Journal of Scleroderma and Related Disorders最新文献

{"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":"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":"9 3","pages":"242-247"},"PeriodicalIF":1.2,"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":"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":"9 3","pages":"192-202"},"PeriodicalIF":1.2,"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":"Systemic sclerosis and acute heart failure in prosthetic heart valve patients: A retrospective analysis.","authors":"Georges Khattar, Chapman Wei, Alanna Davis, Fares Saliba, Laurence Aoun, Omar Mourad, Michel Al Achkar, Angela Rosenberg, Radu Grovu, Stefan Bradu, Suzanne El-Sayegh, Ahmad Mustafa","doi":"10.1177/23971983241278853","DOIUrl":"10.1177/23971983241278853","url":null,"abstract":"<p><strong>Background: </strong>Acute heart failure in patients with prosthetic heart valves is a complex problem with clinical and therapeutic challenges. Systemic sclerosis is a chronic autoimmune disease frequently associated with valvular abnormalities. The association between systemic sclerosis and acute heart failure in patients with prosthetic heart valves remains understudied.</p><p><strong>Methods: </strong>Prosthetic valve patients were extracted from the National Inpatient Sample Database. Baseline patient demographics, comorbidities, and known acute heart failure risk factors were collected from the database using International Classification of Diseases, 10th Revision codes. Patients were subsequently stratified by the diagnosis of systemic sclerosis. The primary outcome was acute heart failure. while secondary outcome included pulmonary outcomes. Univariate and multivariate logistic regression analyses were performed. 1:1 matching was performed to verify our findings.</p><p><strong>Results: </strong>Among 188,615 patients, 235 patients had systemic sclerosis. Systemic sclerosis patients had higher rates of acute heart failure relative to non-systemic sclerosis patients (28.5% vs 22.6%). On multivariate analysis, systemic sclerosis was associated with increased acute heart failure (adjusted OR: 1.38 (1.02-1.85), p = 0.036). After matching, systemic sclerosis was still associated with an increased incidence of acute heart failure (OR: 1.94 (1.25-3.03), p = 0.003). On subgroup analysis, patients with CREST syndrome did not show significantly increased acute heart failure (OR: 1.44 (0.84-2.47), p = 0.184). Patients with systemic sclerosis also showed a significantly higher rate of acute respiratory failure compared to non-systemic sclerosis patients (20.9% vs 13.7%, p = 0.001).</p><p><strong>Conclusion: </strong>Systemic sclerosis may increase the risk for acute heart failure in patients with prosthetic valves. Closer monitoring for heart failure symptoms should be considered in systemic sclerosis patients with prosthetic valves.</p>","PeriodicalId":17036,"journal":{"name":"Journal of Scleroderma and Related Disorders","volume":" ","pages":"23971983241278853"},"PeriodicalIF":1.4,"publicationDate":"2024-09-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11559524/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142621878","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":"National Registry for Childhood Onset Scleroderma I: Insights from the first 341 juvenile localized scleroderma patients.","authors":"Samantha A Branton, Leigh A Stubbs, Haley J Havrilla, Kathryn S Torok","doi":"10.1177/23971983241272460","DOIUrl":"10.1177/23971983241272460","url":null,"abstract":"<p><strong>Objectives: </strong>There is an under recognition of juvenile-onset localized scleroderma and its extracutaneous manifestations leading to delay in systemic treatment. Our study aims to address this gap by describing the demographics, presentation, associated family history, concurrent autoimmune disease, extracutaneous manifestations, laboratory evaluation, treatment, and course of disease in juvenile-onset localized scleroderma patients enrolled in the National Registry for Childhood Onset Scleroderma.</p><p><strong>Methods: </strong>Participants for this study were derived from the National Registry for Childhood Onset Scleroderma and included 341 patients with juvenile-onset localized scleroderma. Demographic, and prospectively collected outcome measures, such as the Localized Scleroderma Cutaneous Assessment Tool, physical exam findings, laboratory values, and patient-reported outcomes were reviewed.