Journal of Scleroderma and Related Disorders最新文献

{"title":"Efficacy and safety of digital nerve block for pain management during sharp debridement of digital ulcers in systemic sclerosis: A prospective observational study.","authors":"Riccardo Bixio, Francesca Mastropaolo, Francesca Nava, Olta Veliaj, Elena Fracassi, Ombretta Viapiana, Maurizio Rossini, Luca Idolazzi","doi":"10.1177/23971983241285206","DOIUrl":"10.1177/23971983241285206","url":null,"abstract":"<p><strong>Introduction: </strong>Systemic sclerosis (SSc) is a chronic autoimmune disease characterized by fibrosis and vascular abnormalities, often leading to the development of digital ulcers (DUs). DUs are painful and debilitating, significantly impacting patients' quality of life. Effective pain management during debridement is crucial, yet there is no consensus on the optimal approach. This study evaluates the efficacy and safety of digital nerve block (DNB) using lidocaine and mepivacaine for pain control during sharp debridement of DUs in SSc patients.</p><p><strong>Methods: </strong>This prospective observational study was conducted from September 2023 to May 2024 at the Rheumatology Operating Unit, University of Verona. Patients were randomized to receive either 1 mL of 2% lidocaine or 2% mepivacaine for DNB. Pain levels were assessed using a categorical grading scale during the injection and debridement. The outcomes were pain control and performance of lidocaine versus mepivacaine.</p><p><strong>Results: </strong>The cohort developed 46 ulcers. The pain was abolished in almost all patients. Lidocaine achieved faster anaesthesia (127.92 ± 34.32 s) compared with mepivacaine (252.65 ± 49.89 s, <i>p</i> < 0.001). Mepivacaine resulted in less pain during injection (<i>p</i> = 0.006). No significant difference in pain levels during debridement was observed between the two anaesthetics. Three mild adverse effects (Raynaud's phenomenon) were reported. All procedures were completed successfully, and 35 ulcers healed with a mean time of 6.1 ± 7.77 weeks.</p><p><strong>Conclusions: </strong>DNB with mepivacaine provides effective pain control during DU debridement in SSc patients, with lower injection site pain and comparable efficacy to lidocaine. The procedure is safe, well-tolerated and facilitates successful ulcer healing. Further studies with larger cohorts are warranted to confirm these findings.</p>","PeriodicalId":17036,"journal":{"name":"Journal of Scleroderma and Related Disorders","volume":" ","pages":"23971983241285206"},"PeriodicalIF":1.4,"publicationDate":"2024-10-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11559528/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142621687","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":"The importance of body image and aesthetic medicine in systemic sclerosis.","authors":"Martina Orlandi, Amelia Spinella, Marco De Pinto, Gilda Sandri, Giorgio De Santis, Dilia Giuggioli","doi":"10.1177/23971983241285212","DOIUrl":"10.1177/23971983241285212","url":null,"abstract":"<p><p>Systemic sclerosis is a progressive fibrotic autoimmune disease which mainly affects young women causing fibrosis of the skin and internal organs. In systemic sclerosis patients, facial and hand alterations are significant as these may have an impact on body image. In fact, systemic sclerosis could cause disfigurement of the most visible parts of the body, hindering self-confidence and quality of life. Therefore, the assessment of body image in systemic sclerosis is crucial in order to optimize a tailored care of the patient. In the near future, the creation of a pathway involving dedicated specialists (psychologists, physiotherapists, dermatologists, and plastic surgeons) should become an integral part of the disease management.