Journal of Scleroderma and Related Disorders最新文献

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Factors related to acute heart failure in systemic sclerosis.
IF 1.4
Journal of Scleroderma and Related Disorders Pub Date : 2025-04-02 DOI: 10.1177/23971983251325681
Yusuf Ziya Sener, Seher Sener
{"title":"Factors related to acute heart failure in systemic sclerosis.","authors":"Yusuf Ziya Sener, Seher Sener","doi":"10.1177/23971983251325681","DOIUrl":"10.1177/23971983251325681","url":null,"abstract":"","PeriodicalId":17036,"journal":{"name":"Journal of Scleroderma and Related Disorders","volume":" ","pages":"23971983251325681"},"PeriodicalIF":1.4,"publicationDate":"2025-04-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11966619/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143795685","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}
引用次数: 0
Association between beta-1-adrenoceptor blockade and risk of Raynaud's phenomenon: Mendelian randomisation study.
IF 1.4
Journal of Scleroderma and Related Disorders Pub Date : 2025-03-25 DOI: 10.1177/23971983241312543
Jacob C Williams, Kira Rogers, Kathryn Coulson, David M Hughes, Michael Hughes, Sizheng Steven Zhao
{"title":"Association between beta-1-adrenoceptor blockade and risk of Raynaud's phenomenon: Mendelian randomisation study.","authors":"Jacob C Williams, Kira Rogers, Kathryn Coulson, David M Hughes, Michael Hughes, Sizheng Steven Zhao","doi":"10.1177/23971983241312543","DOIUrl":"10.1177/23971983241312543","url":null,"abstract":"<p><strong>Introduction/objectives: </strong>Raynaud's phenomenon is a common vasospastic disorder associated with reduced health-related quality of life and, occasionally, ischaemic tissue damage depending on aetiology. The effect of beta-1-adrenoceptor blockers (e.g. bisoprolol, atenolol) on Raynaud's phenomenon remains unclear. We aimed to assess the association between genetically mimicked beta-1-adrenoceptor blockade and the risk of Raynaud's phenomenon.</p><p><strong>Methods: </strong>We used two protein-coding single nucleotide polymorphisms in the <i>ADRB1</i> gene, rs1801252 (A > G; Ser49Gly) and rs1801253 (G > C; Arg389Gly), to derive an unweighted allele count as the instrumental variable, using individual-level UK Biobank data. Raynaud's phenomenon was defined using International Classification of Diseases or Read codes. We used the ratio method and analysis was performed separately using systolic and diastolic blood pressure as the biomarker. To examine the validity of this approach and the Raynaud's phenomenon case definition, we also tested the known association between phosphodiesterase-5 inhibition and Raynaud's phenomenon risk.</p><p><strong>Results: </strong>Analysis included 4743 individuals with Raynaud's phenomenon (mean age 58 years, 68% female) and 403,762 controls. There was no evidence of an effect of genetically mimicked beta-1-adrenoreceptor blockade on the risk of Raynaud's phenomenon, using systolic blood pressure (odds ratio = 0.93 per mmHg reduction; 95% confidence interval = [0.83, 1.04]; p = 0.19) or diastolic blood pressure (odds ratio = 0.91 per mmHg reduction; 95% confidence interval = [0.78, 1.05]; p = 0.19). The positive control exposure phosphodiesterase-5 inhibition was associated with reduced Raynaud's phenomenon risk.</p><p><strong>Conclusions: </strong>We found no genetic evidence to support a causal association between beta-1-adrenoceptor blockade and Raynaud's phenomenon risk in either direction. Randomised controlled trials are required to confirm the safety of beta-1-adrenoceptor blockers in people with Raynaud's phenomenon.</p>","PeriodicalId":17036,"journal":{"name":"Journal of Scleroderma and Related Disorders","volume":" ","pages":"23971983241312543"},"PeriodicalIF":1.4,"publicationDate":"2025-03-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11948267/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143753136","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}
引用次数: 0
The minimal clinically important difference of the scleroderma clinical trials consortium damage index.
