Pub Date : 2024-06-03DOI: 10.1177/23971983241255550
E. Hoekstra, S. Liem, Saad Ahmed, Nivine Levarht, Cynthia M. Fehres, A. Giucă, N. Ajmone Marsan, Tom WJ Huizinga, J. D. de Vries-Bouwstra
Troponin I has been suggested as a more specific diagnostic biomarker for myocardial involvement in systemic sclerosis than the frequently used troponin T. The aim of this study is to evaluate the additive value of troponin I to detect myocardial involvement in systemic sclerosis. To this end, we evaluated the association between troponin I levels and myocardial involvement in systemic sclerosis patients. A cross-sectional observational study was performed, including 20 healthy controls and four groups of each 20 systemic sclerosis patients from the Leiden Combined Care in Systemic Sclerosis cohort: (1) patients with myocardial involvement, (2) patients with myositis, (3) patients with elevated troponin T and creatine kinase levels but without organ involvement, and (4) patients without any signs of organ involvement. Troponin I levels were measured using enzyme-linked immunosorbent assay. Troponin I levels were compared between the different groups using the Mann–Whitney U and Kruskal–Wallis tests. The mean age of the 80 included patients was 56 years; 61% of the study population was female. Troponin I levels were not significantly different between patients with and without myocardial involvement (2.7 (0.5–15.3) vs 1.2 (0.1–6.6) ng/L; p = 0.117). Systemic sclerosis patients were more often positive for troponin I than healthy controls (70.0% vs 30.0%; p = 0.001). Elevated troponin I was not of additional value to diagnose myocardial involvement in systemic sclerosis patients.
与常用的肌钙蛋白 T 相比，肌钙蛋白 I 被认为是诊断系统性硬化症心肌受累的更具特异性的生物标志物。这项研究的目的是评估肌钙蛋白 I 在检测系统性硬化症心肌受累方面的附加价值。为此，我们评估了肌钙蛋白 I 水平与系统性硬化症患者心肌受累之间的关联。我们进行了一项横断面观察研究，其中包括 20 名健康对照者和莱顿系统性硬化症联合护理队列中的四组系统性硬化症患者，每组 20 人：(1) 心肌受累患者；(2) 肌炎患者；(3) 肌钙蛋白 T 和肌酸激酶水平升高但无器官受累的患者；(4) 无任何器官受累迹象的患者。肌钙蛋白 I 水平采用酶联免疫吸附测定法进行测量。采用 Mann-Whitney U 检验和 Kruskal-Wallis 检验比较不同组别之间的肌钙蛋白 I 水平。80 名患者的平均年龄为 56 岁，61% 为女性。有心肌受累和无心肌受累患者的肌钙蛋白 I 水平无明显差异（2.7 (0.5-15.3) vs 1.2 (0.1-6.6) ng/L；p = 0.117）。系统性硬化症患者的肌钙蛋白 I 阳性率高于健康对照组（70.0% vs 30.0%；p = 0.001）。肌钙蛋白 I 升高对诊断系统硬化症患者心肌受累没有额外价值。
{"title":"Troponin I levels in systemic sclerosis patients with myocardial involvement","authors":"E. Hoekstra, S. Liem, Saad Ahmed, Nivine Levarht, Cynthia M. Fehres, A. Giucă, N. Ajmone Marsan, Tom WJ Huizinga, J. D. de Vries-Bouwstra","doi":"10.1177/23971983241255550","DOIUrl":"https://doi.org/10.1177/23971983241255550","url":null,"abstract":"Troponin I has been suggested as a more specific diagnostic biomarker for myocardial involvement in systemic sclerosis than the frequently used troponin T. The aim of this study is to evaluate the additive value of troponin I to detect myocardial involvement in systemic sclerosis. To this end, we evaluated the association between troponin I levels and myocardial involvement in systemic sclerosis patients. A cross-sectional observational study was performed, including 20 healthy controls and four groups of each 20 systemic sclerosis patients from the Leiden Combined Care in Systemic Sclerosis cohort: (1) patients with myocardial involvement, (2) patients with myositis, (3) patients with elevated troponin T and creatine kinase levels but without organ involvement, and (4) patients without any signs of organ involvement. Troponin I levels were measured using enzyme-linked immunosorbent assay. Troponin I levels were compared between the different groups using the Mann–Whitney U and Kruskal–Wallis tests. The mean age of the 80 included patients was 56 years; 61% of the study population was female. Troponin I levels were not significantly different between patients with and without myocardial involvement (2.7 (0.5–15.3) vs 1.2 (0.1–6.6) ng/L; p = 0.117). Systemic sclerosis patients were more often positive for troponin I than healthy controls (70.0% vs 30.0%; p = 0.001). Elevated troponin I was not of additional value to diagnose myocardial involvement in systemic sclerosis patients.","PeriodicalId":506053,"journal":{"name":"Journal of Scleroderma and Related Disorders","volume":"35 3","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-06-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141270570","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}
Pub Date : 2024-03-26DOI: 10.1177/23971983241238921
Benoit Gachet, M. Jouvray, V. Koether, A. Collet, S. Morell-Dubois, S. Sanges, V. Sobanski, E. Hachulla, David Launay
Anemia is considered a risk factor of severity in systemic sclerosis. Yet, limited data are available on the frequency and causes of anemia in systemic sclerosis. The objectives of our study were to determine the frequency and causes of anemia in systemic sclerosis, to analyze the clinical and biological characteristics of patients with anemia, and to assess the association between anemia and systemic sclerosis prognosis. We conducted a prospective single-center study from January 2017 to May 2022. Patients underwent a hemoglobin assay and a standardized etiological workup to determine the causes of anemia at the initial visit. Clinical and biological parameters were compared between patients with anemia and those with normal hemoglobin levels. We followed up patients until May 2023 and compared their survival. A total of 502 systemic sclerosis patients, including 107 diffuse cutaneous systemic sclerosis, were included. At enrollment, 100 