate and immunoglobulin M levels ( p < 0.05); higher prevalence of leukopenia, neutropenia, anemia, low complement 3, and low complement 4 ( p < 0.05); and higher titers of antinuclear antibody, anti-Sjögren syndrome A, anti-Ro52, and rheumatoid factor positivity ( p < 0.05) than men, whereas men with pSS had a higher prevalence of parotid enlargement and interstitial lung disease ( p < 0.05).
CONCLUSIONS
Women with pSS are associated with more dryness, cytopenia, hypocomplementemia, and autoantibody positivity. Although men with pSS probably have lighter sicca symptoms and lower immunoactivity and serologic responses, regular monitoring of interstitial lung disease in men is vital.
Topics: Humans; Male; Female; Sex Characteristics; Dental Caries; Sjogren's Syndrome; Lung Diseases, Interstitial; Medical Records
PubMed: 37068269
DOI: 10.1097/RHU.0000000000001962 -
Journal of Neurology Feb 2023Sjögren's syndrome is a potentially treatable cause of Small Fiber Neuropathy (SFN)-a condition that severely affects patients' quality of life. We therefore aimed to...
Sjögren's syndrome is a potentially treatable cause of Small Fiber Neuropathy (SFN)-a condition that severely affects patients' quality of life. We therefore aimed to characterize patients with SFN and Sjögren's syndrome to raise awareness of this disease and facilitate its early recognition as an essential step for appropriate treatment. In 97 SFN patients (median age 48 years, 77% female), we studied the clinical features associated with Sjögren's syndrome compared to the idiopathic SFN subtype. According to the current ACR/EULAR classification criteria (Shiboski et al., Ann Rheum Dis 76:9-16, 2017), 24/97 individuals (25%, median age 48.5 years, 75% female) were diagnosed with Sjögren's syndrome. We did not observe any differences in SFN-defining sensory plus symptoms. Furthermore, intraepidermal nerve fiber densities (IENFD) were significantly lower in patients with SFN and Sjögren's syndrome (mean 2.6 ± 1.2/mm) compared to patients with idiopathic SFN (mean 3.2 ± 1.5/mm; p = 0.048). There were no significant group differences when analyzing cerebrospinal fluid (CSF) parameters. We conclude that Sjögren's syndrome-associated SFN is difficult to distinguish from idiopathic forms based on initial clinical symptoms and CSF results. However, lower IENFD values in patients with Sjögren's syndrome-associated SFN might indicate a distinct different pathomechanism in this entity compared to idiopathic SFN.
Topics: Humans; Female; Middle Aged; Male; Small Fiber Neuropathy; Sjogren's Syndrome; Quality of Life; Biopsy
PubMed: 36331613
DOI: 10.1007/s00415-022-11431-1 -
Biomolecules Apr 2023Dry eye disease is a chronic disease of the ocular surface characterized by abnormal tear film composition, tear film instability, and ocular surface inflammation,... (Review)
Review
Dry eye disease is a chronic disease of the ocular surface characterized by abnormal tear film composition, tear film instability, and ocular surface inflammation, affecting 5% to 50% of the population worldwide. Autoimmune rheumatic diseases (ARDs) are systemic disorders with multi-organ involvement, including the eye, and play a significant role in dry eye. To date, most studies have focused on Sjögren's syndrome (one of the ARDs) since it manifests as two of the most common symptoms-dry eyes and a dry mouth-and attracts physicians to explore the relationship between dry eye and ARDs. Many patients complained of dry eye related symptoms before they were diagnosed with ARDs, and ocular surface malaise is a sensitive indicator of the severity of ARDs. In addition, ARD related dry eye is also associated with some retinal diseases directly or indirectly, which are described in this review. This review also summarizes the incidence, epidemiological characteristics, pathogenesis, and accompanying ocular lesions of ARD's related dry eye, emphasizing the potential role of dry eye in recognition and monitoring among ARDs patients.
Topics: Humans; Dry Eye Syndromes; Sjogren's Syndrome; Tears; Retinal Diseases; Respiratory Distress Syndrome
PubMed: 37238594
DOI: 10.3390/biom13050724 -
BMC Oral Health Mar 2024Observational studies indicated a controversial relationship between periodontitis (PD) and Sjogren's syndrome (SS). To overcome restrictions in conventional...