IF 1.4
Journal of Scleroderma and Related Disorders Pub Date : 2025-03-25 DOI: 10.1177/23971983251327808
Murray Baron, Dylan Hansen, Susanna Proudman, Wendy Stevens, Mianbo Wang, Mandana Nikpour
{"title":"The minimal clinically important difference of the scleroderma clinical trials consortium damage index.","authors":"Murray Baron, Dylan Hansen, Susanna Proudman, Wendy Stevens, Mianbo Wang, Mandana Nikpour","doi":"10.1177/23971983251327808","DOIUrl":"10.1177/23971983251327808","url":null,"abstract":"<p><strong>Objective: </strong>The Scleroderma Clinical Trials Consortium Damage Index is an index of global damage in systemic sclerosis. The objective of this study is to determine the minimal clinically important difference of the Scleroderma Clinical Trials Consortium Damage Index.</p><p><strong>Methods: </strong>Patients in the Canadian Scleroderma Research Group registry and the Australian Scleroderma Cohort Study who completed Scleroderma Clinical Trials Consortium Damage Index scores and the SF36v2 at baseline and the first full follow-up visit were studied. To calculate the minimal clinically important difference, an anchor question came from SF36v2: \"Compared to one year ago, how would you rate your health in general?.\" Options were: much better, somewhat better, about the same, somewhat worse and much worse. We use the \"somewhat worse\" or \"much worse\" categories to indicate those with any worsening. We used four anchor methods: receiver operating characteristic curve, change difference, regression analysis, and average change.</p><p><strong>Results: </strong>We studied 1672 patients. Mean disease duration was 11.4 ± 10.0 years; 62.5% had diffuse cutaneous systemic sclerosis. Baseline mean Damage Index was 5.3 ± 4.2; mean change of Damage Index over 1 year was 0.9 ± 1.8 units. The calculated minimal clinically important difference values were 1 for receiver operating characteristic method, 0.625 for change difference, 0.1879 for regression analysis, and 1.37 for average change. Omitting the regression analysis method as an outlier, the mean of the other methods was 1.</p><p><strong>Conclusion: </strong>The most appropriate minimal clinically important difference for the Scleroderma Clinical Trials Consortium Damage Index is a change of ⩾ 1.0 units in the Scleroderma Clinical Trials Consortium Damage Index as is already recognized by patients as a significant change after 1 year. This can be applied to group means as well as to individuals where an ordinal change is required.</p>","PeriodicalId":17036,"journal":{"name":"Journal of Scleroderma and Related Disorders","volume":" ","pages":"23971983251327808"},"PeriodicalIF":1.4,"publicationDate":"2025-03-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11948227/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143753142","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}
引用次数: 0
Comparison of appearance dissatisfaction and appearance-related social discomfort among people with systemic sclerosis and burn injury.
IF 1.4
Journal of Scleroderma and Related Disorders Pub Date : 2025-03-24 DOI: 10.1177/23971983251326746
Julia Langleben, Tiffany Dal Santo, Meira Golberg, Kexin Li, Brett D Thombs
{"title":"Comparison of appearance dissatisfaction and appearance-related social discomfort among people with systemic sclerosis and burn injury.","authors":"Julia Langleben, Tiffany Dal Santo, Meira Golberg, Kexin Li, Brett D Thombs","doi":"10.1177/23971983251326746","DOIUrl":"10.1177/23971983251326746","url":null,"abstract":"<p><strong>Introduction/objective: </strong>Visible differences from medical conditions and injuries are associated with body image concerns, particularly among females and young adults. We compared dissatisfaction with appearance and social discomfort between people with systemic sclerosis and burn injury, since the extent and implications of appearance changes are well-established in burn injury.</p><p><strong>Methods: </strong>We searched PubMed, PsycInfo, EMBASE, and CINAHL to 8 December 2024 for studies that used the Satisfaction with Appearance Scale among adults with burn injury or systemic sclerosis. We emailed study authors and requested Satisfaction with Appearance Scale Dissatisfaction with Appearance and Social Discomfort subscale means and standard deviations for subgroups defined by sex (female, male) and age (18-44 years, 45-64 years, ⩾ 65 years). For each subgroup, we conducted a random-effects meta-analysis to estimate the difference between mean scores for people with burn injury and systemic sclerosis.