Journal of Scleroderma and Related Disorders最新文献

{"title":"Are there more acute cardiac hospitalizations in winter in patients with systemic sclerosis? An analysis from the National Inpatient Sample.","authors":"Yiming Luo, Laura Ross, Jiayi Zheng, Elana J Bernstein","doi":"10.1177/23971983231197268","DOIUrl":"10.1177/23971983231197268","url":null,"abstract":"<p><strong>Objective: </strong>Cold-induced transient myocardial ischemia has been described in patients with systemic sclerosis. The clinical impact of cold exposure in systemic sclerosis patients with acute cardiac conditions is unknown. We compared the seasonal variation of acute cardiac hospitalizations in patients with and without systemic sclerosis.</p><p><strong>Methods: </strong>We performed a retrospective cross-sectional study using the National Inpatient Sample from 2016 to 2019. The primary outcome was acute cardiac hospitalization primarily due to heart failure, acute myocardial infarction, or cardiac arrhythmias. We compared the proportion of acute cardiac hospitalizations in each season in patients with and without systemic sclerosis. We also performed a subgroup analysis by US geographic region (Northeast, Midwest, South, West).</p><p><strong>Results: </strong>There were a total of 10,118,002 acute cardiac hospitalizations over the 4-year study period. Compared to those without systemic sclerosis, patients with systemic sclerosis who were hospitalized for acute cardiac care were younger (mean age 67 ± 13 vs 70 ± 14 years, p < 0.01), a greater proportion were female (82% vs 45%, p < 0.01), and a smaller proportion were Caucasian (68% vs 71%, p < 0.01). There was a lesser proportion of traditional cardiovascular risk factors in systemic sclerosis compared to non-systemic sclerosis patients. There was no significant difference in the proportion of winter admissions between systemic sclerosis and non-systemic sclerosis patients for total acute cardiac hospitalizations (26.4% vs 25.9%, p = 0.51), heart failure (27.0% vs 26.5%, p = 0.64), acute myocardial infarction (26.9% vs 25.5%, p = 0.50), or arrhythmias (24.3% vs 25.0%, p = 0.68). The results were consistent across all four US geographic regions.</p><p><strong>Conclusion: </strong>Our study did not support that patients with systemic sclerosis had a disproportionally higher risk of acute cardiac hospitalization in winter compared to the general population. We found that systemic sclerosis patients hospitalized for acute cardiac care had a lower burden of traditional cardiovascular risk factors than their non-systemic sclerosis counterparts.</p>","PeriodicalId":17036,"journal":{"name":"Journal of Scleroderma and Related Disorders","volume":"1 1","pages":"59-66"},"PeriodicalIF":1.4,"publicationDate":"2024-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10848930/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"42742143","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":"Approval status of essential therapeutic drugs for systemic sclerosis versus that of drugs for rheumatoid arthritis","authors":"Ki Won Moon, Soo-Hee Hwang, Jieun Yun, E. Lee","doi":"10.1177/23971983231222368","DOIUrl":"https://doi.org/10.1177/23971983231222368","url":null,"abstract":"Systemic sclerosis, a rare disease characterized by chronic multisystem fibrosis, requires lifelong management, necessitating enough insurance coverage for the patient. Official drug approval is the first step to ensuring that the drug is covered by insurance. In this study, we investigated the approval status of essential therapeutic drugs for systemic sclerosis across eight countries and compared it with that of drugs for rheumatoid arthritis. The essential therapeutic drug lists for systemic sclerosis and rheumatoid arthritis were taken from the guidelines of the American College of Rheumatology and the European Alliance of Associations for Rheumatology. Official drug approval status for the selected drugs was confirmed by searching representative Internet databases from eight countries: the United States, the United Kingdom, Germany, France, Italy, Switzerland, Japan, and the Republic of Korea. A total of 21 and 16 drugs were selected for systemic sclerosis and rheumatoid arthritis, respectively. The drug approval rates of the 21 drugs for systemic sclerosis varied among countries. Most drugs used to treat pulmonary arterial hypertension, which were developed recently and are expensive, are approved by most countries; however, most older drugs—which are still essential for management of Raynaud’s phenomenon, digital ulcers, interstitial lung disease, and skin fibrosis—are not approved by most countries. By contrast, almost all of the 16 drugs used to treat rheumatoid arthritis, whether old or new, are approved by most countries. Approval rates for drugs used to treat systemic sclerosis, a rare disease, are much lower than those for drugs used