patients had anemia. The primary cause of anemia was iron deficiency (40%). Among the 32 patients with iron deficiency-associated anemia who underwent gastrointestinal exploration, 56% had digestive bleeding, with upper gastrointestinal tract involvement being the main cause (90%). Patients with anemia at enrollment had higher systemic sclerosis severity scores and more gastrointestinal symptoms compared to patients without anemia (p < 0.05). They exhibited higher systolic pulmonary artery pressure, lower anemia-corrected diffusing capacity for carbon monoxide, and lower forced vital capacity (p < 0.05). During follow-up, 65 patients (14.8%) died. After adjusting for age, systemic sclerosis subtypes, forced vital capacity, and pulmonary arterial hypertension, anemia at inclusion was associated with a higher mortality rate (hazard ratio: 1.94 (95% confidence interval: 1.39–2.48), p = 0.02). Our study shows a high frequency of anemia among patients with systemic sclerosis. Most anemias are due to iron deficiency, and gastrointestinal exploration can identify bleeding in the majority of the cases. In addition, our study confirms that systemic sclerosis patients with anemia have a more severe disease and a higher mortality rate.
{"title":"Prevalence, causes, and clinical associations of anemia in patients with systemic sclerosis: A cohort study","authors":"Benoit Gachet, M. Jouvray, V. Koether, A. Collet, S. Morell-Dubois, S. Sanges, V. Sobanski, E. Hachulla, David Launay","doi":"10.1177/23971983241238921","DOIUrl":"https://doi.org/10.1177/23971983241238921","url":null,"abstract":"Anemia is considered a risk factor of severity in systemic sclerosis. Yet, limited data are available on the frequency and causes of anemia in systemic sclerosis. The objectives of our study were to determine the frequency and causes of anemia in systemic sclerosis, to analyze the clinical and biological characteristics of patients with anemia, and to assess the association between anemia and systemic sclerosis prognosis. We conducted a prospective single-center study from January 2017 to May 2022. Patients underwent a hemoglobin assay and a standardized etiological workup to determine the causes of anemia at the initial visit. Clinical and biological parameters were compared between patients with anemia and those with normal hemoglobin levels. We followed up patients until May 2023 and compared their survival. A total of 502 systemic sclerosis patients, including 107 diffuse cutaneous systemic sclerosis, were included. At enrollment, 100 patients had anemia. The primary cause of anemia was iron deficiency (40%). Among the 32 patients with iron deficiency-associated anemia who underwent gastrointestinal exploration, 56% had digestive bleeding, with upper gastrointestinal tract involvement being the main cause (90%). Patients with anemia at enrollment had higher systemic sclerosis severity scores and more gastrointestinal symptoms compared to patients without anemia (p < 0.05). They exhibited higher systolic pulmonary artery pressure, lower anemia-corrected diffusing capacity for carbon monoxide, and lower forced vital capacity (p < 0.05). During follow-up, 65 patients (14.8%) died. After adjusting for age, systemic sclerosis subtypes, forced vital capacity, and pulmonary arterial hypertension, anemia at inclusion was associated with a higher mortality rate (hazard ratio: 1.94 (95% confidence interval: 1.39–2.48), p = 0.02). Our study shows a high frequency of anemia among patients with systemic sclerosis. Most anemias are due to iron deficiency, and gastrointestinal exploration can identify bleeding in the majority of the cases. In addition, our study confirms that systemic sclerosis patients with anemia have a more severe disease and a higher mortality rate.","PeriodicalId":506053,"journal":{"name":"Journal of Scleroderma and Related Disorders","volume":"64 11","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-03-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140378313","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}
Pub Date : 2024-03-13DOI: 10.1177/23971983241235708
María Paula Álvarez-Hernández, Y. Allanore, Ivo Andrade, Maya H Buch, Gerry Coghlan, F. del Galdo, C. Denton, Dinesh Khanna, D. Kiely, John D Pauling, Sheila Ramjug, Michael Hughes
The objectives were to explore rheumatologists’ current clinical screening practices of pulmonary arterial hypertension in patients with systemic sclerosis in the United Kingdom and to identify barriers to screening and consider potential solutions. A survey of 31 questions was developed and included six sections: clinician demographics, the importance of screening, screening practices, barriers to screening, treatment and patient education. The survey was disseminated among rheumatologists working in the United Kingdom. Forty-four rheumatologists working in the United Kingdom participated in the study, and the majority completed all the questions. Around one-third (37.0%) worked in specialised systemic sclerosis units (university or general hospitals (54.5% and 45.4%, respectively)). The majority recognised that systemic sclerosis-pulmonary arterial hypertension is a major cause of morbidity and mortality. Over half (60.0%) reported using the DETECT algorithm to screen for systemic sclerosis-pulmonary arterial hypertension, although other algorithms were also sometimes used. All of the respondents utilised transthoracic echocardiogram, and