</p><p><strong>Results: </strong>We identified 17 eligible studies from nine unique cohorts. We obtained subgroup results from two of three eligible burn cohorts (2658 participants, 98% of total eligible) and five of six eligible systemic sclerosis cohorts (3402 participants, 99% of total eligible participants). Dissatisfaction with Appearance subscale scores were higher among people with systemic sclerosis compared to burn injury by 2.2 to 5.7 points (standardized mean difference = 0.20 to 0.53) for females and males across all age groups (<i>p</i> < 0.05 for males aged 18-44 and 45-64 years). For social discomfort, differences were close to zero (standardized mean difference < 0.10) for females aged 18-44 and 45-64 years. For females aged ⩾ 65 years and all male age groups, scores were higher in systemic sclerosis than burn injury (standardized mean difference = 0.22 to 0.45), although none were statistically significant.</p><p><strong>Conclusion: </strong>Dissatisfaction with appearance and social discomfort appear to be similar or greater among people with systemic sclerosis compared to people who have been hospitalized with a burn injury.</p>","PeriodicalId":17036,"journal":{"name":"Journal of Scleroderma and Related Disorders","volume":" ","pages":"23971983251326746"},"PeriodicalIF":1.4,"publicationDate":"2025-03-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11948249/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143753137","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}
引用次数: 0
Physician-patient communication in the treatment of systemic sclerosis-associated interstitial lung disease: A narrative review and recommendations.
IF 1.4
Journal of Scleroderma and Related Disorders Pub Date : 2025-03-20 DOI: 10.1177/23971983251324803
Masataka Kuwana, Aiko Saito, Sue Farrington, Ilaria Galetti, Christopher P Denton, Dinesh Khanna
{"title":"Physician-patient communication in the treatment of systemic sclerosis-associated interstitial lung disease: A narrative review and recommendations.","authors":"Masataka Kuwana, Aiko Saito, Sue Farrington, Ilaria Galetti, Christopher P Denton, Dinesh Khanna","doi":"10.1177/23971983251324803","DOIUrl":"10.1177/23971983251324803","url":null,"abstract":"<p><p>Interstitial lung disease is a common complication and cause of mortality in patients with systemic sclerosis. Pharmacotherapy for systemic sclerosis-associated interstitial lung disease was mostly limited to off-label use of immunosuppressive drugs until recently, when two drugs became licenced for this condition: nintedanib, an antifibrotic agent, and tocilizumab, a targeted anti-inflammatory/immunomodulatory therapy licenced in the United States. In chronic diseases, communication between physicians and patients is associated with treatment adherence, patient satisfaction, and clinical outcomes. This review of physician-patient communication during systemic sclerosis-associated interstitial lung disease treatment covers key issues identified by studies in Japan, the United States and Europe, as well as the clinical experience, opinion, and recommendations of the physician and patient advocate authors. As discussed, recent surveys in Japan found low usage of guideline-recommended immunosuppressive drugs for systemic sclerosis-associated interstitial lung disease and physician dissatisfaction with them. Physicians and patients in Japan also had differing perceptions about what had been said during consultations, suggesting the need to improve physician-patient communication. Other studies in Japan, the United States and Europe made several key findings. Notably, most patients feel uneasy at the diagnosis of systemic sclerosis-associated interstitial lung disease, and both physicians and patients avoid discussing prognosis and mortality. Furthermore, a white-coat barrier hinders patients raising topics important to them. For physicians, listening and empathy may be key for building rapport with patients. Importantly, physicians and patients have different cognitive models of systemic sclerosis-associated interstitial lung disease, creating communication challenges. There are also similarities and differences in clinical practice and physician-patient communication between countries that are important to consider. From the patient's perspective, key factors include the quality of the first consultation, physician empathy and active listening, and space to ask questions. Efforts to improve physician-patient communication include peer mentoring, patient self-education (such as the 'Self-Manage Scleroderma' website from the University of Michigan), and shared decision-making - although not all activities will necessarily be appropriate everywhere.</p>","PeriodicalId":17036,"journal":{"name":"Journal of Scleroderma and Related Disorders","volume":" ","pages":"23971983251324803"},"PeriodicalIF":1.4,"publicationDate":"2025-03-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11926815/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143692439","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}
引用次数: 0
International expert agreement on World Scleroderma Foundation/Heart Failure Association consensus-based definition and guidance on screening, diagnosis, and follow-up assessment of systemic sclerosis-associated primary heart involvement.
