scholarly journals Similarities between COVID-19 and systemic sclerosis early vasculopathy: A “viral” challenge for future research in scleroderma

2021 ◽  
pp. 102899
Author(s):  
Marco Matucci Cerinic ◽  
Michael Hughes ◽  
Gloria Taliani ◽  
Bashar Kahaleh
Keyword(s):  
Systemic Sclerosis ◽  
Future Research ◽  
Viral Challenge

scholarly journals Update on morbidity and mortality in systemic sclerosis–related interstitial lung disease

2020 ◽  
pp. 239719832091504
Author(s):  
Elizabeth R Volkmann ◽  
Aryeh Fischer
Keyword(s):  
Systemic Sclerosis ◽  
Interstitial Lung Disease ◽  
Lung Disease ◽  
Standard Of Care ◽  
Morbidity And Mortality ◽  
Therapeutic Interventions ◽  
Future Research ◽  
Current Standard ◽  
Term Survival ◽  
Hematopoietic Stem

Contemporary studies of systemic sclerosis consistently demonstrate that interstitial lung disease is a leading cause of disease-related death. This review summarizes morbidity and mortality outcomes in systemic sclerosis-related interstitial lung disease patients from high-quality observational and interventional studies over the last 50 years. The data presented suggest a trend for improved morbidity and mortality outcomes among present day systemic sclerosis–associated interstitial lung disease patients. Specifically, systemic sclerosis–associated interstitial lung disease patients appear to be living longer from the time of the initial diagnosis. Despite improved survival, the number one cause of death for most systemic sclerosis–associated interstitial lung disease patients remains respiratory failure from interstitial lung disease. This review describes the most important demographic, clinical, and biological factors, which affect mortality in systemic sclerosis–associated interstitial lung disease, and could be used to help stratify patients for closer monitoring and more aggressive initial treatment. The review concludes with an overview of future research needed to (1) understand how to personalize the care of systemic sclerosis–associated interstitial lung disease patients to improve morbidity and mortality outcomes; and (2) investigate whether novel therapeutic interventions (e.g. anti-fibrotics, hematopoietic stem cell transplantation) offer any meaningful long-term survival advantage over the current standard of care.

Pulmonary arterial hypertension screening of systemic sclerosis patients in clinical practice: an independent chart review

2017 ◽  
Vol 2 (3) ◽  
pp. 231-234 ◽  
Author(s):  
Dylan Kelly ◽  
Karen A. Beattie ◽  
Maggie J. Larché
Keyword(s):  
Systemic Sclerosis ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Chart Review ◽  
Screening Tests ◽  
Future Research ◽  
Increased Risk ◽  
Annual Screening ◽  
Pulmonary Arterial ◽  
Function Testing

Patients with systemic sclerosis (SSc) are at increased risk of pulmonary arterial hypertension (PAH). Guidelines recommend annual screening with pulmonary function testing (PFT) and transthoracic echocardiogram (TTE). Through auditing the charts of 11 rheumatologists associated with McMaster University, we evaluated the proportion of SSc patients without PAH or pulmonary fibrosis who receive annual TTE, PFT, and dyspnea screening. Screening rates between self-identified SSc experts and non-experts were compared. In cases where screening tests were abnormal, charts were reviewed for evidence of cardiologist or respirologist referral. In total, 136 patients’ charts were included. Annual screening for dyspnea was very common (88% of patients, 119/134). Annual PAH screening via TTE (74%, 100/135) and PFT (79%, 107/136) was less common. Annual dyspnea screening, TTE, and PFT were more commonly performed by SSc experts than by non-experts (94% vs. 83%, p = 0.03; 85% vs. 61%, p = 0.002; 93% vs. 62%, p<0.001, respectively). Nearly all patients with an abnormal TTE (10/11, 91%) and PFT (12/14, 86%) received appropriate referrals. Future research should explore reasons for differences in screening rates between SSc experts and non-experts. Given that rheumatologists screen for dyspnea more often than they order PFT and TTE, there may be barriers to ordering these tests that warrant further investigation.

