Journal of Scleroderma and Related Disorders最新文献

{"title":"Views of nutrition needs in patients with systemic sclerosis.","authors":"De-Arne Samm, Aimee Macoustra, Rhiannon Crane, Leah McWilliams, Susanna Proudman, Lee-Anne S Chapple","doi":"10.1177/23971983241264868","DOIUrl":"10.1177/23971983241264868","url":null,"abstract":"<p><strong>Background and aims: </strong>Systemic sclerosis is a debilitating inflammatory condition synonymous with gastrointestinal symptoms which have the potential to impact dietary intake and nutritional status. This study aimed to describe symptoms experienced by patients with systemic sclerosis that may impact on dietary intake and assess nutrition education preferences in this cohort.</p><p><strong>Methods: </strong>A 24-item online qualitative and quantitative survey distributed via REDCap^® was conducted in adult patients (aged ⩾18 years) living with systemic sclerosis and attending outpatient services at a single healthcare setting from January to March 2022. Data were collected on demographics, symptoms that may impact dietary intake, nutrition priorities and preferred nutrition education models. Data are mean ± standard deviation or number (%).</p><p><strong>Results: </strong>Of 322 eligible patients, 156 (48%) participated (63 ± 12 years, 86% female, body mass index 27 ± 7 kg/m²). Most patients experienced gastrointestinal conditions (n = 123/155; 79%), which occurred daily in 26% (n = 40/155) of patients. A third of patients (n = 48/156; 31%) reported diet manipulation for symptom management. Recent weight loss was common (n = 36/154; 23% of patients). Less than a third of patients had seen a dietitian (n = 45; 29%), while 69% of patients (n = 107) desired dietetic consultancy. The preferred methods of consultation were written resources and face-to-face, respectively, and systemic sclerosis symptom management (n = 100; 64%) and losing weight (n = 53; 34%) were the most desired education topics reported.</p><p><strong>Conclusion: </strong>Gastrointestinal conditions are common and occur frequently in patients with systemic sclerosis. Patients want to engage with dietetics services to better manage symptoms via face-to-face consultations and written resources. These results will inform future dietetic service delivery.</p>","PeriodicalId":17036,"journal":{"name":"Journal of Scleroderma and Related Disorders","volume":null,"pages":null},"PeriodicalIF":1.4,"publicationDate":"2024-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11462576/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142400577","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Atypical morphoea, a herald of two malignancies: Lung adenocarcinoma and a neuroendocrine tumour.","authors":"Harmeet Bhullar, Miki Wada, Mandana Nikpour, Amanda M Saracino","doi":"10.1177/23971983241254442","DOIUrl":"https://doi.org/10.1177/23971983241254442","url":null,"abstract":"<p><strong>Introduction: </strong>Morphoea is a disorder characterised by fibrosis and inflammation of the skin and on rare occasions can be precipitated by malignancy. Here, we describe a case of morphoea unmasking two malignancies.</p><p><strong>Case description: </strong>A 73-year-old woman presented with circumferential lower limb skin thickening, associated with violaceous, doughy oedema and significantly impaired mobility. Histology confirmed dermal sclerosis with no increased mucin and broader investigations excluded systemic sclerosis, scleromyxoedema and scleroedema. An atypical morphoea was diagnosed. In the context of atypical and subsequently treatment-resistant disease, further imaging uncovered a lung adenocarcinoma which was promptly treated. Despite this, the patient's atypical oedematous skin sclerosis continued to progress proximally, and she developed flatulence, bloating and atypical flushing. This prompted further investigation, which revealed a metastatic neuroendocrine tumour. The patient was commenced on octreotide, with rapid improvement in all her cutaneous and systemic symptoms.</p><p><strong>Conclusion: </strong>Atypical morphoea can be a herald for an underlying malignancy, representing a paraneoplastic presentation. Progressive treatment-resistant morphoea may be an indicator of metastatic disease, or in our case a second malignancy.</p>","PeriodicalId":17036,"journal":{"name":"Journal of Scleroderma and Related Disorders","volume":null,"pages":null},"PeriodicalIF":1.4,"publicationDate":"2024-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11457777/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142391425","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"N-terminal pro-brain natriuretic peptide is associated with pulmonary hypertension or diastolic dysfunction in patients with systemic sclerosis: An Australian prospective cross-sectional study.","authors":"Francis J Ha, Zoe Brown, Wendy Stevens, David Prior, Laura Ross, Nava Ferdowsi, Mandana Nikpour, Andrew T Burns","doi":"10.1177/23971983241249209","DOIUrl":"https://doi.org/10.1177/23971983241249209","url":null,"abstract":"<p><strong>Introduction: </strong>Pulmonary arterial hypertension and left ventricular diastolic dysfunction are associated with significant morbidity and mortality in systemic sclerosis. N-terminal pro-brain natriuretic peptide has been proposed as part of composite screening algorithms for pulmonary arterial hypertension. Our aim was to assess the prevalence of pulmonary hypertension and diastolic dysfunction, and evaluate their association with serum N-terminal pro-brain natriuretic peptide in systemic sclerosis patients.