OBJECTIVES
Observational studies indicated a controversial relationship between periodontitis (PD) and Sjogren's syndrome (SS). To overcome restrictions in conventional observational studies, we conducted a two-sample Mendelian randomization (MR) analysis to assess the potential bidirectional relationship between PD and SS.
METHODS
We utilized the largest available genome-wide association study (GWAS) of European ancestry on both PD (17,353 cases-28,210 controls) and SS (2495 cases-365,533 controls) for MR genetic instrument selection. The random-effect inverse-variance weighted (IVW) method complemented by Causal Analysis Using Summary Effect (CAUSE), weighted median, weighted mode, simple mode, MR-Egger regression, and MR-pleiotropy residual sum and outlier (MR-PRESSO) was used for MR analysis. Subsequent pleiotropy and heterogeneity tests were conducted.
RESULTS
IVW analysis exhibited neither an effect of PD on SS (OR = 0.939, 95%CI = 0.525-1.677, P = 0.8304) nor that of SS on PD (OR = 1.007, 95%CI = 0.977-1.038, P = 0.6440). The other five complementary methods further recognized the null association with an effect size close to one. No significant pleiotropy was detected in the relationship between PD and SS (P > 0.05). Heterogeneity existed in the effect of PD on SS but not vice versa.
CONCLUSIONS
No genetic causality between PD and SS or vice versa was supported by our results under MR assumptions and limitations. The study results provided new insights into the relationship between periodontal status and sjogren's syndrome, highlighting the need for a more prudent medical intervention.
Topics: Humans; Sjogren's Syndrome; Genome-Wide Association Study; Mendelian Randomization Analysis; Periodontitis
PubMed: 38528503
DOI: 10.1186/s12903-024-04151-7 -
Clinical and Experimental Rheumatology 2021Sjögren's syndrome (SS) is a multifactorial systemic autoimmune disease of unknown aetiology characterised by a wide spectrum of different clinical manifestations and... (Review)
Review
Sjögren's syndrome (SS) is a multifactorial systemic autoimmune disease of unknown aetiology characterised by a wide spectrum of different clinical manifestations and scattered complications. Recently, great efforts have been made to elucidate mechanisms involved in the pathogenesis of the disease in order to identify exploitable therapeutic targets in SS. Similarly, novel insights have enabled to better define disease phenotypes and different outcomes. Ultimately, the discovery of new potential therapeutic targets and a better stratification of patients are paving new avenues for novel treatment options and treat-to-target therapeutic approach. In this review, we will provide a critical digest of the recent literature published in 2020 on SS pathogenesis, clinical manifestations and novel treatment options.
Topics: Humans; Phenotype; Sjogren's Syndrome
PubMed: 34874834
DOI: 10.55563/clinexprheumatol/eojaol -
Expert Opinion on Therapeutic Targets Oct 2022Sjögren's syndrome (SS) is an immune-mediated inflammatory condition characterized by sicca syndrome, musculoskeletal pain, and fatigue. Extra-glandular manifestations... (Review)
Review
INTRODUCTION
Sjögren's syndrome (SS) is an immune-mediated inflammatory condition characterized by sicca syndrome, musculoskeletal pain, and fatigue. Extra-glandular manifestations are common and there is a markedly increased risk of lymphoma development. SS is associated with high health-economic burden driven largely by the symptom burden on patients. Currently, there is no approved disease-modifying treatment and management is based on empirical evidence. Progress in the understanding of SS pathogenesis has led to an expanding portfolio of more targeted therapies under development.
AREAS COVERED
This review summarizes the key development in targeted biological therapies in SS including emerging targets. It also highlights the challenges in therapeutic development in SS such as disease heterogeneity and defining appropriate disease assessment tools to evaluate therapeutic efficacy.
