1,720,962 research outputs found
One year in review 2017: systemic lupus erythematosus
No full textSystemic lupus erythematosus (SLE) is a chronic autoimmune disease that predominately affects women. It is characterised by a broad spectrum of clinical manifestations, however, its course and organ involvement are unpredictable. Although over the last few decades an improvement in survival for SLE patients has been observed, pathogenic mechanisms underlying this disease are still unclear. Comorbidities, due to both disease and treatment, as well as multiple aspects of SLE, are under intensive investigation. Following the previous annual reviews of this series, we hereby provide a critical digest of the recent papers on SLE focusing on pathogenesis, clinical and laboratory features, as well as current and new therapeutic strategies published over the last year
One year in review 2017: systemic lupus erythematosus
No full textSystemic lupus erythematosus (SLE) is a chronic autoimmune disease that predominately affects women. It is characterised by a broad spectrum of clinical manifestations, however, its course and organ involvement are unpredictable. Although over the last few decades an improvement in survival for SLE patients has been observed, pathogenic mechanisms underlying this disease are still unclear. Comorbidities, due to both disease and treatment, as well as multiple aspects of SLE, are under intensive investigation. Following the previous annual reviews of this series, we hereby provide a critical digest of the recent papers on SLE focusing on pathogenesis, clinical and laboratory features, as well as current and new therapeutic strategies published over the last year
Lymphoma and Lymphomagenesis in Primary Sjögren’s Syndrome
No full textPrimary Sjögren’s syndrome (pSS) is a systemic autoimmune disease mainly affecting exocrine glands and leading to impaired secretory function. The clinical picture is dominated by signs and symptoms of mucosal dryness and the course of the disease is mild and indolent in the majority of cases. However, a subgroup of patients can also experience extraglandular manifestations that worsen the disease prognosis. pSS patients are consistently found to have a higher risk of developing non-Hodgkin lymphoma (NHL) compared with patients with other autimmune disorders and to the general population. NHL is the most severe comorbidity that can occur in pSS, therefore recent research has aimed to identify reliable clinical, serological, and histological biomarkers able to predict NHL development in these subjects. This review article encompasses the body of evidence published so far in this field highlighting the challenges and pitfalls of different biomarkers within clinical practice. We also provide an overview of epidemiological data, diagnostic procedures, and evidence-based treatment strategies for NHL in pSS
One year in review 2018: Sjögren's syndrome
No full textSjögren'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
Nailfold capillaroscopy in Sjogren's Syndrome: Systematic literature review and analysis of a monocentric cohort
No full textBackground: Nailfold capillaroscopy (NC) is a non-invasive, reproducible imaging method to assess microcirculation in rheumatic diseases. In contrast to well-defined capillaroscopic findings in patients with systemic sclerosis, specific capillaroscopic findings in Sjögren’s Syndrome (SS) have not been documented.
Methods: The literature was systematically reviewed in three databases (Pubmed/Embase/Web of Science) to identify the role of NC in SS. The results were gathered for capillaroscopic parameters, and in a second time, an overview of correlations between these with clinical and laboratory values was given. All original studies to date published were revised. A quality assessment was applied to all included studies based on: sample size, well described target population, presence of a control group, capillaroscopic tool specifications and/or standardly applied methodology, clear definitions and descriptions of capillaroscopic parameters, statistic analysis used. Following the systematic review of literature (SRL), a prospective monocentric study was carried out in the Rheumatology Unit (Department of Medicine, University of Perugia) from September to November 2019. The NC was performed on 14 healthy controls (HC) and 20 patients with Sjögren’s Syndrome (SS).
Either qualitative and quantitative assessment were applied aimed to identify more frequent capillaroscopic alterations and/or pattern. Statistical analysis was performed using Student’s t-test and Mann–Whitney U-test.
Results: The literature search resulted in 826 hits. Based on title and abstract screening 519 original studies were retained and of these, 18 full texts describe an assessment by NC in SS. Finally, seven studies passed the quality assessment and form the object of this review: five case-control studies (only two studies compared SS with HC) and two case-series. The studies included on average of 61 SS patients (range 18-136).