IF 1.4
Journal of Scleroderma and Related Disorders Pub Date : 2025-03-19 DOI: 10.1177/23971983251321691
Vitalii Dubas, Maya H Buch, Petar Seferovic, Marco Matucci-Cerinic, Cosimo Bruni
{"title":"International expert agreement on World Scleroderma Foundation/Heart Failure Association consensus-based definition and guidance on screening, diagnosis, and follow-up assessment of systemic sclerosis-associated primary heart involvement.","authors":"Vitalii Dubas, Maya H Buch, Petar Seferovic, Marco Matucci-Cerinic, Cosimo Bruni","doi":"10.1177/23971983251321691","DOIUrl":"10.1177/23971983251321691","url":null,"abstract":"<p><strong>Objective: </strong>The aim of this study was to evaluate the level of agreement among international experts on the World Scleroderma Foundation/Heart Failure Association consensus-based definition of systemic sclerosis-associated primary heart involvement (SSc-pHI) and on the guidance for its screening, diagnosis, and follow-up assessment, including feasibility and applicability assessments.</p><p><strong>Methods: </strong>An online survey was conducted to assess the level of agreement and feasibility/applicability using a 10-point scale (0 = <i>not at all</i>, 10 = <i>completely agree or completely feasible/applicable</i>). The weblink to the survey was shared with 1199 participants worldwide. A high level of agreement was defined by an average score over 7/10.</p><p><strong>Results: </strong>In total, 161 external experts completed the survey. Most of them were rheumatologists (80.7%), working in Europe (81.4%), and had > 10 years of clinical experience in managing SSc patients (59%). Overall agreement among external experts was high (mean 8.27, SD 1.86). The highest scores regarded items emphasizing the involvement of a multidisciplinary team, personalization of patient management, and initial evaluation techniques. A lower level of agreement was obtained in questions related to cardiac magnetic resonance imaging and endomyocardial biopsy. No factors associated with low level of agreement and feasibility/applicability were identified.</p><p><strong>Conclusion: </strong>The consensus-based definition and guidance on screening, diagnosis, and follow-up assessment of SSc-pHI provides a basis for application by the wider community. A lower level of agreement on the use of advanced or more invasive diagnostic techniques likely reflects regional differences in access and the need for more evidence on its use. This emphasizes the importance of involving a multidisciplinary team.</p>","PeriodicalId":17036,"journal":{"name":"Journal of Scleroderma and Related Disorders","volume":" ","pages":"23971983251321691"},"PeriodicalIF":1.4,"publicationDate":"2025-03-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11924048/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143692419","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}
引用次数: 0
Malignancy-associated risk factors in patients with systemic sclerosis.
IF 1.4
Journal of Scleroderma and Related Disorders Pub Date : 2025-03-19 DOI: 10.1177/23971983251322841
Serdar Baysal, Didem Şahin, Serdar Sezer, Müçteba E Yayla, Emine Uslu, Aşkın Ateş, Tahsin M Turgay, Gülay Kinikli
{"title":"Malignancy-associated risk factors in patients with systemic sclerosis.","authors":"Serdar Baysal, Didem Şahin, Serdar Sezer, Müçteba E Yayla, Emine Uslu, Aşkın Ateş, Tahsin M Turgay, Gülay Kinikli","doi":"10.1177/23971983251322841","DOIUrl":"10.1177/23971983251322841","url":null,"abstract":"<p><strong>Background and aim: </strong>Systemic sclerosis was found to be associated with an increase in cancer incidence. The target of this study was to investigate the most common malignancies and to identify factors that increase the cancer risk in systemic sclerosis patients.</p><p><strong>Materials and methods: </strong>In this single-centre retrospective study, we screened 252 patients attending our outpatient clinic between January 2005 and December 2021.</p><p><strong>Results: </strong>A total of 252 systemic sclerosis patients were included in the study. Data for the patients were obtained by evaluating their medical records retrospectively. A total of 252 patients with systemic sclerosis were analysed. Nineteen patients were diagnosed with malignancies. Lung cancer was the most common malignancy among patients. Malignancies were not correlated with sex, follow-up period, type of systemic sclerosis, organ involvement, smoking history, serological tests, comorbidities, dose and duration of disease-modifying antirheumatic drugs (DMARDs), but advanced age at systemic sclerosis diagnosis increased the risk of malignancy (p = 0.017) in systemic sclerosis patients.</p><p><strong>Conclusion: </strong>In the current study, advanced age at systemic sclerosis diagnosis was determined to be an extra risk factor for the initiation of cancer in systemic sclerosis patients. Particularly in this patient group, additional screenings might be helpful for early diagnosis. Treatments such as methotrexate, cyclophosphamide, azathioprine, and mycophenolate mofetil can be used without additional cancer risk.</p>","PeriodicalId":17036,"journal":{"name":"Journal of Scleroderma and Related Disorders","volume":" ","pages":"23971983251322841"},"PeriodicalIF":1.4,"publicationDate":"2025-03-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11924055/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143692346","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}
引用次数: 0
Interstitial lung disease in anti-U1RNP systemic sclerosis patients: A European Scleroderma Trials and Research analysis.