</p><p><strong>Methods: </strong>Patients with systemic sclerosis were prospectively enrolled to undergo N-terminal pro-brain natriuretic peptide testing and transthoracic echocardiography at a tertiary Australian centre from January to October 2022. We collected demographic and transthoracic echocardiography variables including pulmonary hypertension estimated by tricuspid regurgitant velocity and diastolic dysfunction assessed by the ASE/EACVI 2016 guidelines. Pearson's correlation coefficient was used to evaluate association between N-terminal pro-brain natriuretic peptide and echocardiographic parameters.</p><p><strong>Results: </strong>Sixty-one patients were enrolled (median age = 62 years (interquartile range = 55-69 years); 84% female). Two-thirds of patients had limited systemic sclerosis (40/61). Five patients (8%) had high likelihood of pulmonary hypertension by transthoracic echocardiography. Seven patients (11%) had diastolic dysfunction; however, seven patients (11%) had indeterminate diastology. Six patients underwent right heart catheterisation, with five patients diagnosed with pulmonary hypertension. N-terminal pro-brain natriuretic peptide in patients with pulmonary hypertension or diastolic dysfunction was significantly higher (median = 207 and 226 pg/mL, respectively) compared to patients without either condition (median = 69 pg/mL, p = 0.01). N-terminal pro-brain natriuretic peptide showed a statistically significant although limited correlation with estimated pulmonary pressures measured by tricuspid regurgitant velocity (r = 0.44, p = 0.002) and left ventricular filling pressures (r = 0.27, p = 0.04).</p><p><strong>Conclusion: </strong>Pulmonary hypertension and diastolic dysfunction are both observed in systemic sclerosis. N-terminal pro-brain natriuretic peptide is associated with both conditions; however, it cannot distinguish between the two disease processes. Right heart catheterisation may be required to make this distinction.</p>","PeriodicalId":17036,"journal":{"name":"Journal of Scleroderma and Related Disorders","volume":null,"pages":null},"PeriodicalIF":1.4,"publicationDate":"2024-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11457770/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142391428","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Coronary artery calcification is prevalent in systemic sclerosis and is associated with adverse prognosis.","authors":"Jennifer Rossdale, John Graby, Maredudd Harris, Calum Jones, Davyd Greenish, Jessica Bartlett, Andrew Gilroy, Jamie Sanghera, John D Pauling, Sarah Skeoch, Victoria Flower, Rob Mackenzie Ross, Jay Suntharalingam, Jonathan Cl Rodrigues","doi":"10.1177/23971983241264090","DOIUrl":"10.1177/23971983241264090","url":null,"abstract":"<p><strong>Objective: </strong>Coronary artery calcification assessed on thoracic computed tomography represents the calcific component of established coronary artery disease, is a biomarker of total atheromatous plaque burden and predicts mortality. Systemic sclerosis is a pro-inflammatory condition, and inflammation is also a driver of coronary artery disease. We assessed coronary artery calcification prevalence, mortality risk and potential clinical impact on primary prevention in a cohort of patients with systemic sclerosis, differentiated by clinical phenotype including the presence of interstitial lung disease and pulmonary arterial hypertension.</p><p><strong>Methods: </strong>Retrospective analysis of 258 computed tomographies in systemic sclerosis patients from three prospectively maintained clinical and research databases at a single tertiary rheumatology/pulmonary hypertension (PH) service between March 2007 and September 2020 (mean age = 65 ± 12, 14% male). Co-morbidities, statin prescription and all-cause mortality were recorded. Patients were subtyped according to underlying systemic sclerosis complications. Computed tomographies were re-reviewed for coronary artery calcification; severity was graded using a 4-point scale per vessel and summed for total coronary artery calcification score. The impact of reporting coronary artery calcification was assessed against pre-existing statin prescriptions.</p><p><strong>Results: </strong>Coronary artery calcification was present in 58% (149/258). Coronary artery calcification was more prevalent in systemic sclerosis-pulmonary arterial hypertension than in systemic sclerosis subgroups with interstitial lung disease or without pulmonary arterial hypertension, controlling for age, sex, co-morbidities and smoking status (71%; <i>χ</i> ²(13) = 81.4; <i>p</i> < 0.001). The presence and severity of coronary artery calcification were associated with increased risk of mortality independently of age and co-morbidities (hazard ratio = 2.8; 95% confidence interval = 1.2-6.6; <i>p</i> = 0.018). The 'number needed to report' coronary artery calcification presence to potentially impact management was 3.