to treat rheumatoid arthritis. Thus, approval rates of essential therapeutic drugs for systemic sclerosis need to improve, which will benefit patients by increasing the number of drugs covered by insurance.","PeriodicalId":17036,"journal":{"name":"Journal of Scleroderma and Related Disorders","volume":"19 8","pages":""},"PeriodicalIF":2.0,"publicationDate":"2024-01-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139446688","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Exploratory clinical subgroup clustering in systemic sclerosis Results from the Indian Progressive Systemic Sclerosis Registry","authors":"Shery Susan Philip, R. Janardana, Padmanabha D. Shenoy, C. Kavadichanda, D. Bairwa, G. Sircar, Parasar Ghosh, A. Wakhlu, Sumithra Selvam, Dinesh Khanna, V. Shobha","doi":"10.1177/23971983231215470","DOIUrl":"https://doi.org/10.1177/23971983231215470","url":null,"abstract":"To conduct an exploratory cluster analysis of systemic sclerosis patients from the baseline data of the Indian systemic sclerosis registry. Patients satisfying American College of Rheumatology-European League Against Rheumatism classification criteria for systemic sclerosis were included. The clusters formed using clinical and immunological parameters were compared. Of the 564 systemic sclerosis registry participants, 404 patients were included. We derived four clusters of which three were anti-topoisomerase I predominant and one was anti-centromere antibody 2 dominant. Cluster 1 ( n-82 (20.3%)) had diffuse cutaneous systemic sclerosis patients with the most severe skin disease, anti-topoisomerase I positivity, males, younger age of onset and high prevalence of musculoskeletal, vasculopathic and gastrointestinal features. Cluster 2 ( n-141 (34.9%)) was also diffuse cutaneous systemic sclerosis and anti-topoisomerase I predominant but with less severe skin phenotype than cluster 1 and a lesser prevalence of musculoskeletal, vasculopathic and gastrointestinal features. Cluster 3 ( n-119 (29.5%)) had limited cutaneous systemic sclerosis patients with anti-topoisomerase I positivity along with other antibodies. The proximal muscle weakness was higher and digital pitting scars were lower, while other organ involvement was similar between clusters 2 and 3. Cluster 4 ( n-62 (15.30%)) was the least severe group with limited cutaneous systemic sclerosis and anti-centromere antibody predominance. Age of onset was higher with low musculoskeletal disease and a higher presence of upper gastrointestinal features. The prevalence of interstitial lung disease was similar in the three anti-topoisomerase I predominant clusters. With exploratory cluster analysis, we confirmed the possibility of subclassification of systemic sclerosis along a spectrum based on clinical and immunological characteristics. We also corroborated the presence of anti-topoisomerase I in limited cutaneous systemic sclerosis and the association of interstitial lung disease with anti-topoisomerase I.","PeriodicalId":17036,"journal":{"name":"Journal of Scleroderma and Related Disorders","volume":"24 3","pages":""},"PeriodicalIF":2.0,"publicationDate":"2023-12-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139003253","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Paul Klee’s progressive dyspnea: A series of historical and clinical vignettes, part V","authors":"Richard M Silver","doi":"10.1177/23971983231213140","DOIUrl":"https://doi.org/10.1177/23971983231213140","url":null,"abstract":"Paul Klee (1879–1940), the 20th-century Swiss-German artist, suffered and died from complications of systemic sclerosis (SSc, scleroderma). This is the fifth in a series of clinical and historical vignettes wherein Klee’s cardiopulmonary symptoms are described with an emphasis on how progressive dyspnea impacted Klee’s life.","PeriodicalId":17036,"journal":{"name":"Journal of Scleroderma and Related Disorders","volume":"80 7","pages":""},"PeriodicalIF":2.0,"publicationDate":"2023-12-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"138597966","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Use of exercise tests in screening for pulmonary arterial hypertension in systemic sclerosis: A systematic literature review","authors":"Sarah Madigan, Susanna Proudman, David Schembri, Huw Davies, Robert Adams","doi":"10.1177/23971983231199148","DOIUrl":"https://doi.org/10.1177/23971983231199148","url":null,"abstract":"Background and objective: Patients with systemic sclerosis (SSc) and pulmonary arterial hypertension (PAH) have a poor prognosis, accounting for 30% of all SSc-related deaths. Guidelines recommend annual screening for PAH regardless of symptoms, as early treatment improves outcomes. Current protocols include combinations of clinical features, biomarkers, pulmonary function tests, and echocardiography. None include exercise testing, although early-stage PAH may only be evident during