IF 1.4
Journal of Scleroderma and Related Disorders Pub Date : 2025-03-19 DOI: 10.1177/23971983251324827
Gonçalo Boleto, Corrado Campochiaro, Oliver Distler, Andra Balanescu, David Launay, Christina Bergmann, Paolo Airò, Fahrettin Oksel, Ana Maria Gheorghiu, Branimir Anic, Luc Mouthon, Sule Yavuz, Cristina-Mihaela Tanaseanu, Marco Matucci-Cerinic, Yannick Allanore
{"title":"Interstitial lung disease in anti-U1RNP systemic sclerosis patients: A European Scleroderma Trials and Research analysis.","authors":"Gonçalo Boleto, Corrado Campochiaro, Oliver Distler, Andra Balanescu, David Launay, Christina Bergmann, Paolo Airò, Fahrettin Oksel, Ana Maria Gheorghiu, Branimir Anic, Luc Mouthon, Sule Yavuz, Cristina-Mihaela Tanaseanu, Marco Matucci-Cerinic, Yannick Allanore","doi":"10.1177/23971983251324827","DOIUrl":"10.1177/23971983251324827","url":null,"abstract":"<p><strong>Background: </strong>Interstitial lung disease is the leading cause of morbidity and mortality in systemic sclerosis, but it is characterized by significant heterogeneity in patient outcomes. So far, little is known about the influence of anti-U1RNP antibodies on lung outcomes in systemic sclerosis-associated interstitial lung disease patients.</p><p><strong>Methods: </strong>European Scleroderma Trials and Research group systemic sclerosis patients with radiological-confirmed interstitial lung disease, available %predicted forced vital capacity, and autoantibody status were included. Baseline demographic and disease features were compared between anti-U1RNP positive and anti-U1RNP negative patients. Moreover, longitudinal analyses were done measuring relative change in %predicted forced vital capacity over 12 ± 6, 24 ± 6, and 36 ± 6 months, and changes were classified into stable (⩽ 4%), mild (5%-9%), and major progression (⩾ 10%). Predictors associated with death of any cause or major interstitial lung disease progression were evaluated in systemic sclerosis-associated interstitial lung disease patients with or without anti-U1RNP antibodies. Logistic regression analyses and Cox proportional hazards models adjusted for age and FVC were applied.</p><p><strong>Results: </strong>A total of 6043 systemic sclerosis-associated interstitial lung disease patients were included for the analysis, among which 327 (5.4%) were positive for anti-U1RNP antibodies. Mean age was 56.8 ± 13.2 years and 4971 (82.3%) were women. Anti-U1RNP + systemic sclerosis-associated interstitial lung disease patients had more frequently limited cutaneous systemic sclerosis (63.5.5% vs 53.3%, p < 0.001), higher frequency of joint synovitis (18.1% vs 13.9%, p = 0.039), and myositis (24.0% vs 19.5%, p = 0.048). Anti-U1RNP + patients had a baseline lower mean forced vital capacity (82.0% vs 86.0%, p < 0.001) and lower mean %predicted diffusing capacity for carbon monoxide (57.0% vs 60.5%, p = 0.003). Periods of mild or major FVC decline and mortality rates were not statistically different between the groups.</p><p><strong>Conclusion: </strong>Systemic sclerosis-associated interstitial lung disease patients positive for anti-U1RNP antibodies have more impaired baseline lung function but similar trajectories of forced vital capacity changes and mortality during the first 3 years of follow-up.</p>","PeriodicalId":17036,"journal":{"name":"Journal of Scleroderma and Related Disorders","volume":" ","pages":"23971983251324827"},"PeriodicalIF":1.4,"publicationDate":"2025-03-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11924063/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143692430","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}
引用次数: 0
Quality of life in scleroderma-related interstitial lung disease and its association with respiratory clinical parameters.