almost all (95.0%) performed pulmonary function tests for screening purposes. Various challenges and barriers were identified relating to systemic sclerosis-pulmonary arterial hypertension screening, with the difficulty in interpreting results from other hospitals and extended wait times for diagnostic tests being the most reported (76.0% and 74.0%, respectively). Most respondents agreed that access to key investigations (87.0%), ongoing clinician education (82.0%), multidisciplinary meetings (79.5%) and a better understanding of proposed screening algorithms (79.5%) could be potential solutions. Screening patients with systemic sclerosis for pulmonary arterial hypertension is crucial to improve survival, but variable practices exist among UK rheumatologists. Solutions include educating healthcare professionals on guidelines, sharing information between centres and integrating care services.
{"title":"Attitudes and barriers to pulmonary arterial hypertension screening in systemic sclerosis patients: A survey of UK-based rheumatologists","authors":"María Paula Álvarez-Hernández, Y. Allanore, Ivo Andrade, Maya H Buch, Gerry Coghlan, F. del Galdo, C. Denton, Dinesh Khanna, D. Kiely, John D Pauling, Sheila Ramjug, Michael Hughes","doi":"10.1177/23971983241235708","DOIUrl":"https://doi.org/10.1177/23971983241235708","url":null,"abstract":"The objectives were to explore rheumatologists’ current clinical screening practices of pulmonary arterial hypertension in patients with systemic sclerosis in the United Kingdom and to identify barriers to screening and consider potential solutions. A survey of 31 questions was developed and included six sections: clinician demographics, the importance of screening, screening practices, barriers to screening, treatment and patient education. The survey was disseminated among rheumatologists working in the United Kingdom. Forty-four rheumatologists working in the United Kingdom participated in the study, and the majority completed all the questions. Around one-third (37.0%) worked in specialised systemic sclerosis units (university or general hospitals (54.5% and 45.4%, respectively)). The majority recognised that systemic sclerosis-pulmonary arterial hypertension is a major cause of morbidity and mortality. Over half (60.0%) reported using the DETECT algorithm to screen for systemic sclerosis-pulmonary arterial hypertension, although other algorithms were also sometimes used. All of the respondents utilised transthoracic echocardiogram, and almost all (95.0%) performed pulmonary function tests for screening purposes. Various challenges and barriers were identified relating to systemic sclerosis-pulmonary arterial hypertension screening, with the difficulty in interpreting results from other hospitals and extended wait times for diagnostic tests being the most reported (76.0% and 74.0%, respectively). Most respondents agreed that access to key investigations (87.0%), ongoing clinician education (82.0%), multidisciplinary meetings (79.5%) and a better understanding of proposed screening algorithms (79.5%) could be potential solutions. Screening patients with systemic sclerosis for pulmonary arterial hypertension is crucial to improve survival, but variable practices exist among UK rheumatologists. Solutions include educating healthcare professionals on guidelines, sharing information between centres and integrating care services.","PeriodicalId":506053,"journal":{"name":"Journal of Scleroderma and Related Disorders","volume":"2002 15","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-03-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140246409","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}
Pub Date : 2024-03-05DOI: 10.1177/23971983241232853
Y. T. Chen, A. Hassett, Dinesh Khanna, Susan L Murphy
Systemic sclerosis (SSc) negatively affects quality of life, yet the factors influencing life satisfaction, a key aspect of well-being, remain unclear in this population. Social isolation is common in SSc, potentially linked to decreased life satisfaction, but the underlying mechanisms are unexplored. Resilience, a modifiable psychological resource, may act as a mediator in this relationship among people with SSc. This study aimed to examine the relationship between perceived social isolation and life satisfaction and to investigate whether resilience mediates this relationship. The Patient-Reported Outcomes Measurement Information System Social Isolation Short Form, the Connor-Davidson Resilience Scale, and the Satisfaction with Life Scale were used to assess perceived social isolation, resilience, and life satisfaction. Linear regressions were conducted using the PROCESS macro for SPSS. Among 163 individuals with SSc who provided complete data at baseline (mean age = 54.7 ± 11.9 years), 47% had diffuse cutaneous SSc, and 57% had an early disease duration. Perceived social isolation was negatively associated with life satisfaction. Resilience partially mediated the association between perceived social isolation and life satisfaction in people with SSc. Findings revealed a significant association between perceived social isolation and life satisfaction and the mediating role of resilience in this association among people with SSc. Results suggest resilience may act as a protective mediator, counteracting the negative influence of perceived social isolation on life satisfaction. Findings support the promotion of social connection and resilience to enhance life satisfaction in people with SSc. NCT04908943