</p><p><strong>Conclusions: </strong>Coronary artery calcification is common in systemic sclerosis. Coronary artery calcification predicts mortality independently of age and confounding co-morbidities which suggests this finding has clinical relevance and is a potential target for screening and therapeutic intervention.</p>","PeriodicalId":17036,"journal":{"name":"Journal of Scleroderma and Related Disorders","volume":null,"pages":null},"PeriodicalIF":1.4,"publicationDate":"2024-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11459481/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142391427","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Changes in work and adequacy of financial resources during COVID-19 among people with systemic sclerosis: A Scleroderma Patient-centered Intervention Network study.","authors":"Claire Adams, Elsa-Lynn Nassar, Marie-Eve Carrier, Linda Kwakkenbos, Richard S Henry, Gabrielle Virgili-Gervais, Sophie Hu, Susan J Bartlett, Catherine Fortuné, Amy Gietzen, Karen Gottesman, Geneviève Guillot, Marie Hudson, Amanda Lawrie-Jones, Nancy Lewis, Vanessa Malcarne, Maureen D Mayes, Scott B Patten, Michelle Richard, Maureen Sauvé, John Varga, Joep Welling, Robyn Wojeck, Luc Mouthon, Andrea Benedetti, Brett D Thombs","doi":"10.1177/23971983241262655","DOIUrl":"https://doi.org/10.1177/23971983241262655","url":null,"abstract":"<p><strong>Introduction/objective: </strong>We investigated (1) work status changes during COVID-19, (2) financial resource adequacy, (3) preferences for work requirements (e.g. remote, workplace, mixed) and (4) work requirements versus preferences, among people with systemic sclerosis.</p><p><strong>Methods: </strong>This was a cross-sectional study of participants in the Scleroderma Patient-centered Intervention Network COVID-19 Cohort, which enrolled participants from the ongoing Scleroderma Patient-centered Intervention Network Cohort and externally in April 2020. In August 2022, participants completed questions on work status, financial well-being using the Consumer Financial Protection Bureau Financial Well-Being Scale, work requirements and work requirement preferences.</p><p><strong>Results: </strong>A total of 298 participants with systemic sclerosis were included. Mean age was 58.6 years (SD = 11.4). There were 101 (34%) participants working at the start of the pandemic and still working in August 2022, 179 (60%) not working at the start of the pandemic and still not working, 10 (3%) who stopped working after April 2020 and 8 (3%) who started working. Mean financial well-being did not change from April 2020 to August 2022 (difference: 0.2 points; 95% confidence interval: -1.1 to 0.7). Working participants (N = 109) preferred flexible work requirements (N = 34, 31%) or working entirely remotely (N = 32, 29%), but most were required to work entirely at a workplace (N = 35, 32%) or combined workplace and remotely with a fixed schedule (N = 31, 28%).</p><p><strong>Conclusion: </strong>Work status and financial well-being did not change substantively among people with systemic sclerosis during the pandemic. Flexible work policies may support people with systemic sclerosis to work.</p>","PeriodicalId":17036,"journal":{"name":"Journal of Scleroderma and Related Disorders","volume":null,"pages":null},"PeriodicalIF":1.4,"publicationDate":"2024-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11457775/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142391426","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Clinician and patient experiences of managing and living with oral and dental manifestations of scleroderma: A scoping review.","authors":"Tyler J Mills, Elizabeth Price, Vishal R Aggarwal, Francesco Del Galdo, Liz Walker","doi":"10.1177/23971983231193515","DOIUrl":"10.1177/23971983231193515","url":null,"abstract":"<p><p>Oral and dental manifestations of scleroderma are extremely common, yet they are often overlooked within rheumatology and poorly understood within dentistry. Previous research has indicated the need to understand the oral and dental experiences of people living with scleroderma and those involved in their care. This scoping review aims, for the first time, to comprehensively map what is known regarding the identification and management of oral and dental manifestations of scleroderma, how these are experienced by people living with scleroderma, and to explore key characteristics of barriers and enablers to good oral and dental care in scleroderma. A scoping review was conducted using six databases (Embase, PubMed, PsychINFO, ASSIA, Scopus and SSCI), according to the Preferred Reporting Items for Systematic Reviews and Meta-analyses - extension for Scoping Review. Grey literature was also included. Studies were eligible for inclusion if the full text and abstract were available in English, published between 2002 and 2022, and focused on the concept of oral and dental care in adults with scleroderma, either relating to identification and management, enablers and barriers to best practice, or patient experiences and well-being. Qualitative research which seeks to understand patients' lived experiences was a notable gap in the literature. Similarly, there was a significant lack of focus on the oral and dental manifestations of scleroderma in rheumatology. Three key features were identified which would facilitate best practice in research and clinical contexts: the necessity of multidisciplinary care; the necessity of centralising patient experience; and the necessity of mitigating barriers to dental care. We conclude that increased awareness of scleroderma within dentistry and streamlining referral procedures between the disciplines of dentistry and rheumatology, to enable the early identification and management of scleroderma, are crucial.