IF 1.4
Journal of Scleroderma and Related Disorders Pub Date : 2025-02-24 DOI: 10.1177/23971983251318827
Thayalan Ponniah, Chee Kuan Wong, Choung Min Ng, Jasmin Raja
{"title":"Quality of life in scleroderma-related interstitial lung disease and its association with respiratory clinical parameters.","authors":"Thayalan Ponniah, Chee Kuan Wong, Choung Min Ng, Jasmin Raja","doi":"10.1177/23971983251318827","DOIUrl":"10.1177/23971983251318827","url":null,"abstract":"<p><strong>Objectives: </strong>Interstitial lung disease in systemic sclerosis has a significant impact in the quality of life. This prospective study was designed to determine the correlation between quality of life in systemic sclerosis-interstitial lung disease patients using patient-reported outcome measures and the lung function test parameters, with assessment done at baseline and 1 year.</p><p><strong>Methods: </strong>In total, 63 consecutive systemic sclerosis-interstitial lung disease patients underwent lung function test, 6-minute walk distance, and quality of life-validated questionnaires (King's Brief Interstitial Lung Disease and Functional Assessment of Chronic Illness Therapy dyspnea). King's Brief Interstitial Lung Disease covers three components, namely chest, breathlessness, and psychological symptoms. The Functional Assessment of Chronic Illness Therapy dyspnea score has Part I (breathlessness symptoms) and Part II (physical limitation due to breathlessness). All assessments except for 6-minute walk distance were done both at baseline and at 12 months.</p><p><strong>Results: </strong>Both forced vital capacity predicted percentage at baseline and 12 months had significant negative correlation with the Functional Assessment of Chronic Illness Therapy dyspnea score Part I (<i>p</i> values: 0.038 and <0.001, respectively). Both forced vital capacity and diffuse capacity for carbon monoxide predicted percentage at 12 months were also negatively correlated with Functional Assessment of Chronic Illness Therapy dyspnea score Part II (<i>p</i> values: 0.001 and 0.010, respectively). There was no significant correlation between King's Brief Interstitial Lung Disease score and the lung function parameters, at both baseline and at 1-year interval. Positive significant correlation was observed between forced vital capacity predicted percentage at baseline and 6-minute walk distance (<i>p</i> = 0.001).</p><p><strong>Conclusion: </strong>Systemic sclerosis-interstitial lung disease affects quality of life, which is best assessed using the Functional Assessment of Chronic Illness Therapy dyspnea score as it correlates significantly with the lung function test parameters.</p>","PeriodicalId":17036,"journal":{"name":"Journal of Scleroderma and Related Disorders","volume":" ","pages":"23971983251318827"},"PeriodicalIF":1.4,"publicationDate":"2025-02-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11851579/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143516073","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}
引用次数: 0
The impact of systemic sclerosis on women's health evaluated with an ad hoc-developed patient-reported questionnaire.
IF 1.4
Journal of Scleroderma and Related Disorders Pub Date : 2025-02-24 DOI: 10.1177/23971983251318148
Maria-Grazia Lazzaroni, Liala Moschetti, Eleonora Pedretti, Laura Andreoli, Francesca Ramazzotto, Sonia Zatti, Ilaria Galetti, Paolo Airò, Angela Tincani, Franco Franceschini
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