{"title":"Resilience partially mediates the association between perceived social isolation and life satisfaction in people with systemic sclerosis","authors":"Y. T. Chen, A. Hassett, Dinesh Khanna, Susan L Murphy","doi":"10.1177/23971983241232853","DOIUrl":"https://doi.org/10.1177/23971983241232853","url":null,"abstract":"Systemic sclerosis (SSc) negatively affects quality of life, yet the factors influencing life satisfaction, a key aspect of well-being, remain unclear in this population. Social isolation is common in SSc, potentially linked to decreased life satisfaction, but the underlying mechanisms are unexplored. Resilience, a modifiable psychological resource, may act as a mediator in this relationship among people with SSc. This study aimed to examine the relationship between perceived social isolation and life satisfaction and to investigate whether resilience mediates this relationship. The Patient-Reported Outcomes Measurement Information System Social Isolation Short Form, the Connor-Davidson Resilience Scale, and the Satisfaction with Life Scale were used to assess perceived social isolation, resilience, and life satisfaction. Linear regressions were conducted using the PROCESS macro for SPSS. Among 163 individuals with SSc who provided complete data at baseline (mean age = 54.7 ± 11.9 years), 47% had diffuse cutaneous SSc, and 57% had an early disease duration. Perceived social isolation was negatively associated with life satisfaction. Resilience partially mediated the association between perceived social isolation and life satisfaction in people with SSc. Findings revealed a significant association between perceived social isolation and life satisfaction and the mediating role of resilience in this association among people with SSc. Results suggest resilience may act as a protective mediator, counteracting the negative influence of perceived social isolation on life satisfaction. Findings support the promotion of social connection and resilience to enhance life satisfaction in people with SSc. NCT04908943","PeriodicalId":506053,"journal":{"name":"Journal of Scleroderma and Related Disorders","volume":"24 2","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-03-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140264750","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}
Pub Date : 2024-03-01DOI: 10.1177/23971983241229600
{"title":"WSO Author Index","authors":"","doi":"10.1177/23971983241229600","DOIUrl":"https://doi.org/10.1177/23971983241229600","url":null,"abstract":"","PeriodicalId":506053,"journal":{"name":"Journal of Scleroderma and Related Disorders","volume":"26 3","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140280096","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}
Pub Date : 2024-03-01DOI: 10.1177/23971983231224122
{"title":"Oral abstracts","authors":"","doi":"10.1177/23971983231224122","DOIUrl":"https://doi.org/10.1177/23971983231224122","url":null,"abstract":"","PeriodicalId":506053,"journal":{"name":"Journal of Scleroderma and Related Disorders","volume":"55 4","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140272773","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}
Pub Date : 2024-02-28DOI: 10.1177/23971983241231082
N. Benmostefa, Rachid Malek, Marie Robert, B. Chaigne, Samy Slimani, Samir Rouabhia, Daoud Roula, Mallem Djamel, L. Mouthon
The study aimed to assess the construct validity of the Arab Hand Function Index and the Arabic Health Assessment Questionnaire in Algerian patients with systemic sclerosis. Consecutive Algerian patients who fulfilled the American College of Rheumatology/European League Against Rheumatism criteria for systemic sclerosis were included. In addition to disease characteristics, global disability and hand disability were assessed using the Arabic Health Assessment Questionnaire and the Arab Hand Function Index, respectively. Construct validity was assessed by convergent and divergent validity (Spearman’s rank correlation coefficient) and factor analysis. The scale reliability was assessed using the Cronbach’s alpha. We evaluated 100 systemic sclerosis patients (83 females) of mean ± standard deviation age 46.7 ± 12.3 years, including 59 limited cutaneous systemic sclerosis and 41 diffuse cutaneous systemic sclerosis. Raynaud’s phenomenon was detected in 99 patients and digital ulcers in 25. Gastrointestinal tract involvement and interstitial lung disease were detected in 86/100 (86%) and 46/72 (63.9%) patients, respectively. Anti-topoisomerase I and anti-centromere antibodies were detected in 33/76 (43.4%) and 23/76 (30.3%) patients, respectively. The Arab Hand Function Index had a good construct validity with a total score explaining 61% of the variance of the Arabic Health Assessment Questionnaire which also had a good construct validity. Factor analysis of the Arab Hand Function Index and the Arabic Health Assessment Questionnaire items extracted two factors explaining 64% of the variance for the Arab Hand Function Index and one factor explaining 55% of the variance for the Arabic Health Assessment Questionnaire. The Arab Hand Function Index and the Arabic Health Assessment Questionnaire were reliable questionnaires with a Cronbach’s alpha >0.8. In Algerian patients with systemic sclerosis, Arab Hand Function Index and Arabic Health Assessment Questionnaire have a good construct validity and reliability.