</p>","PeriodicalId":17036,"journal":{"name":"Journal of Scleroderma and Related Disorders","volume":" ","pages":"23971983241285212"},"PeriodicalIF":1.4,"publicationDate":"2024-10-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11559588/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142621885","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":"Improving organisation to improve care: ERN ReCONNET organisational reference model for systemic sclerosis patients' care pathway.","authors":"Rosaria Talarico, Diana Marinello, Ilaria Palla, Sara Cannizzo, Ilaria Galetti, Sue Farrington, Silvia Aguilera, Jeanette Andersen, Eva Ceccatelli, Alain Cornet, Gema Cutillas, Marco Esteves, Charissa Frank, Catarina Leite, Gabi Niehaus, Elisabeth Perez Gomez, Katleen Polfliet, Silvia Sandulescu, Rita Schriemer, Simone Barsotti, Silvia Bellando-Randone, Lorenzo Beretta, Vera Bernardino, Goncalo Boleto, Stefano Bombardieri, Gerd Burmester, Ilaria Cavazzana, Veronica Codullo, Maurizio Cutolo, Virgil Dalm, Laura Damian, Alessandra Della Rossa, Andrea Doria, Meryem-Maud Farhat, João Eurico Fonseca, Eric Hachulla, Frédéric Houssiau, Maria Grazia Lazzaroni, Maarten Limper, Valentina Lorenzoni, Carlomaurizio Montecucco, Marta Mosca, Luc Mouthon, Ulf Müeller-Ladner, Micheline Pha, Cristina Ponte, Julia Spierings, Alberto Sulli, Anna Viola Taulaigo, Simone Ticciati, Angela Tincani, Natasha Toplak, Leopoldo Trieste, P M van Hagen, Jacob van Laar, Marie Vanthuyne, Barbara Vigone, Jeska K de Vries-Bouwstra, Margherita Zen, Giuseppe Turchetti, Vanessa Smith, Marco Matucci Cerinic","doi":"10.1177/23971983241269109","DOIUrl":"10.1177/23971983241269109","url":null,"abstract":"<p><strong>Objective: </strong>To optimise the organisation of care and encourage the adoption of good clinical practices, the RarERN Path^© methodology was designed within ERN ReCONNET. The aim of our work was to report the application of RarERN Path^© on systemic sclerosis within the ERN ReCONNET centres, providing a feasible and flexible organisational reference model for optimising the systemic sclerosis care pathway in different countries.</p><p><strong>Methods: </strong>RarERN Path^© is a six-phase methodology which enables the creation of a reference organisational model co-designed on the basis of the expertise of different stakeholders. It foresees six phases, ranging from the map of existing patients' care pathways and patients' stories, to the consensus on a common organisational patient care pathways, and its key performance indicators definition.</p><p><strong>Results: </strong>The agreed reference model highlights the importance of having an organisational flow for referrals that foresees how patients may access directly the specialised unit from the different referrals. Specific specialised visits were considered as mandatory to be organised and they included cardiologist, pneumologist, gastroenterologist, psychologist, nephrologist, dermatologist, wound care specialist/nurses and other healthcare professionals (such as nurses, social workers and nutritional counselling). Moreover, specific services related to therapy were highlighted as strongly recommended to be organised, mainly represented by infusion therapy and wound care, as well as occupation therapy and physiotherapy.</p><p><strong>Conclusion: </strong>The organisational model emerged from our investigation emphasises that the organisation of specific services for systemic sclerosis treatment should be organised as a solid support for implementing the existing recommendations on systemic sclerosis management in real life.</p>","PeriodicalId":17036,"journal":{"name":"Journal of Scleroderma and Related Disorders","volume":" ","pages":"23971983241269109"},"PeriodicalIF":1.4,"publicationDate":"2024-10-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11559522/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142621843","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":"Views of nutrition needs in patients with systemic sclerosis.","authors":"De-Arne Samm, Aimee Macoustra, Rhiannon Crane, Leah McWilliams, Susanna Proudman, Lee-Anne S Chapple","doi":"10.1177/23971983241264868","DOIUrl":"10.1177/23971983241264868","url":null,"abstract":"<p><strong>Background and aims: </strong>Systemic sclerosis is a debilitating inflammatory condition synonymous with gastrointestinal symptoms which have the potential to impact dietary intake and nutritional status. This study aimed to describe symptoms experienced by patients with systemic sclerosis that may impact on dietary intake and assess nutrition education preferences in this cohort.</p><p><strong>Methods: </strong>A 24-item online qualitative and quantitative survey distributed via REDCap^® was conducted in adult patients (aged ⩾18 years) living with systemic sclerosis and attending outpatient services at a single healthcare setting from January to March 2022. Data were collected on demographics, symptoms that may impact dietary intake, nutrition priorities and preferred nutrition education models. Data are mean ± standard deviation or number (%).