Journal of Scleroderma and Related Disorders最新文献

{"title":"Systemic sclerosis and anorectal dysfunction: The Leeds experience","authors":"Nikhil Suresh, Ranjitha Karanth, Ramsah Cheah, John Casey, David G Jayne, F. del Galdo","doi":"10.1177/23971983241241203","DOIUrl":"https://doi.org/10.1177/23971983241241203","url":null,"abstract":"Systemic sclerosis is an autoimmune disorder which frequently affects the gastrointestinal tract. Anorectal dysfunction is common in systemic sclerosis and is manifested mainly by atrophy of internal anal sphincter. Faecal incontinence is the result of internal anal sphincter atrophy secondary to systemic sclerosis. In this study, we aimed to assess the internal anal sphincter in 17 patients with faecal incontinence and systemic sclerosis using anorectal manometry and endoanal ultrasound and compare them with an age and gender-matched control group without systemic sclerosis. Most patients have limited cutaneous systemic sclerosis. Majority of the patients with systemic sclerosis and faecal incontinence presented with symptoms of faecal leakage and urgency. Systemic sclerosis patients had low basal sphincter pressures. The mean thickness of internal anal sphincter in systemic sclerosis group was significantly lower than the control group (p < 0.001). Rectal sensation is preserved in systemic sclerosis. There was no difference in the mean thickness of the external anal sphincter between the two groups. To conclude internal anal sphincter is atrophic in systemic sclerosis resulting in decreased resting sphincter pressures and passive faecal leakage. Further investigations and studies are needed to determine the natural course of faecal incontinence in systemic sclerosis, associated risk factors and efficacy of therapeutic interventions.","PeriodicalId":17036,"journal":{"name":"Journal of Scleroderma and Related Disorders","volume":null,"pages":null},"PeriodicalIF":2.0,"publicationDate":"2024-04-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140732456","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":"Evaluation of autologous fat grafting in the treatment of juvenile localized scleroderma with facial involvement","authors":"Louise Sander, I. Foeldvari, Daniel Glaser, J. Klotsche, Rebecca Stier, Juan Camilo Roldán","doi":"10.1177/23971983241241099","DOIUrl":"https://doi.org/10.1177/23971983241241099","url":null,"abstract":"The objective of this study was to evaluate the effect of autologous fat grafting in patients with juvenile localized scleroderma with facial involvement. We retrospectively studied patients with juvenile localized scleroderma who were followed at the Hamburg Center for Pediatric and Adolescent Rheumatology at least 6 months post-operative follow-up and received at least one autologous fat transplantation for a facial lesion. Autologous fat grafting was conducted independent of disease-modifying treatment and/or disease activity. The effectiveness of the intervention was evaluated by assessing the immediate volume enhancement after injection of fat tissue compared with volume retained after 6 months. The volume enhancement was calculated from three-dimensional photo images analyzed in Mirror Medical Imaging Software, Canfield Scientific (New Jersey, USA). Data of 22 juvenile patients were assessed from March 2006 to September 2021. In six patients, the photos could not be evaluated for the study. The median age of the remaining 16 patients at the beginning of the first fat graft was 8 years (range = 2.5–22 years). Patients underwent mean three interventions (range = 1–9). Evaluation of three-dimensional images showed that the volume retention at 6 months post autologous fat grafting is approximately 50%. The Modified Localized Scleroderma Skin Severity Index value did not correlate to volume changes at 6 months. In 4 of 16 patients, a decrease of the Localized Scleroderma Damage Index occurred. In all seven patients with cheek involvement, mean tragus-nose distance difference decreased (mean decrease 1.09 cm). We did not observe any significant clinical side effects. In this first bigger pediatric case series, the mean facial lesion volume increased around 50% after a mean of three interventions at 25 months of follow-up. No flares of the underlying disease were noted. Autologous fat grafting is a promising method to improve the cosmetic appearance of those patients.","PeriodicalId":17036,"journal":{"name":"Journal of Scleroderma and Related Disorders","volume":null,"pages":null},"PeriodicalIF":2.0,"publicationDate":"2024-04-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140731442","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":"Physical activity during COVID-19 in people with systemic sclerosis: A Scleroderma Patient-centred Intervention Network COVID-19 Cohort longitudinal study","authors":"Amanda Wurz, Richard S Henry, L. Kwakkenbos, M. Carrier, Scott B. Patten, Susan J Bartlett, L. Mouthon, John Varga, Andrea Benedetti, S. Culos-Reed, Brett D. Thombs","doi":"10.1177/23971983241241593","DOIUrl":"https://doi.org/10.1177/23971983241241593","url":null,"abstract":"People with systemic sclerosis (SSc) face barriers to physical activity. Few studies have described physical activity in SSc, and none have explored physical activity longitudinally during COVID-19. We evaluated physical activity from April 2020 to March 2022 among people with SSc. The