EXPERT OPINION
Early trials in SS failed to meet their primary outcomes which may in part due to the use of inappropriate or insensitive study endpoints. Recent trials targeting B-cells, B-T cell co-stimulation and IFN signaling have shown promising results. Development of composite endpoints including patient reported outcomes and objective disease measure may provide a more holistic approach to disease assessment. The impact of these new tools on therapeutic development that benefit patients remains to be fully evaluated.
Topics: Humans; Sjogren's Syndrome; B-Lymphocytes; Lymphoma
PubMed: 36576336
DOI: 10.1080/14728222.2022.2157259 -
Clinical and Experimental Rheumatology Dec 2022Primary Sjögren's syndrome may be difficult to diagnose when antibodies against Ro/SSA are lacking, and can be grouped in at least four clusters indicating different... (Review)
Review
Primary Sjögren's syndrome may be difficult to diagnose when antibodies against Ro/SSA are lacking, and can be grouped in at least four clusters indicating different pathophysiological pathways. Novel biomarkers, in particular autoantibodies, would be helpful in diagnosing Sjögren's syndrome and in further identification and characterisation of the clusters.In this review, we describe new technologies that may be utilised in the rapid identification of novel autoantibodies, and an example of how well characterised patients, here from the HarmonicSS cohort, are a prerequisite in the discovery of clinically meaningful biomarkers. This translational approach hold promise to optimise the diagnosis and treatment of individual pSS patient subsets.
Topics: Humans; Autoantibodies; Sjogren's Syndrome; Biomarkers
PubMed: 36226613
DOI: 10.55563/clinexprheumatol/sba8k2 -
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 -
Frontiers in Immunology 2023Sjögren's syndrome (SS) is a systemic autoimmune disease, which affects the exocrine glands leading to glandular dysfunction and, particularly, symptoms of oral and...
INTRODUCTION
Sjögren's syndrome (SS) is a systemic autoimmune disease, which affects the exocrine glands leading to glandular dysfunction and, particularly, symptoms of oral and ocular dryness. The aetiology of SS remains unclear, and the disease lacks distinctive clinical features. The current diagnostic work-up is complex, invasive and often time-consuming. Thus, there is an emerging need for identifying disease-specific and, ideally, non-invasive immunological and molecular biomarkers that can simplify the diagnostic process, allow stratification of patients, and assist in monitoring the disease course and outcome of therapeutic intervention in SS.
METHODS
This systematic review addresses the use of proteomics and miRNA-expression profile analyses in this regard.
RESULTS AND DISCUSSION
Out of 272 papers that were identified and 108 reviewed, a total of 42 papers on proteomics and 23 papers on miRNA analyses in saliva, blood and salivary gland tissue were included in this review. Overall, the proteomic and miRNA studies revealed considerable variations with regard to candidate biomarker proteins and miRNAs, most likely due to variation in sample size, processing and analytical methods, but also reflecting the complexity of SS and patient heterogeneity. However, interesting novel knowledge has emerged and further validation is needed to confirm their potential role as biomarkers in SS.
Topics: Humans; Sjogren's Syndrome; MicroRNAs; Proteomics; Saliva; Biomarkers
PubMed: 37275849
DOI: 10.3389/fimmu.2023.1183195 -
Rheumatology (Oxford, England) Feb 2023SSc is an auto-immune disease characterized by life-threatening manifestations such as lung fibrosis or pulmonary arterial hypertension. Symptoms with a detrimental... (Review)
Review
SSc is an auto-immune disease characterized by life-threatening manifestations such as lung fibrosis or pulmonary arterial hypertension. Symptoms with a detrimental impact on quality of life are also reported and sicca syndrome (xerostomia, xeropthalmia) is present in up to 80% of patients with SSc. Sicca syndrome can occur in the absence of overlap with Sjögren's disease and recent studies highlight that fibrosis of minor and major salivary glands, directly linked to the pathogenesis of SSc, could be a major contributor of xerostomia in SSc. This narrative review provides an overview of the clinical presentation, diagnostic strategies, management and future perspectives on sicca syndrome in patients with SSc.
Topics: Humans; Sjogren's Syndrome; Quality of Life; Scleroderma, Systemic; Xerostomia; Salivary Glands
PubMed: 35866609
DOI: 10.1093/rheumatology/keac412