</p><p><strong>Results: </strong>Of 322 eligible patients, 156 (48%) participated (63 ± 12 years, 86% female, body mass index 27 ± 7 kg/m²). Most patients experienced gastrointestinal conditions (n = 123/155; 79%), which occurred daily in 26% (n = 40/155) of patients. A third of patients (n = 48/156; 31%) reported diet manipulation for symptom management. Recent weight loss was common (n = 36/154; 23% of patients). Less than a third of patients had seen a dietitian (n = 45; 29%), while 69% of patients (n = 107) desired dietetic consultancy. The preferred methods of consultation were written resources and face-to-face, respectively, and systemic sclerosis symptom management (n = 100; 64%) and losing weight (n = 53; 34%) were the most desired education topics reported.</p><p><strong>Conclusion: </strong>Gastrointestinal conditions are common and occur frequently in patients with systemic sclerosis. Patients want to engage with dietetics services to better manage symptoms via face-to-face consultations and written resources. These results will inform future dietetic service delivery.</p>","PeriodicalId":17036,"journal":{"name":"Journal of Scleroderma and Related Disorders","volume":"9 3","pages":"216-222"},"PeriodicalIF":1.4,"publicationDate":"2024-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11462576/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142400577","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Efficacy assessment of intravenous immunoglobulin for gastrointestinal involvement in systemic sclerosis using UCLA SCTC GIT: Case-based review.","authors":"Marcelo Neto, Fernando Albuquerque, João Oliveira, Maria João Cadório, Maria João Salvador, Tânia Santiago","doi":"10.1177/23971983241273852","DOIUrl":"10.1177/23971983241273852","url":null,"abstract":"<p><strong>Objectives: </strong>To summarize the published evidence in the literature on the use of intravenous immunoglobulin in gastrointestinal tract involvement in systemic sclerosis patients and report the experience of our department.</p><p><strong>Methods: </strong>A systematic literature review was performed; and a literature search was conducted in MEDLINE and Embase until 1/5/2024, using the participants, intervention, comparator and outcomes framework. Only full-text articles involving systemic sclerosis adults, submitted to intravenous immunoglobulin (at least one administration) to treat primary gastrointestinal tract manifestations. The outcome was the University of California at Los Angeles Scleroderma Clinical Trial Consortium Gastrointestinal Tract 2.0 score to evaluate response to treatment. Two reviewers performed the assessment of data extraction and synthesis, independently.</p><p><strong>Results: </strong>Four papers (two case reports and two retrospective studies) out of 35 references were included. In addition, we added two systemic sclerosis patients from our department in this review. In 25 systemic sclerosis patients, with various gastrointestinal tract manifestations, the intravenous immunoglobulin therapy was found to improve digestive tract symptoms in SSc patients, as shown by the decrease in the scores of University of California at Los Angeles Scleroderma Clinical Trial Consortium Gastrointestinal Tract 2.0. No adverse events were reported, except for one case of low-grade fever post-administration.</p><p><strong>Conclusion: </strong>The results from this systematic literature review based on case series suggest that intravenous immunoglobulin may improve gastrointestinal tract symptoms assessed by the University of California at Los Angeles Scleroderma Clinical Trial Consortium Gastrointestinal Tract 2.0 scale, with only minor reported adverse events, suggestive of an acceptable safety profile. We believe that this systematic literature review will contribute to shed light on the efficacy and safety aspects of intravenous immunoglobulin treatment in the management of gastrointestinal tract symptoms; and multicenter randomized placebo-controlled trials are urgently needed to foster progress in this field.