{"title":"Assessing hand and global disability in a cohort of Algerian patients with systemic sclerosis: Construct validities of the Arab Hand Function Index and the Arabic Health Assessment Questionnaire","authors":"N. Benmostefa, Rachid Malek, Marie Robert, B. Chaigne, Samy Slimani, Samir Rouabhia, Daoud Roula, Mallem Djamel, L. Mouthon","doi":"10.1177/23971983241231082","DOIUrl":"https://doi.org/10.1177/23971983241231082","url":null,"abstract":"The study aimed to assess the construct validity of the Arab Hand Function Index and the Arabic Health Assessment Questionnaire in Algerian patients with systemic sclerosis. Consecutive Algerian patients who fulfilled the American College of Rheumatology/European League Against Rheumatism criteria for systemic sclerosis were included. In addition to disease characteristics, global disability and hand disability were assessed using the Arabic Health Assessment Questionnaire and the Arab Hand Function Index, respectively. Construct validity was assessed by convergent and divergent validity (Spearman’s rank correlation coefficient) and factor analysis. The scale reliability was assessed using the Cronbach’s alpha. We evaluated 100 systemic sclerosis patients (83 females) of mean ± standard deviation age 46.7 ± 12.3 years, including 59 limited cutaneous systemic sclerosis and 41 diffuse cutaneous systemic sclerosis. Raynaud’s phenomenon was detected in 99 patients and digital ulcers in 25. Gastrointestinal tract involvement and interstitial lung disease were detected in 86/100 (86%) and 46/72 (63.9%) patients, respectively. Anti-topoisomerase I and anti-centromere antibodies were detected in 33/76 (43.4%) and 23/76 (30.3%) patients, respectively. The Arab Hand Function Index had a good construct validity with a total score explaining 61% of the variance of the Arabic Health Assessment Questionnaire which also had a good construct validity. Factor analysis of the Arab Hand Function Index and the Arabic Health Assessment Questionnaire items extracted two factors explaining 64% of the variance for the Arab Hand Function Index and one factor explaining 55% of the variance for the Arabic Health Assessment Questionnaire. The Arab Hand Function Index and the Arabic Health Assessment Questionnaire were reliable questionnaires with a Cronbach’s alpha >0.8. In Algerian patients with systemic sclerosis, Arab Hand Function Index and Arabic Health Assessment Questionnaire have a good construct validity and reliability.","PeriodicalId":506053,"journal":{"name":"Journal of Scleroderma and Related Disorders","volume":"107 14","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-02-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140422548","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}
Pub Date : 2024-02-27DOI: 10.1177/23971983241231620
M. Farhat, Pierre Guerreschi, S. Morell-Dubois, Valérie Deken, Julien Labreuche, S. Sanges, V. Sobanski, E. Hachulla, Olivier Cottencin, David Launay
Systemic sclerosis is a fibrotic disease. Body image assessments could be key in optimizing care; however, data are scarce. The main objective was to assess the perception of aesthetic impairment using a visual aesthetic evaluation scale in patients with systemic sclerosis compared with healthy subjects. The secondary objectives were to assess associations between the perception of aesthetic impairment and scores on standardized questionnaires for aesthetic impairment as well as clinical, psychological/quality of life, and functional parameters of patients with systemic sclerosis. This study evaluated and compared the perception of aesthetic impairment in two populations: patients with systemic sclerosis from a referral center at Lille Hospital, France, and healthy controls. This study included 88 patients (69 (78.4%) women) with a median age of 52 years and 88 controls (49 (55.7%) women) with a median age of 45 years. The perception of aesthetic impairment assessed using the aesthetic evaluation scale was poorer in systemic sclerosis patients than in controls (3.7 ± 0.3 vs 2.8 ± 0.3, p = 0.028) and was statistically correlated with assessments using the adapted satisfaction with appearance, a specific aesthetic impact assessment questionnaire for patients with systemic sclerosis. Patients with anxiety or depressive symptoms had significantly higher aesthetic evaluation scale scores. Systemic sclerosis patients with facial involvement and pitting scars had a worse perception of aesthetic impairment. Compared with healthy controls, systemic sclerosis patients had a worse perception of aesthetic impairment, especially systemic sclerosis patients with anxiety or depression and those with facial and hand involvement. The aesthetic evaluation scale appears to be an easy-to-use tool to evaluate body image. Correlations of the aesthetic evaluation scale score with psychological and quality of life parameters reflect the importance of these parameters for body image evaluation and its complex assessment. Clinical Trial NCT03271320 (Registered 9 January 2017, https://www.clinicaltrials.gov/ct2/show/NCT03271320?term=NCT03271320&cntry=FR&draw=2&rank=1 ).