</p><p><strong>Results: </strong>Most patients were female (71%), Caucasian (94%), had a linear subtype (56%), and had the onset of disease at age 7.5 (±4.2) years, and diagnosis 1.9 (±2.6) years after symptom onset. Most patients experienced at least one extracutaneous manifestation (70%), most commonly musculoskeletal (57%), followed by neurological (46%), and ophthalmological (11%). Those with musculoskeletal extracutaneous manifestation have significantly abnormal inflammatory and antibody laboratory values. Of patients with 1-year follow-up, a majority were treated with systemic therapy and globally improved with significant reduction in both modified Localized Scleroderma Skin Index (<i>p</i> < 0.001) and Localized Scleroderma Damage Index (<i>p</i> = 0.001).</p><p><strong>Conclusion: </strong>The study highlights need for earlier recognition of juvenile-onset localized scleroderma after demonstrating the delay in diagnosis and frequent extracutaneous manifestations with significant disease burden in a juvenile-onset localized scleroderma cohort. The benefits of systemic treatment and full extracutaneous manifestation screening in juvenile-onset localized scleroderma is supported.</p>","PeriodicalId":17036,"journal":{"name":"Journal of Scleroderma and Related Disorders","volume":" ","pages":"23971983241272460"},"PeriodicalIF":1.4,"publicationDate":"2024-09-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11559527/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142621846","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":"Intermittent versus continuous intravenous epoprostenol for the treatment of digital ischemia.","authors":"Sarah Alsomairy, Kristen T Pogue, Karin M Durant, Adamo Brancaccio","doi":"10.1177/23971983241276677","DOIUrl":"10.1177/23971983241276677","url":null,"abstract":"<p><p>Digital ischemia can be a painful complication of Raynaud's phenomenon or systemic sclerosis, which is caused by narrowing of blood vessels in the toes and hands. Epoprostenol is a potent vasodilator that may be used to treat digital ischemia in this patient population. Our institution provides epoprostenol infusion using two different administration techniques: a 30-h continuous infusion option and a 5-day intermittent 6-h infusion. In this retrospective chart review, we compared two administration techniques of intravenous epoprostenol administered to patients with digital ischemia. The primary outcome was to compare the efficacy of intravenous epoprostenol 30-h continuous infusion versus 5-day intermittent infusion, as defined by the presence of treatment failure. Between June 2019 and June 2020, 72 adult patient encounters met the inclusion criteria (intermittent: <i>n</i> = 20; continuous: <i>n</i> = 52). The primary outcome did not achieve a statistically significant difference between the two groups: intermittent 20% versus continuous 33.3% <i>p</i> = 0.390, odds ratio = 0.57 (95% confidence interval = 0.17-1.90). Adverse reactions were documented in 28% of patients across both treatment groups, and there was no difference detected when treatment groups were compared (25% vs 28.8%). Patients who received the 5-day infusion experienced a significantly longer average length of stay, with a mean of 8.9 days versus 3 days for those treated with the continuous 30-h infusion (<i>p</i> < 0.05; 95% confidence interval = 2.15-9.47). This study determined that the efficacy and safety profiles of the two administration techniques may not be comparable. Each protocol offers advantages over the other, and selection should be guided by patient history and risk factors to optimize management.</p>","PeriodicalId":17036,"journal":{"name":"Journal of Scleroderma and Related Disorders","volume":" ","pages":"23971983241276677"},"PeriodicalIF":1.4,"publicationDate":"2024-09-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11561961/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142648120","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":"Morphea-like tattoo reaction in a patient with systemic sclerosis: Case report and review of the literature.","authors":"Catherine Grace P Hobayan, Jasmine Thai, Abraham M Korman, Judith Lin","doi":"10.1177/23971983241272719","DOIUrl":"10.1177/23971983241272719","url":null,"abstract":"<p><strong>Background: </strong>Morphea-like tattoo reactions are rare phenomena, with few cases reported in the literature. We present a case of a morphea-like tattoo reaction and a literature review of such reactions for comparison.</p><p><strong>Case description: </strong>A 38-year-old woman with known history of systemic sclerosis presented with abnormal healing and skin thickening over a red tattoo. Histopathological examination revealed sclerosing dermatitis, consistent with morphea-like tattoo reaction. Treatment included topical clobetasol and oral mycophenolate mofetil.