</p>","PeriodicalId":17036,"journal":{"name":"Journal of Scleroderma and Related Disorders","volume":" ","pages":"23971983241273852"},"PeriodicalIF":1.4,"publicationDate":"2024-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11559525/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142621841","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Atypical morphoea, a herald of two malignancies: Lung adenocarcinoma and a neuroendocrine tumour.","authors":"Harmeet Bhullar, Miki Wada, Mandana Nikpour, Amanda M Saracino","doi":"10.1177/23971983241254442","DOIUrl":"https://doi.org/10.1177/23971983241254442","url":null,"abstract":"<p><strong>Introduction: </strong>Morphoea is a disorder characterised by fibrosis and inflammation of the skin and on rare occasions can be precipitated by malignancy. Here, we describe a case of morphoea unmasking two malignancies.</p><p><strong>Case description: </strong>A 73-year-old woman presented with circumferential lower limb skin thickening, associated with violaceous, doughy oedema and significantly impaired mobility. Histology confirmed dermal sclerosis with no increased mucin and broader investigations excluded systemic sclerosis, scleromyxoedema and scleroedema. An atypical morphoea was diagnosed. In the context of atypical and subsequently treatment-resistant disease, further imaging uncovered a lung adenocarcinoma which was promptly treated. Despite this, the patient's atypical oedematous skin sclerosis continued to progress proximally, and she developed flatulence, bloating and atypical flushing. This prompted further investigation, which revealed a metastatic neuroendocrine tumour. The patient was commenced on octreotide, with rapid improvement in all her cutaneous and systemic symptoms.</p><p><strong>Conclusion: </strong>Atypical morphoea can be a herald for an underlying malignancy, representing a paraneoplastic presentation. Progressive treatment-resistant morphoea may be an indicator of metastatic disease, or in our case a second malignancy.</p>","PeriodicalId":17036,"journal":{"name":"Journal of Scleroderma and Related Disorders","volume":"9 3","pages":"NP1-NP4"},"PeriodicalIF":1.4,"publicationDate":"2024-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11457777/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142391425","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"N-terminal pro-brain natriuretic peptide is associated with pulmonary hypertension or diastolic dysfunction in patients with systemic sclerosis: An Australian prospective cross-sectional study.","authors":"Francis J Ha, Zoe Brown, Wendy Stevens, David Prior, Laura Ross, Nava Ferdowsi, Mandana Nikpour, Andrew T Burns","doi":"10.1177/23971983241249209","DOIUrl":"https://doi.org/10.1177/23971983241249209","url":null,"abstract":"<p><strong>Introduction: </strong>Pulmonary arterial hypertension and left ventricular diastolic dysfunction are associated with significant morbidity and mortality in systemic sclerosis. N-terminal pro-brain natriuretic peptide has been proposed as part of composite screening algorithms for pulmonary arterial hypertension. Our aim was to assess the prevalence of pulmonary hypertension and diastolic dysfunction, and evaluate their association with serum N-terminal pro-brain natriuretic peptide in systemic sclerosis patients.</p><p><strong>Methods: </strong>Patients with systemic sclerosis were prospectively enrolled to undergo N-terminal pro-brain natriuretic peptide testing and transthoracic echocardiography at a tertiary Australian centre from January to October 2022. We collected demographic and transthoracic echocardiography variables including pulmonary hypertension estimated by tricuspid regurgitant velocity and diastolic dysfunction assessed by the ASE/EACVI 2016 guidelines. Pearson's correlation coefficient was used to evaluate association between N-terminal pro-brain natriuretic peptide and echocardiographic parameters.</p><p><strong>Results: </strong>Sixty-one patients were enrolled (median age = 62 years (interquartile range = 55-69 years); 84% female). Two-thirds of patients had limited systemic sclerosis (40/61). Five patients (8%) had high likelihood of pulmonary hypertension by transthoracic echocardiography. Seven patients (11%) had diastolic dysfunction; however, seven patients (11%) had indeterminate diastology. Six patients underwent right heart catheterisation, with five patients diagnosed with pulmonary hypertension. N-terminal pro-brain natriuretic peptide in patients with pulmonary hypertension or diastolic dysfunction was significantly higher (median = 207 and 226 pg/mL, respectively) compared to patients without either condition (median = 69 pg/mL, p = 0.01). N-terminal pro-brain natriuretic peptide showed a statistically significant although limited correlation with estimated pulmonary pressures measured by tricuspid regurgitant velocity (r = 0.44, p = 0.002) and left ventricular filling pressures (r = 0.27, p = 0.04).