{"title":"Perception of aesthetic impairment in patients with systemic sclerosis determined using a semi-quantitative scale and its association with disease characteristics","authors":"M. Farhat, Pierre Guerreschi, S. Morell-Dubois, Valérie Deken, Julien Labreuche, S. Sanges, V. Sobanski, E. Hachulla, Olivier Cottencin, David Launay","doi":"10.1177/23971983241231620","DOIUrl":"https://doi.org/10.1177/23971983241231620","url":null,"abstract":"Systemic sclerosis is a fibrotic disease. Body image assessments could be key in optimizing care; however, data are scarce. The main objective was to assess the perception of aesthetic impairment using a visual aesthetic evaluation scale in patients with systemic sclerosis compared with healthy subjects. The secondary objectives were to assess associations between the perception of aesthetic impairment and scores on standardized questionnaires for aesthetic impairment as well as clinical, psychological/quality of life, and functional parameters of patients with systemic sclerosis. This study evaluated and compared the perception of aesthetic impairment in two populations: patients with systemic sclerosis from a referral center at Lille Hospital, France, and healthy controls. This study included 88 patients (69 (78.4%) women) with a median age of 52 years and 88 controls (49 (55.7%) women) with a median age of 45 years. The perception of aesthetic impairment assessed using the aesthetic evaluation scale was poorer in systemic sclerosis patients than in controls (3.7 ± 0.3 vs 2.8 ± 0.3, p = 0.028) and was statistically correlated with assessments using the adapted satisfaction with appearance, a specific aesthetic impact assessment questionnaire for patients with systemic sclerosis. Patients with anxiety or depressive symptoms had significantly higher aesthetic evaluation scale scores. Systemic sclerosis patients with facial involvement and pitting scars had a worse perception of aesthetic impairment. Compared with healthy controls, systemic sclerosis patients had a worse perception of aesthetic impairment, especially systemic sclerosis patients with anxiety or depression and those with facial and hand involvement. The aesthetic evaluation scale appears to be an easy-to-use tool to evaluate body image. Correlations of the aesthetic evaluation scale score with psychological and quality of life parameters reflect the importance of these parameters for body image evaluation and its complex assessment. Clinical Trial NCT03271320 (Registered 9 January 2017, https://www.clinicaltrials.gov/ct2/show/NCT03271320?term=NCT03271320&cntry=FR&draw=2&rank=1 ).","PeriodicalId":506053,"journal":{"name":"Journal of Scleroderma and Related Disorders","volume":"5 11‐12","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-02-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140424763","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}
Pub Date : 2024-02-19DOI: 10.1177/23971983241227133
J. Raja, Shanthini Muthusamy, Choung Min Ng, S. Mahadeva
To study the prevalence of Helicobacter pylori in systemic sclerosis patients and its gastrointestinal manifestations in comparison with Helicobacter pylori-negative systemic sclerosis patients. Systemic sclerosis gastrointestinal outcome post Helicobacter pylori eradication was evaluated. In total, 70 systemic sclerosis patients and 70 age-, gender- and race-matched healthy controls had their urea breath test done. Gastrointestinal manifestations in systemic sclerosis were assessed using University of California at Los Angeles 2.0 and Leeds Dyspepsia Questionnaire questionnaires. Systemic sclerosis patients with confirmed Helicobacter pylori infection were given standard Helicobacter pylori eradication therapy. Urea breath test was repeated 6 weeks posteradication therapy with their gastrointestinal symptoms reassessed. The prevalence of Helicobacter pylori was low in both systemic sclerosis patients (10%) and healthy controls (2.9%). There was no significant difference in gastrointestinal symptoms between Helicobacter pylori-positive and Helicobacter pylori-negative systemic sclerosis patients. However, the Helicobacter pylori-positive patients reported higher median severity scores for the gastrointestinal symptoms of reflux (0.5 vs 0.35), abdominal distension (1.5 vs 0.75) and social functioning impairment score (0.5 vs 0.16) using the University of California at Los Angeles 2.0 score. The Helicobacter pylori-positive patients also indicated increased upper abdominal pain (3.0 vs 0.0), regurgitation (2.0 vs 0.0) and burping (3.0 vs 0.0), observed from the Leeds Dyspepsia Questionnaire scores. Gastrointestinal outcomes post- Helicobacter pylori eradication showed either an improvement or complete resolution of symptoms. Gastrointestinal symptoms in systemic sclerosis patients are unlikely to be caused by Helicobacter pylori in the recent years in our cohort of patients. No significant difference in gastrointestinal symptoms was seen between Helicobacter pylori-positive and Helicobacter pylori-negative systemic sclerosis patients. Helicobacter pylori can be readily tested by urea breath test to look for present infection.