</p>","PeriodicalId":17036,"journal":{"name":"Journal of Scleroderma and Related Disorders","volume":null,"pages":null},"PeriodicalIF":1.4,"publicationDate":"2024-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11188842/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"48410320","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Is anticoagulative therapy in systemic sclerosis to be reconsidered?","authors":"Francesco Marongiu, Maria Filomena Ruberto, Doris Barcellona","doi":"10.1177/23971983241256250","DOIUrl":"10.1177/23971983241256250","url":null,"abstract":"<p><p>Systemic sclerosis is a rare disease with a high mortality rate. It is a multisystem connective tissue disease due to endothelial autoimmune activation along with tissue and vascular fibrosis, inducing vasculopathy, with an angiogenesis wasting. The endothelial damage provokes platelet activation and immune cell adhesion. The detachment of endothelial cells leads to the interaction of platelets and collagen present in the exposed subendothelial layer. This provokes the activation of several coagulative factors, inducing a pro-thrombotic condition by thrombin generation, which converts fibrinogen into fibrin. Moreover, thrombin has other functions, such as the induction of hyperplasia in smooth muscle cells and fibroblasts, thereby favouring fibrosis. An increased risk of venous thromboembolism has been found in systemic sclerosis, whereas pulmonary hypertension may be due to the obstruction of small pulmonary arteries. Pulmonary veno-occlusive disease may also occur. Warfarin showed inconsistent results, while the outcomes of a randomised, placebo-controlled clinical trial on apixaban versus placebo are still awaited. A new anticoagulation strategy based on anti-factor XI drugs is being developed, with the aim of achieving optimal anticoagulation along with a low risk of bleeding. The molecule types under investigation in this category include monoclonal antibodies, small molecules, natural inhibitors, antisense oligonucleotides, and aptamers. Patients with systemic sclerosis may be ideal candidates for clinical trials planned to analyse the efficacy and safety of these molecules.</p>","PeriodicalId":17036,"journal":{"name":"Journal of Scleroderma and Related Disorders","volume":null,"pages":null},"PeriodicalIF":1.4,"publicationDate":"2024-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11188846/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141442935","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Can machine learning assist in systemic sclerosis diagnosis and management? A scoping review","authors":"Eric P. McMullen, Rajan Grewal, Kyle Storm, Lawrence Mbuagbaw, Maxine Maretzki, Maggie J Larché","doi":"10.1177/23971983241253718","DOIUrl":"https://doi.org/10.1177/23971983241253718","url":null,"abstract":"This scoping review aims to summarize the existing literature on how machine learning can be used to impact systemic sclerosis diagnosis, management, and treatment. Following Preferred Reporting Items for Systematic reviews and Meta-Analyses extension for Scoping Reviews (PRISMA-ScR) reporting guidelines, Embase, Web of Science, Medline (PubMed), IEEE Xplore, and ACM Digital Library were searched from inception to 3 March 2024, for primary literature reporting on machine learning models in any capacity regarding scleroderma. Following robust triaging, 11 retrospective studies were included in this scoping review. Three studies focused on the diagnosis of scleroderma to influence preferred management and nine studies on treatment and predicting treatment response to scleroderma. Nine studies used supervision in their machine learning model training; two used supervised and unsupervised training and one used solely unsupervised training. A total of 817 patients were included in the data sets. Seven of the included articles used patients from the United States, one from Belgium, two from Japan, and two from China. Although currently limited to retrospective studies, the results indicate that machine learning modeling may have a role in early diagnosis, management, therapeutic decision-making, and in the development of future therapies for systemic sclerosis. Prospective studies examining the use of machine learning in clinical practice are recommended to confirm the utility of machine learning in patients with systemic sclerosis.","PeriodicalId":17036,"journal":{"name":"Journal of Scleroderma and Related Disorders","volume":null,"pages":null},"PeriodicalIF":2.0,"publicationDate":"2024-05-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141102012","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Oral health in systemic sclerosis: A scoping review","authors":"Maria