</p><p><strong>Conclusion: </strong>Pulmonary hypertension and diastolic dysfunction are both observed in systemic sclerosis. N-terminal pro-brain natriuretic peptide is associated with both conditions; however, it cannot distinguish between the two disease processes. Right heart catheterisation may be required to make this distinction.</p>","PeriodicalId":17036,"journal":{"name":"Journal of Scleroderma and Related Disorders","volume":"9 3","pages":"178-184"},"PeriodicalIF":1.4,"publicationDate":"2024-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11457770/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142391428","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Changes in work and adequacy of financial resources during COVID-19 among people with systemic sclerosis: A Scleroderma Patient-centered Intervention Network study.","authors":"Claire Adams, Elsa-Lynn Nassar, Marie-Eve Carrier, Linda Kwakkenbos, Richard S Henry, Gabrielle Virgili-Gervais, Sophie Hu, Susan J Bartlett, Catherine Fortuné, Amy Gietzen, Karen Gottesman, Geneviève Guillot, Marie Hudson, Amanda Lawrie-Jones, Nancy Lewis, Vanessa Malcarne, Maureen D Mayes, Scott B Patten, Michelle Richard, Maureen Sauvé, John Varga, Joep Welling, Robyn Wojeck, Luc Mouthon, Andrea Benedetti, Brett D Thombs","doi":"10.1177/23971983241262655","DOIUrl":"https://doi.org/10.1177/23971983241262655","url":null,"abstract":"<p><strong>Introduction/objective: </strong>We investigated (1) work status changes during COVID-19, (2) financial resource adequacy, (3) preferences for work requirements (e.g. remote, workplace, mixed) and (4) work requirements versus preferences, among people with systemic sclerosis.</p><p><strong>Methods: </strong>This was a cross-sectional study of participants in the Scleroderma Patient-centered Intervention Network COVID-19 Cohort, which enrolled participants from the ongoing Scleroderma Patient-centered Intervention Network Cohort and externally in April 2020. In August 2022, participants completed questions on work status, financial well-being using the Consumer Financial Protection Bureau Financial Well-Being Scale, work requirements and work requirement preferences.</p><p><strong>Results: </strong>A total of 298 participants with systemic sclerosis were included. Mean age was 58.6 years (SD = 11.4). There were 101 (34%) participants working at the start of the pandemic and still working in August 2022, 179 (60%) not working at the start of the pandemic and still not working, 10 (3%) who stopped working after April 2020 and 8 (3%) who started working. Mean financial well-being did not change from April 2020 to August 2022 (difference: 0.2 points; 95% confidence interval: -1.1 to 0.7). Working participants (N = 109) preferred flexible work requirements (N = 34, 31%) or working entirely remotely (N = 32, 29%), but most were required to work entirely at a workplace (N = 35, 32%) or combined workplace and remotely with a fixed schedule (N = 31, 28%).</p><p><strong>Conclusion: </strong>Work status and financial well-being did not change substantively among people with systemic sclerosis during the pandemic. Flexible work policies may support people with systemic sclerosis to work.</p>","PeriodicalId":17036,"journal":{"name":"Journal of Scleroderma and Related Disorders","volume":"9 3","pages":"242-247"},"PeriodicalIF":1.4,"publicationDate":"2024-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11457775/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142391426","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"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":1.4,"publicationDate":"2024-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11459481/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142391427","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"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}