{"title":"Helicobacter pylori in gastrointestinal manifestation of systemic sclerosis","authors":"J. Raja, Shanthini Muthusamy, Choung Min Ng, S. Mahadeva","doi":"10.1177/23971983241227133","DOIUrl":"https://doi.org/10.1177/23971983241227133","url":null,"abstract":"To study the prevalence of Helicobacter pylori in systemic sclerosis patients and its gastrointestinal manifestations in comparison with Helicobacter pylori-negative systemic sclerosis patients. Systemic sclerosis gastrointestinal outcome post Helicobacter pylori eradication was evaluated. In total, 70 systemic sclerosis patients and 70 age-, gender- and race-matched healthy controls had their urea breath test done. Gastrointestinal manifestations in systemic sclerosis were assessed using University of California at Los Angeles 2.0 and Leeds Dyspepsia Questionnaire questionnaires. Systemic sclerosis patients with confirmed Helicobacter pylori infection were given standard Helicobacter pylori eradication therapy. Urea breath test was repeated 6 weeks posteradication therapy with their gastrointestinal symptoms reassessed. The prevalence of Helicobacter pylori was low in both systemic sclerosis patients (10%) and healthy controls (2.9%). There was no significant difference in gastrointestinal symptoms between Helicobacter pylori-positive and Helicobacter pylori-negative systemic sclerosis patients. However, the Helicobacter pylori-positive patients reported higher median severity scores for the gastrointestinal symptoms of reflux (0.5 vs 0.35), abdominal distension (1.5 vs 0.75) and social functioning impairment score (0.5 vs 0.16) using the University of California at Los Angeles 2.0 score. The Helicobacter pylori-positive patients also indicated increased upper abdominal pain (3.0 vs 0.0), regurgitation (2.0 vs 0.0) and burping (3.0 vs 0.0), observed from the Leeds Dyspepsia Questionnaire scores. Gastrointestinal outcomes post- Helicobacter pylori eradication showed either an improvement or complete resolution of symptoms. Gastrointestinal symptoms in systemic sclerosis patients are unlikely to be caused by Helicobacter pylori in the recent years in our cohort of patients. No significant difference in gastrointestinal symptoms was seen between Helicobacter pylori-positive and Helicobacter pylori-negative systemic sclerosis patients. Helicobacter pylori can be readily tested by urea breath test to look for present infection.","PeriodicalId":506053,"journal":{"name":"Journal of Scleroderma and Related Disorders","volume":"76 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-02-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140449211","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}
Pub Date : 2024-01-11DOI: 10.1177/23971983231209809
V. Riccieri, G. Pellegrino, E. Cipolletta, D. Giuggioli, G. Bajocchi, S. Bellando-Randone, L. Dagna, G. Zanframundo, Rosario Foti, F. Cacciapaglia, G. Cuomo, A. Ariani, E. Rosato, G. Lepri, F. Girelli, E. Zanatta, S. Bosello, I. Cavazzana, F. Ingegnoli, M. De Santis, G. Murdaca, G. Abignano, N. Romeo, A. Della Rossa, Maurizio Caminiti, A. Iuliano, G. Ciano, L. Beretta, G. Bagnato, E. Lubrano, I. de Andres, A. Giollo, M. Saracco, C. Agnes, F. Lumetti, A. Spinella, Luca Magnani, C. Campochiaro, G. de Luca, V. Codullo, E. Visalli, C. Di Vico, A. Gigante, F. Saccon, Maria Grazia Lazzaroni, F. Franceschini, E. Generali, G. Mennillo, S. Barsotti, G. Pagano Mariano, Francesca Calabrese, F. Furini, Licia Vultaggio, S. Parisi, C. Peroni, Gerolamo Bianchi, Fabrizio Conti, Franco Cozzi, Salvatore D'Angelo, Andrea Doria, E. Fusaro, M. Govoni, S. Guiducci, F. Iannone, C. Salvarani, G. Sebastiani, C. Ferri, M. Matucci-Cerinic, R. De Angelis
Intravenous iloprost has been widely used for the treatment of systemic sclerosis peripheral vasculopathy. No agreement has been found on the regimen and the dosage of intravenous iloprost in different scleroderma subset conditions. This study aimed to evaluate the modalities of intravenous iloprost administration within a large cohort of systemic sclerosis patients from the SPRING Registry and to identify any associated clinical-demographic, instrumental or therapeutic data. Data of systemic sclerosis patients treated with intravenous iloprost for at least 1 year (case group) were retrospectively analyzed, including different timing and duration of intravenous iloprost session, and compared with those of untreated patients (control group). Out of 1895 analyzed patients, 937 (49%) received intravenous iloprost treatment, while 958 (51%) were assigned to the control group. Among cases, about 70% were treated every 4 weeks, 24% with an interval of more than 4 weeks, and only 6% of less than 4 weeks. Most patients receiving the treatment every 4 weeks, or less, underwent infusion cycle for 1 day only, while if it was scheduled with an interval of more than 4 weeks, a total number of 5 consecutive days of infusions was the preferred regimen. The comparison between the two groups revealed that patients treated with intravenous iloprost had a higher frequency of DUs (p < 0.001), pitting scars (p < 0.001), diffuse cutaneous involvement (p < 0.001), interstitial lung disease (p < 0.002), as well as higher rates of anti-topoisomerase I, “ late” scleroderma pattern at nailfold videocapillaroscopy. These findings were confirmed by multivariate analysis. Our data provide a picture on the Italian use of intravenous iloprost among systemic sclerosis patients and showed that it was usually employed in patients with a more aggressive spectrum of the disease. The disparity of intravenous iloprost treatment strategies in the different centers suggests the need of a rational therapeutical approach based on the clinical characteristics of different patients’ subsets.