exercise. This systematic review assessed the performance of exercise tests in predicting the presence of PAH in patients with SSc, where PAH was confirmed through right heart catheterisation (RHC). Methods: Comprehensive literature searches were performed using MEDLINE, EMBASE, Cochrane Database of Systematic Reviews and Cochrane Central Register of Controlled Trails, CINAHL, Scopus and Web of Science from inception to May 2023. Articles were screened for eligibility by two independent reviewers. Eligibility criteria included the use of a non-invasive exercise test to screen adult patients to detect PAH in a population without a previous diagnosis of PAH, with diagnosis confirmed by RHC. Results: Eight studies met the inclusion criteria, describing at least one of three different non-invasive exercise tests: cardiopulmonary exercise test, six-minute walk test and stress Doppler echocardiography. All studies found that exercise tests had some ability to predict the presence of PAH, with sensitivity between 50% and 100% and specificity from 73% to 91%. Conclusion: Exercise tests are infrequently used for screening for PAH in SSc but can predict the presence of PAH. More data are required to establish which tests are most effective.","PeriodicalId":17036,"journal":{"name":"Journal of Scleroderma and Related Disorders","volume":"16 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2023-10-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"135899953","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Nailfold capillaroscopy and candidate-biomarker levels in systemic sclerosis-associated pulmonary hypertension: A cross-sectional study.","authors":"Jacqueline Mj Lemmers,&nbsp;Arjan Pm van Caam,&nbsp;Brigit Kersten,&nbsp;Cornelia Hm van den Ende,&nbsp;Hanneke Knaapen,&nbsp;Arie Pj van Dijk,&nbsp;Wanda Hagmolen Of Ten Have,&nbsp;Frank Hj van den Hoogen,&nbsp;Hans Koenen,&nbsp;Sander I van Leuven,&nbsp;Wynand Alkema,&nbsp;Ruben L Smeets,&nbsp;Madelon C Vonk","doi":"10.1177/23971983231175213","DOIUrl":"https://doi.org/10.1177/23971983231175213","url":null,"abstract":"<p><strong>Objectives: </strong>Pulmonary hypertension is one of the leading causes of death in systemic sclerosis. Early detection and treatment of pulmonary hypertension in systemic sclerosis is crucial. Nailfold capillaroscopy microscopy, vascular autoantibodies AT1R and ETAR, and several candidate-biomarkers have the potential to serve as noninvasive tools to identify systemic sclerosis patients at risk for developing pulmonary hypertension. Here, we explore the classifying potential of nailfold capillaroscopy microscopy characteristics and serum levels of selected candidate-biomarkers in a sample of systemic sclerosis patients with and without different forms of pulmonary hypertension.</p><p><strong>Methods: </strong>A total of 81 consecutive systemic sclerosis patients were included, 40 with systemic sclerosis pulmonary hypertension and 41 with no pulmonary hypertension. In each group, quantitative and qualitative nailfold capillaroscopy microscopy characteristics, vascular autoantibodies AT1R and ETAR, and serum levels of 24 soluble serum factors were determined. For evaluation of the nailfold capillaroscopy microscopy characteristics, linear regression analysis accounting for age, sex, and diffusing capacity of the lungs for carbon monoxide percentage predicted was used. Autoantibodies and soluble serum factor levels were compared using two-sample <i>t</i> test with equal variances.</p><p><strong>Results: </strong>No statistically significant differences were observed in quantitative or qualitative nailfold capillaroscopy microscopy characteristics, or vascular autoantibody ETAR and AT1R titer between systemic sclerosis-pulmonary hypertension and systemic sclerosis-no pulmonary hypertension. In contrast, several serum levels of soluble factors differed between groups: Endostatin, sVCAM, and VEGFD were increased, and CXCL4, sVEGFR2, and PDGF-AB/BB were decreased in systemic sclerosis-pulmonary hypertension. Random forest classification identified Endostatin and CXCL4 as the most predictive classifiers to distinguish systemic sclerosispulmonary hypertension from systemic sclerosis-no pulmonary hypertension.</p><p><strong>Conclusion: </strong>This study shows the potential for several soluble serum factors to distinguish systemic sclerosis-pulmonary hypertension from systemic sclerosis-no pulmonary hypertension. We found no classifying potential for qualitative or quantitative nailfold capillaroscopy microscopy characteristics, or vascular autoantibodies.