Five studies performed a quantitative assessment of the NC images describing presence of dilations and haemorrhages in SS whereas abnormal morphology and tortuosity were equally seen in SS patients as in HC. One study performed a semi-quantitative assessment and any significant difference between SS and HC was shown. Six studies performed a qualitative assessment describing normal, non-specific and scleroderma pattern in SS patients. Only one study suggests an association between Raynaud’s phenomenon (RP), anticentromere autoantibodies (ACA) and capillaroscopic findings, though the results were not confirmed by analysis in external cohorts.
In our study the only significant difference between SS and HC was observed for the mean number of enlarged capillaries. No capillaroscopic differences in SS patients with and without RP were found, SS patients with double positivity (anti-SSA/Ro, anti-SSB/La antibodies) more frequently showed haemorrhages than patients with isolated positive anti-SSA/Ro antibodies.
Conclusion: Until now, the nailfold capillaroscopic assessment in patients with SS has not been uniformly clarified. Overall, the reviewed studies in literature show that capillaroscopic findings observed in SS are not significantly different from those observed in HC. In our study we tried to overcome some limits of previous investigations. We observed two statistically significant differences between SS and HC. In particular, more enlarged capillaries was seen in SS. In addition an increased number of haemorrhages was observed in double positive SS also compared to subjects with isolated anti-Ro circulating antibodies. Further research in this domain and on a larger sample of patients is needed to attest the reliability of this results
The prevalence and relevance of traditional cardiovascular risk factors in primary Sjögren's syndrome
No full textAccelerated atherosclerosis is a distinct feature of some inflammatory and autoimmune disorders and several specific autoimmune mechanisms and persistent inflammation have been identified to exert a pivotal role in precocious atherosclerotic damage in these disorders. Although increased atherosclerotic risk has been well established in some rheumatic autoimmune systemic diseases, such as systemic lupus erythematosus and rheumatoid arthritis, reliable data regarding the prevalence and pathogenetic mechanisms associated with increased atherosclerotic damage in primary Sjögren's syndrome are scarse. Indeed, primary Sjögren's syndrome is an autoimmune disorder characterised by chronic inflammation and autoimmune dysregulation that shares many pathogenic mechanisms and clinical features with systemic lupus erythematosus and rheumatoid arthitis. Higher prevalence of subclinical atherosclerosis has been observed in primary Sjögren's syndrome patients and recent population-based studies demonstrated an increased risk of cardiovascular events in these patients in comparison to general population. Among mechanisms associated with atherosclerotic damage, the prevalence and the role of traditional cardiovascular risk factors have been poorly investigated. In particular, the issue of whether the presence of these cardiovascular risk factors is associated with an increased risk of cardiovascular events needs to be further explored
Discrepancy between subjective symptoms, objective measures and disease activity indexes: the lesson of primary Sjögren's syndrome
No full textMucosal dryness is a key clinical feature in primary Sjögren's syndrome (pSS) and its assessment relies on both objective measurement of residual secretion and subjective symptoms reported by patients. However, while the objective assessment and grading of glandular dysfunction can be easily performed, the spectrum of clinical symptoms encompassed by the terms 'dry eye' and 'dry mouth' is wide and heterogeneous. Therefore, patient reported outcomes (PROs) for dryness in pSS poorly correlate with the amount of glandular secretion. In addition, subjective dryness is not correlated with the severity of systemic disease and severely affects the patient quality of life even in presence of active extraglandular manifestations. The purpose of this review article is to provide an overview of glandular dysfunction in pSS as well as the impact of discrepancy between objective assessment, subjective symptom and extraglandular disease activity on disease management
One year in review 2019: Sjögren's syndrome
No full textPrimary 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
Going Beyond Counting First Authors in Author Co-citation Analysis
Full text linkThe present study examines one of the fundamental aspects of author co-citation analysis (ACA) - the way co-citation
counts are defined. Co-citation counting provides the data on which all subsequent statistical analyses and mappings
are based, and we compare ACA results based on two different types of co-citation counting - the traditional type that
only counts the first one among a cited work's authors on the one hand and a non-traditional type that takes into
account the first 5 authors of a cited work on the other hand. Results indicate that the picture produced through this non-traditional author co-citation counting contains more coherent author groups and is therefore considerably clearer. However, this picture represents fewer specialties in the research field being studied than that produced through the traditional first-author co-citation counting when the same number of top-ranked authors is selected and analyzed. Reasons for these effects are discussed
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