131 Sleep Quality as a Pathway from Stress to Cold Symptom Severity

SLEEP ◽  
2021 ◽  
Vol 44 (Supplement_2) ◽  
pp. A53-A54
Author(s):  
Muskan Bansal ◽  
Sarah Ghose ◽  
Natalie Dautovich
Keyword(s):  
Sleep Quality ◽  
Perceived Stress ◽  
Common Cold ◽  
Respiratory Illness ◽  
Self Report ◽  
Future Research ◽  
Indirect Pathway ◽  
Present Sample ◽  
Viral Challenge ◽  
The Common

Abstract Introduction Stress is a known contributor to immune system suppression associated with higher illness susceptibility, including acute infectious respiratory illness, or the common cold. Sleep quality, shown to impact immunity, is an additional mechanism that may underlie the association between stress and cold symptomatology. Although the associations between stress and sleep and cold symptomatology have been examined separately, little is known about the mechanistic role of sleep in these associations. The present study examined sleep quality as a potential pathway between stress and cold symptomatology difference scores. Methods Archival data from the Common Cold Project (Pittsburgh Cold Study 3) were utilized for the present study. Participants were 213 adults (Mean Age=30.1 yrs., SD=10.9 yrs., 42.3% female) who completed a 5-day viral challenge and self-report measures of cold severity (Jackson Symptom Score; measured from beginning to end of viral challenge), baseline sleep quality (PSQI), and perceived stress as part of study participation. SPSS v 27 and Hayes’ PROCESS mediation macro were used to assess study aims. Age and sex were included as covariates. Results Greater perceived stress was significantly associated with worse sleep quality [B=.15, 95% CI .10, .21]. Sleep quality fully mediated the association between stress and changes in symptomatology; better sleep was associated with larger changes in cold severity [B=-.23, 95% CI -.43, -.04], defined as differences in symptomatology from beginning to end of the viral challenge, beyond stress alone. Zero-order correlation analyses revealed a trend level (r=.04, p=.06) association between sleep quality and aggregate cold severity, suggesting that as sleep improves, symptoms decrease. Conclusion Within the present sample, sleep quality surfaced as an indirect pathway linking stress to changes in cold severity. Better sleep was associated with greater changes in cold severity above perceived stress. These findings, together with the trend level, positive association between sleep quality and cold symptomatology, suggest that better sleep may be associated with less severe symptomatology. Future research should attend to mechanisms underlying the associations between stress, sleep, and cold symptomatology. Support (if any):

scholarly journals Sudden Cardiac Death in Systemic Sclerosis: Diagnostics to Assess Risk and Inform Management

Diagnostics ◽  
2021 ◽  
Vol 11 (10) ◽  
pp. 1781
Author(s):  
Laura Ross ◽  
Elizabeth Paratz ◽  
Murray Baron ◽  
André La Gerche ◽  
Mandana Nikpour
Keyword(s):  
Heart Disease ◽  
Systemic Sclerosis ◽  
Sudden Cardiac Death ◽  
Cardiac Disease ◽  
Cardiac Death ◽  
Diffuse Myocardial Fibrosis ◽  
Future Research ◽  
Disease Course ◽  
Future Research Agenda ◽  
Increased Risk

Cardiac disease is a leading cause of death in systemic sclerosis (SSc) and sudden cardiac death (SCD) is thought to occur more commonly in SSc than in the general population. Diffuse myocardial fibrosis, myocarditis and ischaemic heart disease are all prevalent in SSc and can be reasonably hypothesised to contribute to an increased risk of SCD. Despite this, SCD remains a relatively understudied area of SSc with little understood about SSc-specific risk factors and opportunities for primary prevention. In this review, we present an overview of the possible mechanisms of SCD in SSc and our current understanding of how each of these mechanisms may contribute to cardiac death. This review highlights the need for a future research agenda that addresses the underlying epidemiology of SCD in SSc and identifies opportunities for intervention to modify the disease course of heart disease in SSc.