</p><p><strong>Methods: </strong>A literature search of PubMed and EMBASE was performed in August 2023 for known morphea-like tattoo reactions. No time or language filters were applied.</p><p><strong>Results: </strong>A total of six articles were included. Case reports of morphea-like tattoo reactions in patients with no significant past medical history comprise five articles. One review article notes that red tattoo ink with and without cinnabar is associated with adverse skin reactions.</p><p><strong>Conclusions: </strong>Morphea-like tattoo reactions can be triggered by ingredients of tattoo ink, possibly due to local hypersensitivity or the Koebner phenomenon. We encourage high clinical suspicion for morphea-like tattoo reactions when a patient with known history of connective tissue disease presents with skin changes around a tattoo.</p>","PeriodicalId":17036,"journal":{"name":"Journal of Scleroderma and Related Disorders","volume":" ","pages":"23971983241272719"},"PeriodicalIF":1.4,"publicationDate":"2024-09-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11561960/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142648057","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":"Association of support group leader experience with Scleroderma Patient-centered Intervention Network Support group Leader EDucation Program outcomes: Secondary analysis of a two-arm parallel partially nested randomized controlled trial.","authors":"Brett D Thombs, Brooke Levis, Marie-Eve Carrier, Laura Dyas, Violet Konrad, Maureen Sauvé, Andrea Benedetti","doi":"10.1177/23971983241272742","DOIUrl":"10.1177/23971983241272742","url":null,"abstract":"<p><strong>Introduction/objective: </strong>The Scleroderma Patient-centered Intervention Network Support group Leader EDucation Program was found in a randomized controlled trial to substantially improve leader self-efficacy. Whether the program is effective for leaders with different levels of experience, including candidate leaders with no prior experience and leaders with ⩽3 years experience or ⩾4 years experience, is not known. The objective of the present post hoc secondary analysis was to evaluate outcomes by leader experience, age, and education.</p><p><strong>Methods: </strong>The trial was a pragmatic, two-arm partially nested randomized controlled trial with 1:1 allocation to intervention or waitlist control. Eligible participants were existing or candidate support group leaders. The 13-session leader training was delivered in groups of five to six participants weekly via videoconference in 60- to 90-min sessions. The primary outcome was leader self-efficacy, measured by the Support Group Leader Self-efficacy Scale (SGLSS) immediately post-intervention. Secondary outcomes were Support Group Leader Self-efficacy Scale scores 3 months post-intervention and emotional distress, leader burnout, and volunteer satisfaction post-intervention and 3 months post-intervention. Leaders were classified as having no experience, ⩽3 years experience, or ⩾4 years experience.</p><p><strong>Results: </strong>A total of 148 participants were randomized to intervention (N = 74) or waitlist (N = 74). Compared to leaders with ⩾4 years of experience, Support Group Leader Self-efficacy Scale scores were non-statistically significantly higher post-intervention for leaders with 0-3 years experience and lower for leaders with no experience. The 3 months post-intervention Support Group Leader Self-efficacy Scale scores were significantly lower for leaders without experience and similar for leaders with 0-3 years to those with ⩾4 years experience. There were no differences by experience on other outcomes or by age and education on any outcomes.</p><p><strong>Conclusion: </strong>Support group leader education improved leader self-efficacy but was most effective for leaders with experience prior to initiating the program.</p><p><strong>Trial registration: </strong>NCT03965780; registered on May 29, 2019.</p>","PeriodicalId":17036,"journal":{"name":"Journal of Scleroderma and Related Disorders","volume":" ","pages":"23971983241272742"},"PeriodicalIF":1.4,"publicationDate":"2024-09-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11561947/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142648127","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":"Vitamin D serum levels in patients with systemic sclerosis and very early systemic sclerosis (VEDOSS).","authors":"Giovanna Cuomo, Maria Consiglia Trotta, Giovanbattista D'Amico, Claudio Di Vico, Carlo Iandoli, Danilo Perretta, Caterina Naclerio, Tiziana Nava, Domenico Cozzolino, Ciro Romano","doi":"10.1177/23971983241265583","DOIUrl":"10.1177/23971983241265583","url":null,"abstract":"<p><strong>Introduction: </strong>Vitamin D may be capable of interfering with the pathophysiological pathways involved in systemic sclerosis, by virtue of its well-known immunomodulatory effects. In this study, we aimed at evaluating the differences and the correlations between vitamin D levels in systemic sclerosis patients versus patients with very early systemic sclerosis.