</p>","PeriodicalId":17036,"journal":{"name":"Journal of Scleroderma and Related Disorders","volume":"8 3","pages":"221-230"},"PeriodicalIF":2.0,"publicationDate":"2023-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10515989/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"41124450","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":"Anti-thymocyte globulin exposure in patients with diffuse cutaneous systemic sclerosis undergoing autologous haematopoietic stem cell transplantation.","authors":"Yu-Hsiang Chiu,&nbsp;Anouk Drijver,&nbsp;Rick Admiraal,&nbsp;Anna van Rhenen,&nbsp;Stefan Nierkens,&nbsp;Jacob M van Laar,&nbsp;Julia Spierings","doi":"10.1177/23971983231188232","DOIUrl":"https://doi.org/10.1177/23971983231188232","url":null,"abstract":"<p><strong>Introduction: </strong>Autologous haematopoietic stem cell transplantation improves event-free survival and lung function and reduces skin thickening in patients with progressive diffuse cutaneous systemic sclerosis. Anti-thymocyte globulin is a key lymphoablative constituent of conditioning protocols and is administered in a weight-based dosage. However, whether anti-thymocyte globulin exposure contributes to response to autologous haematopoietic stem cell transplantation and lymphocyte reconstitution in diffuse cutaneous systemic sclerosis patients is unknown. We aimed to explore the relationship between anti-thymocyte globulin exposure, lymphocyte reconstitution and treatment response in diffuse cutaneous systemic sclerosis patients undergoing autologous haematopoietic stem cell transplantation.</p><p><strong>Methods: </strong>A retrospective cohort of 15 diffuse cutaneous systemic sclerosis patients undergoing autologous haematopoietic stem cell transplantation was performed. Clinical characteristics and routine laboratory results were retrieved from electronic medical records. Anti-thymocyte globulin concentrations were measured in cryopreserved plasma samples at four time points (day 1 and week 1, 2 and 4) after stem cell reinfusion. Anti-thymocyte globulin exposure was estimated using a validated population pharmacokinetic model.</p><p><strong>Results: </strong>During a median follow-up of 45 months (interquartile range 19-66), 11 (73%) patients had a treatment response, and 4 (27%) were non-responders. Although all patients received the same weight-based anti-thymocyte globulin dosage, 7.5 mg/kg divided over 3 days, anti-thymocyte globulin exposure varied. Anti-thymocyte globulin exposure was higher in responders than in non-responders (163 AU*day/mL (interquartile range 153-183) and 137 AU*day/mL (interquartile range 101-149), respectively, <i>p</i> = .026). Anti-thymocyte globulin exposure was not correlated with lymphocyte reconstitution or infection rate.</p><p><strong>Conclusion: </strong>Weight-based dosing of anti-thymocyte globulin results in variable anti-thymocyte globulin exposure and treatment response across individuals.</p>","PeriodicalId":17036,"journal":{"name":"Journal of Scleroderma and Related Disorders","volume":"8 3","pages":"241-246"},"PeriodicalIF":2.0,"publicationDate":"2023-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10515999/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"41134026","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":"Support and information needs of people with systemic sclerosis by time since diagnosis: A cross-sectional study.","authors":"Sabrina Provencher,&nbsp;Richard S Henry,&nbsp;Carolina Bacalao,&nbsp;Marie-Eve Carrier,&nbsp;Linda Kwakkenbos,&nbsp;Brett D Thombs","doi":"10.1177/23971983231181726","DOIUrl":"https://doi.org/10.1177/23971983231181726","url":null,"abstract":"<p><strong>Background: </strong>How support and informational needs of people with systemic sclerosis (SSc) may differ by time since diagnosis is not known. Our objective was to determine if informational and support needs of recently diagnosed individuals with systemic sclerosis differ from people diagnosed for longer periods of time.</p><p><strong>Methods: </strong>The North American Scleroderma Support Group Members survey included 30 items on reasons for attending support groups. Respondents were classified by time since diagnosis of 0-3 years, 4-9 years or 10+ years. Survey item responses were dichotomized into <i>Not Important</i> or <i>Somewhat Important</i> versus <i>Important</i> or <i>Very Important</i>. We conducted Chi-square tests with Hochberg's Sequential Method to identify item differences by time since diagnosis.</p><p><strong>Results: </strong>A total of 175 respondents completed the survey. Most support needs were rated as <i>Important</i> or <i>Very Important</i> by respondents, regardless of disease duration, particularly needs related to interpersonal and social support (10 items; median 81%) and learning about disease treatment and management strategies (11 items; median 82%). Discussing other aspects of living with systemic sclerosis (e.g. spirituality, discussing disease with family and friends) was rated lower (9 items; 44%). Respondents with 0-3 years since diagnosis were the highest on 29 of 30 items. Respondents with 0-3 years since diagnosis were significantly higher on items related to discussing medical care and 4 items on other aspects (spirituality, talking with family and friends, financial issues, sexual issues).