{"title":"Practice pattern for the use of intravenous iloprost for the treatment of peripheral vasculopathy in systemic sclerosis: A case–control study from the Italian national multicenter “SPRING” (Systemic Sclerosis Progression InvestiGation) Registry","authors":"V. Riccieri, G. Pellegrino, E. Cipolletta, D. Giuggioli, G. Bajocchi, S. Bellando-Randone, L. Dagna, G. Zanframundo, Rosario Foti, F. Cacciapaglia, G. Cuomo, A. Ariani, E. Rosato, G. Lepri, F. Girelli, E. Zanatta, S. Bosello, I. Cavazzana, F. Ingegnoli, M. De Santis, G. Murdaca, G. Abignano, N. Romeo, A. Della Rossa, Maurizio Caminiti, A. Iuliano, G. Ciano, L. Beretta, G. Bagnato, E. Lubrano, I. de Andres, A. Giollo, M. Saracco, C. Agnes, F. Lumetti, A. Spinella, Luca Magnani, C. Campochiaro, G. de Luca, V. Codullo, E. Visalli, C. Di Vico, A. Gigante, F. Saccon, Maria Grazia Lazzaroni, F. Franceschini, E. Generali, G. Mennillo, S. Barsotti, G. Pagano Mariano, Francesca Calabrese, F. Furini, Licia Vultaggio, S. Parisi, C. Peroni, Gerolamo Bianchi, Fabrizio Conti, Franco Cozzi, Salvatore D'Angelo, Andrea Doria, E. Fusaro, M. Govoni, S. Guiducci, F. Iannone, C. Salvarani, G. Sebastiani, C. Ferri, M. Matucci-Cerinic, R. De Angelis","doi":"10.1177/23971983231209809","DOIUrl":"https://doi.org/10.1177/23971983231209809","url":null,"abstract":"Intravenous iloprost has been widely used for the treatment of systemic sclerosis peripheral vasculopathy. No agreement has been found on the regimen and the dosage of intravenous iloprost in different scleroderma subset conditions. This study aimed to evaluate the modalities of intravenous iloprost administration within a large cohort of systemic sclerosis patients from the SPRING Registry and to identify any associated clinical-demographic, instrumental or therapeutic data. Data of systemic sclerosis patients treated with intravenous iloprost for at least 1 year (case group) were retrospectively analyzed, including different timing and duration of intravenous iloprost session, and compared with those of untreated patients (control group). Out of 1895 analyzed patients, 937 (49%) received intravenous iloprost treatment, while 958 (51%) were assigned to the control group. Among cases, about 70% were treated every 4 weeks, 24% with an interval of more than 4 weeks, and only 6% of less than 4 weeks. Most patients receiving the treatment every 4 weeks, or less, underwent infusion cycle for 1 day only, while if it was scheduled with an interval of more than 4 weeks, a total number of 5 consecutive days of infusions was the preferred regimen. The comparison between the two groups revealed that patients treated with intravenous iloprost had a higher frequency of DUs (p < 0.001), pitting scars (p < 0.001), diffuse cutaneous involvement (p < 0.001), interstitial lung disease (p < 0.002), as well as higher rates of anti-topoisomerase I, “ late” scleroderma pattern at nailfold videocapillaroscopy. These findings were confirmed by multivariate analysis. Our data provide a picture on the Italian use of intravenous iloprost among systemic sclerosis patients and showed that it was usually employed in patients with a more aggressive spectrum of the disease. The disparity of intravenous iloprost treatment strategies in the different centers suggests the need of a rational therapeutical approach based on the clinical characteristics of different patients’ subsets.","PeriodicalId":506053,"journal":{"name":"Journal of Scleroderma and Related Disorders","volume":"55 15","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-01-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139533464","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}
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