</p><p><strong>Methods: </strong>One hundred twenty-six patients (80 definite systemic sclerosis and 46 very early systemic sclerosis) were included in this case control study. Anthropometric, clinical, biochemical, and instrumental data were recorded and correlated with serum vitamin D levels.</p><p><strong>Results: </strong>Briefly, systemic sclerosis patients and very early systemic sclerosis subjects significantly differed for telangectasias, scleredema, autoantibody profile, and videocapillaroscopic pattern. In addition, the mean vitamin D levels were significantly lower in systemic sclerosis patients when compared to those of very early systemic sclerosis subjects. When systemic sclerosis patients were divided into two groups, that is, those with ⩽20 ng/ml versus >20 ng/ml vitamin D serum levels, significantly higher serum vitamin D levels were observed in patients with a lesser skin and vascular involvement. With regard to very early systemic sclerosis subjects, who exhibited baseline satisfactory vitamin D levels, only the autoantibody profile was found to correlate with vitamin D serum levels.</p><p><strong>Conclusion: </strong>Vitamin D serum levels were found to be generally satisfactory in very early systemic sclerosis subjects, but they were reduced in systemic sclerosis patients. Advanced skin and microvascular involvement were found to predispose to hypovitaminosis D. Due to the well-documented immunomodulatory properties of vitamin D, studies are needed to determine whether vitamin D supplementation may prevent the subsequent evolution of very early systemic sclerosis into definite systemic sclerosis.</p>","PeriodicalId":17036,"journal":{"name":"Journal of Scleroderma and Related Disorders","volume":" ","pages":"23971983241265583"},"PeriodicalIF":1.4,"publicationDate":"2024-08-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11561934/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142648157","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":"9 2","pages":"81-85"},"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":"Alveolar epithelial-to-mesenchymal transition in scleroderma interstitial lung disease: Technical challenges, available evidence and therapeutic perspectives.","authors":"Enrico De Lorenzis, Christopher William Wasson, Francesco Del Galdo","doi":"10.1177/23971983231181727","DOIUrl":"10.1177/23971983231181727","url":null,"abstract":"<p><p>The alveolar epithelial-to-mesenchymal transition is the process of transformation of differentiated epithelial cells into mesenchymal-like cells through functional and morphological changes. A partial epithelial-to-mesenchymal transition process can indirectly contribute to lung fibrosis through a paracrine stimulation of the surrounding cells, while a finalized process could also directly enhance the pool of pulmonary fibroblasts and the extracellular matrix deposition. The direct demonstration of alveolar epithelial-to-mesenchymal transition in scleroderma-related interstitial lung disease is challenging due to technical pitfalls and the limited availability of lung tissue samples. Similarly, any inference on epithelial-to-mesenchymal transition occurrence driven from preclinical models should consider the limitations of cell cultures and animal models. Notwithstanding, while the occurrence or the relevance of this phenomenon in scleroderma-related interstitial lung disease have not been directly and conclusively demonstrated until now, pre-clinical and clinical evidence supports the potential role of epithelial-to-mesenchymal transition in the development and progression of lung fibrosis. Evidence consolidation on scleroderma-related interstitial lung disease epithelial-to-mesenchymal transition would pave the way for new therapeutic opportunities to prevent, slow or even reverse lung fibrosis, drawing lessons from current research lines in neoplastic epithelial-to-mesenchymal transition.</p>","PeriodicalId":17036,"journal":{"name":"Journal of Scleroderma and Related Disorders","volume":"1 1","pages":"7-15"},"PeriodicalIF":2.0,"publicationDate":"2024-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10848925/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"65969812","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}