</p><p><strong>Conclusion: </strong>People with systemic sclerosis have a wide range of information and support needs, regardless of their disease duration, but people with recent diagnoses have greater needs.</p>","PeriodicalId":17036,"journal":{"name":"Journal of Scleroderma and Related Disorders","volume":"8 3","pages":"247-252"},"PeriodicalIF":2.0,"publicationDate":"2023-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/8d/2a/10.1177_23971983231181726.PMC10515994.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"41162890","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":"Therapeutic effects of thalidomide on patients with systemic sclerosis-associated interstitial lung disease.","authors":"Jie Pan, Fei Dong, Li Ma, Cheng Zhao, Fang Qin, Jing Wen, Wanling Wei, Ling Lei","doi":"10.1177/23971983231180077","DOIUrl":"10.1177/23971983231180077","url":null,"abstract":"<p><strong>Objective: </strong>To evaluate the clinical efficacy of thalidomide in patients with systemic sclerosis-associated interstitial lung disease.</p><p><strong>Methods: </strong>Ninety-six systemic sclerosis-associated interstitial lung disease patients who received basic glucocorticoid treatment and admitted between 2016 and 2020 were included in this study, including 48 cases in the thalidomide group (combination of thalidomide and cyclophosphamide) and 48 cases in control group (cyclophosphamide monotherapy). Evaluation items included clinical symptoms, modified Rodnan skin score, pulmonary function test, chest high-resolution computed tomography scores, and adverse effects between two groups after 24 weeks of treatment.</p><p><strong>Results: </strong>Remarkable improvements in several aspects were found in the thalidomide group, including modified Rodnan skin score, expiratory dyspnea score, cough visual analog scale score, total ground-glass opacity score, and total interstitial lung disease score. Compared to the control group, improvements in the thalidomide group were found, such as significantly decreased cough visual analog scale score and expectoration; increased number of platelets; improved pulmonary fibrosis (<i>p</i> <i>=</i> 0.056), and reduced carbon monoxide diffusing capacity (<i>p</i> = 0.053). There were no statistically significant differences in the expiratory dyspnea score and predicted forced vital capacity between the two groups. Patients who experienced at least one adverse event in the control group and thalidomide group were 33.3% and 64.6% (<i>p</i> = 0.002); while those with serious adverse events were 8.3% versus 12.5% (<i>p</i> = 0.504). Venous thrombosis was found in one case in the thalidomide group.</p><p><strong>Conclusion: </strong>Thalidomide combined with cyclophosphamide can improve the symptoms of cough and expectoration in patients with systemic sclerosis-associated interstitial lung disease, and may slightly delay the progression of pulmonary fibrosis, but with the possibility of an increased risk of adverse events.</p>","PeriodicalId":17036,"journal":{"name":"Journal of Scleroderma and Related Disorders","volume":"8 3","pages":"231-240"},"PeriodicalIF":1.4,"publicationDate":"2023-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10515992/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"41139654","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":"Scleroderma renal crisis triggered by ibuprofen: Insights on complement-directed therapy.","authors":"Prochore Kamgang Semeu, Maxime Taghavi, Caroline Geers, Luc Mouthon, Leonel Barreto Gutierrez, Patrick Stordeur","doi":"10.1177/23971983231163663","DOIUrl":"10.1177/23971983231163663","url":null,"abstract":"<p><p>Scleroderma renal crisis is a severe complication of systemic sclerosis with a poor prognosis. Therefore, identifying precipitating factors is essential. Among known risk factors, only few are reversible. On the contrary, anti-C5 therapy appears effective, at least in some cases. We describe a 59-year-old man with diffuse cutaneous systemic sclerosis who developed life-threatening scleroderma renal crisis following ibuprofen administration. Despite aggressive management, he did not improve. Renal biopsy have displayed features of thrombotic microangiopathy but no complement deposition. We then discuss the pathomechanism of scleroderma renal crisis that could drive eculizumab treatment since some renal biopsies exhibit complement deposits and others do not.</p>","PeriodicalId":17036,"journal":{"name":"Journal of Scleroderma and Related Disorders","volume":"8 3","pages":"NP6-NP8"},"PeriodicalIF":1.4,"publicationDate":"2023-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10515997/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"41130919","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}