Patient-reported outcome instruments in clinical trials of systemic sclerosis

2019 ◽  
Vol 5 (2) ◽  
pp. 90-102 ◽  
Author(s):  
John D Pauling ◽  
Joana Caetano ◽  
Corrado Campochiaro ◽  
Giacomo De Luca ◽  
Ana Maria Gheorghiu ◽  
...  
Keyword(s):  
Clinical Trial ◽  
Clinical Trials ◽  
Systemic Sclerosis ◽  
Patient Reported Outcome ◽  
Future Research ◽  
Valuable Insight ◽  
Surrogate Outcome ◽  
American College Of Rheumatology ◽  
Patient Reported ◽  
And Performance

Patient-reported outcome instruments provide valuable insight into disease-related morbidity known only to the patient and complement more objective outcome tools in the clinical trial setting. They are of particular importance in systemic sclerosis owing to the challenges around defining disease activity, the episodic nature of many disease-specific manifestations and the paucity of validated objective surrogate outcome measures for use in clinical trials. Early clinical trials of systemic sclerosis often incorporated legacy patient-reported outcome instruments, but the last 20 years has witnessed the emergence of several scleroderma-specific instruments that are now being routinely used alongside other outcomes in systemic sclerosis clinical trials. More recently, the value of patient-reported outcomes has been highlighted by their prominence in the American College of Rheumatology Combined Response Index for Systemic Sclerosis that has been utilized as the primary endpoint of recent clinical trials of early diffuse systemic sclerosis. This review considers the role and performance of the various patient-reported outcome instruments utilized in systemic sclerosis clinical trials, the current positioning of patient-reported outcome instruments within clinical trial endpoint models across the range of systemic sclerosis disease manifestations and, where applicable, we shall highlight areas for future research.

scholarly journals Gastrointestinal involvement in systemic sclerosis: Effects on morbidity and mortality and new therapeutic approaches

2019 ◽  
pp. 239719831989128 ◽  
Author(s):  
Anna-Maria Hoffmann-Vold ◽  
Elizabeth R. Volkmann
Keyword(s):  
Systemic Sclerosis ◽  
Gastrointestinal Symptoms ◽  
Morbidity And Mortality ◽  
Future Research ◽  
Gastrointestinal Involvement ◽  
New Therapeutic Approaches ◽  
Recent Developments ◽  
Tract Involvement ◽  
Future Research Directions ◽  
Promising Treatment

The majority of research studies in systemic sclerosis focus largely on addressing skin and cardiopulmonary manifestations. Fewer studies assess the pathogenesis and treatment of gastrointestinal tract involvement in systemic sclerosis, despite the fact that the majority of patients with systemic sclerosis have gastrointestinal manifestations and these manifestations are a leading cause of death in systemic sclerosis. The present review provides a comprehensive update on morbidity and mortality outcomes related to gastrointestinal involvement in systemic sclerosis. This review also describes conventional and emerging approaches to managing gastrointestinal symptoms in systemic sclerosis. Recent developments in systemic sclerosis–gastrointestinal research efforts have revealed promising treatment targets, including specific auto-antibodies and microbiota alterations. This review will conclude with an overview of future research directions that may improve our understanding of systemic sclerosis–gastrointestinal involvement and ultimately help to alleviate suffering from this devastating dimension of systemic sclerosis.

scholarly journals EULAR recommendations for the treatment of systemic sclerosis: a report from the EULAR Scleroderma Trials and Research group (EUSTAR)

2009 ◽  
Vol 68 (5) ◽  
pp. 620-628 ◽  
Author(s):  
O Kowal-Bielecka ◽  
R Landewé ◽  
J Avouac ◽  
S Chwiesko ◽  
I Miniati ◽  
...  
Keyword(s):  
Systemic Sclerosis ◽  
Clinical Research ◽  
Research Group ◽  
Task Force ◽  
Multiple Organ ◽  
Future Research ◽  
Scleroderma Renal Crisis ◽  
Evidence Based ◽  
Future Research Agenda ◽  
Organ Systems