Gagarine, Mara Cañedo-Ayala, Vanessa Cook, Marie-Nicole Discepola, Geneviève Guillot, David M Leader, Mathew Awt Lim, Tami Yap, Daniel E Furst, Amy Gietzen, Jo-Ann Lapointe McKenzie, Shelley Van Pelt, Louise Vidiricaire, M. Carrier, Ankur Krishnan, Lydia Tao, L. Kwakkenbos, Brett D. Thombs","doi":"10.1177/23971983241252899","DOIUrl":"https://doi.org/10.1177/23971983241252899","url":null,"abstract":"Limited research on oral health in systematic sclerosis poses a significant challenge for people with systematic sclerosis and health care professionals. We conducted a scoping review to map existing research on oral health–related quality of life in systematic sclerosis across domains including (1) oral manifestations or symptoms, (2) functional consequences, (3) psychosocial aspects, (4) associated patient factors and (5) self-care and management considerations. We searched MEDLINE, EMBASE, SCOPUS and CINAHL databases to 10 March 2023 for studies on oral health in people with systematic sclerosis. Study data were mapped into pre-specified domains of oral health–related quality of life, subtopics and types of research and reported in tabular form. Of 1460 unique studies reviewed, 91 were eligible and included in the scoping review; of these, 69 (76%) were published as full-text versions, 72 (79%) were from Europe or North America, and 63 (69%) included ⩽50 participants. Only 1 study had >200 participants (N = 382). By domains, 85 (93%) studies reported aspects of oral manifestations or symptoms, with the most common subtopics being oral opening and mandibular function, sicca, periodontitis and soft tissue changes and pathology; 36 (40%) studies provided information on associated patient factors, 23 (25%) on self-care and management, 19 (21%) on psychosocial aspects and 6 (7%) on functional consequences. No studies related to interventions included >50 participants. Most studies on oral health in systematic sclerosis report on manifestations or symptoms, and there are relatively few studies on other important domains. Most studies have been conducted with small samples. Updated systematic reviews should evaluate the quality of existing evidence in domains where enough studies have been done. Large, well-conducted primary studies are needed to address knowledge gaps across domains, including studies that test approaches for supporting oral self-care, improving routine professional care and addressing major disability-causing manifestations.","PeriodicalId":17036,"journal":{"name":"Journal of Scleroderma and Related Disorders","volume":null,"pages":null},"PeriodicalIF":2.0,"publicationDate":"2024-05-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141102238","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Within-person fluctuations of fatigue in patients with a clinical diagnosis of systemic sclerosis and its relationship with mood, pain, sleep and physical activity","authors":"A. Velauthapillai, M. Vonk, Cornelia H M van den Ende, Johanna E. Vriezekolk","doi":"10.1177/23971983241242836","DOIUrl":"https://doi.org/10.1177/23971983241242836","url":null,"abstract":"To explore the within-person fluctuations of fatigue in systemic sclerosis and its association with negative affect, positive affect, pain, perceived exertion of physical activity and quality of sleep. We performed an ecological momentary assessment study in adult patients with a clinical diagnosis of systemic sclerosis. During 14 days, patients completed daily assessments of fatigue severity, negative affect, positive affect, pain, quality of sleep and perceived exertion of physical activity at four fixed time points. The day-to-day fluctuations in fatigue were quantified by the intra-individual variance and probability of acute change, capturing the magnitude and frequency of clinical relevant within-person day-to-day fluctuations, respectively. Using multilevel models, the within-person association between fatigue and the daily assessments were analysed. Fifty-seven patients with systemic sclerosis participated. The mean (standard deviation) intra-individual variance was 1.08 (0.42) and the probability of acute change was mean (standard deviation) 0.40 (0.14), ranging from 0.08 to 0.77. For fatigue, a within-person variation of 51% was observed. Multilevel analyses showed that higher average levels and daily increases in negative affect, pain and perceived exertion of physical activity were associated with more fatigue, while the opposite was observed for positive affect and quality of sleep. Positive affect demonstrated the strongest association with fatigue fluctuations. This is the first quantitative study showing that fatigue in systemic sclerosis is characterized by a dynamic course and that approximately half of the day-to-day fluctuations within persons are clinically meaningful. Furthermore, our results indicate that integrating activities with positive impact on mood into fatigue treatment strategies might reduce the frequency of fatigue fluctuations.","PeriodicalId":17036,"journal":{"name":"Journal of Scleroderma and Related Disorders","volume":null,"pages":null},"PeriodicalIF":2.0,"publicationDate":"2024-05-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141022136","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}