Purpose:The optimal treatment of systemic sclerosis (SSc) is a challenge because the pathogenesis of SSc is unclear and it is an uncommon and clinically heterogeneous disease affecting multiple organ systems. The aim of the European League Against Rheumatism (EULAR) Scleroderma Trials and Research group (EUSTAR) was to develop evidence-based, consensus-derived recommendations for the treatment of SSc.Methods:To obtain and maintain a high level of intrinsic quality and comparability of this approach, EULAR standard operating procedures were followed. The task force comprised 18 SSc experts from Europe, the USA and Japan, two SSc patients and three fellows for literature research. The preliminary set of research questions concerning SSc treatment was provided by 74 EUSTAR centres.Results:Based on discussion of the clinical research evidence from published literature, and combining this with current expert opinion and clinical experience, 14 recommendations for the treatment of SSc were formulated. The final set includes the following recommendations: three on SSc-related digital vasculopathy (Raynaud’s phenomenon and ulcers); four on SSc-related pulmonary arterial hypertension; three on SSc-related gastrointestinal involvement; two on scleroderma renal crisis; one on SSc-related interstitial lung disease and one on skin involvement. Experts also formulated several questions for a future research agenda.Conclusions:Evidence-based, consensus-derived recommendations are useful for rheumatologists to help guide treatment for patients with SSc. These recommendations may also help to define directions for future clinical research in SSc.

Prevalence of systemic sclerosis in Colombia: Data from the National Health Registry 2012–2016

2019 ◽  
Vol 5 (2) ◽  
pp. 137-142 ◽  
Author(s):  
Daniel G Fernández-Ávila ◽  
Santiago Bernal-Macías ◽  
Juan M Gutiérrez ◽  
Diana N Rincón ◽  
Diego Rosselli
Keyword(s):  
Latin America ◽  
Systemic Sclerosis ◽  
National Health ◽  
National Health System ◽  
Public Information ◽  
Epidemiological Studies ◽  
Future Research ◽  
Ministry Of Health ◽  
Cross Sectional ◽  
Epidemiological Characteristics

Introduction: Systemic sclerosis is an autoimmune disease that characteristically presents with fibrosis and vasculopathy. In Latin America, the information on the epidemiology of this disease is scarce and records are essential to know its behavior in the populations. In Colombia, the Ministry of Health uses the SISPRO tool to collect information on the unified national health system which offers nearly universal coverage (around 95%). This public information makes it possible to perform epidemiological studies on different diseases. Objectives: Using the SISPRO data corresponding to the years 2012–2016, we analyzed the prevalence and characteristics of patients with systemic sclerosis. Methods: A descriptive cross-sectional study was performed based on the SISPRO data; we used as keywords the diagnoses of the International Classification of Diseases related to the diagnosis of systemic sclerosis. Results: A total of 11,300 individuals diagnosed with systemic sclerosis were documented. The estimated prevalence was 23.7 cases per 100,000 inhabitants (based on a total population of 47,663,162); this disease is more frequent in the age group of 65–69 years in females (77%), and has a female:male ratio of 3.27:1. Conclusion: This study describes the epidemiological characteristics of systemic sclerosis in Colombia, based on official statistics from the Ministry of Health. The results provide a new vision of this disease in Latin America and open the door to future research, in order to better understand the particular characteristics of this disease in our country and our region.

Parasite defense mechanisms in bees: behavior, immunity, antimicrobials, and symbionts

2019 ◽  
Vol 4 (1) ◽  
pp. 59-76 ◽  
Author(s):  
Alison E. Fowler ◽  
Rebecca E. Irwin ◽  
Lynn S. Adler
Keyword(s):  
Defense Mechanisms ◽  
Poor Quality ◽  
Future Research ◽  
Immune Priming ◽  
Social Bees ◽  
Bee Health ◽  
Landscape Scales ◽  
And Function ◽  
Solitary Species

Parasites are linked to the decline of some bee populations; thus, understanding defense mechanisms has important implications for bee health. Recent advances have improved our understanding of factors mediating bee health ranging from molecular to landscape scales, but often as disparate literatures. Here, we bring together these fields and summarize our current understanding of bee defense mechanisms including immunity, immunization, and transgenerational immune priming in social and solitary species. Additionally, the characterization of microbial diversity and function in some bee taxa has shed light on the importance of microbes for bee health, but we lack information that links microbial communities to parasite infection in most bee species. Studies are beginning to identify how bee defense mechanisms are affected by stressors such as poor-quality diets and pesticides, but further research on this topic is needed. We discuss how integrating research on host traits, microbial partners, and nutrition, as well as improving our knowledge base on wild and semi-social bees, will help inform future research, conservation efforts, and management.

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