Scleroderma Patient-centred Intervention Network (SPIN) COVID-19 Cohort was launched in April 2020 and included participants from the ongoing SPIN Cohort plus external enrolees. Participants completed measures bi-weekly through July 2020, then every 4 weeks afterwards (28 assessments). Physical activity was assessed via the self-reported International Physical Activity Questionnaire-Elderly. Analyses included estimated means with 95% confidence intervals for physical activity across assessments. Missing data were imputed for main analyses. Sensitivity analyses included evaluating only participants who completed >90% of items for >21 of 28 possible assessments (‘completers’) and stratified analyses by sex, age, country and SSc subtype. A total of 800 people with SSc enrolled. Mean age was 55.6 (standard deviation (SD) = 12.6) years. Physical activity significantly decreased from April 2020 to March 2021 (standardized mean difference (SMD) = −0.17, 95% confidence interval (CI) = −0.26 to −0.07) and was stable from March 2021 to March 2022 (SMD = −0.05, 95% CI = −0.15 to 0.05). Results were similar for completers and subgroups. The proportion of participants who met World Health Organization minimum physical activity recommendations of at least 150 min of moderate-to-vigorous activity per week ranged from 63% to 82% across assessments. Physical activity decreased by a relatively small amount, on average, across the pandemic. Most participants met recommended physical activity levels.","PeriodicalId":17036,"journal":{"name":"Journal of Scleroderma and Related Disorders","volume":null,"pages":null},"PeriodicalIF":2.0,"publicationDate":"2024-04-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140751269","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":"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":null,"pages":null},"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}

{"title":"Role of cognitive impairment and malnutrition as determinants of quality of life in patients with systemic sclerosis","authors":"D. Benfaremo, N. Pacenti, Ilaria Paterno, Cristina Dichiara, Federica Lucia Galli, G. Moroncini","doi":"10.1177/23971983231224522","DOIUrl":"https://doi.org/10.1177/23971983231224522","url":null,"abstract":"Increasing evidence supports the presence of cognitive impairment in patients with systemic sclerosis. Malnutrition is a well-known severe complication of systemic sclerosis and is a consequence of multiple factors, mainly oropharyngeal and gastrointestinal involvement. Recent studies have shown a link between nutrition and cognitive decline in several chronic diseases. Thus, we decided to evaluate a possible association between malnutrition and cognitive impairment in patients with systemic sclerosis. In total, 100 consecutive systemic sclerosis patients were enrolled in a cross-sectional study to assess clinical and demographic features, nutritional status (body mass index, Global Leadership Initiative on Malnutrition criteria), gastrointestinal involvement (University of California Los Angeles Gastrointestinal Scale 2.0, Eat Assessment Tool 10), cognitive function (Montreal Cognitive Assessment), anxiety and depression (Patient Health Questionnaire 9, Beck Depression Inventory II), and quality of life (Short Form 36, Health Assessment Questionnaire-Disability Index, Scleroderma Health Assessment Questionnaire). Patients were stratified for the presence/absence of malnutrition and cognitive decline and compared for clinical characteristics and quality-of-life measures. Half of the patients had cognitive impairment (Montreal Cognitive Assessment < 26). These patients were older, had more comorbidities, and a significantly worse quality of life. There were no statistically significant associations with body mass index, malnutrition, and gastrointestinal involvement. About one-third of patients had clinically relevant malnutrition. They were older, had higher skin score, lung and esophageal involvement. They also showed significantly worse scores for dysphagia, gastrointestinal symptoms, functional disability, and quality of life. Gastrointestinal symptoms and dysphagia, but not body mass index and Montreal Cognitive Assessment, were significantly associated with depression scores, which in turn were negatively associated to quality-of-life measures. With regression analysis, cognitive impairment was predicted only by age, whereas malnutrition was significantly associated with age, dysphagia, and modified Rodnan skin scores. In this study, we showed that cognitive impairment and malnutrition are not directly linked but are both independently associated with greater functional disability and worse quality of life of patients with systemic sclerosis. Early recognition of these comorbidities is therefore pivotal to better address the chronic needs of patients affected by this disease.","PeriodicalId":17036,"journal":{"name":"Journal of Scleroderma and Related Disorders","volume":null,"pages":null},"PeriodicalIF":2.0,"publicationDate":"2024-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139686699","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":"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":null,"pages":null},"PeriodicalIF":2.0,"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":"Recurrent episodes of Takotsubo cardiomyopathy in systemic sclerosis.","authors":"Yumiko L Vreeburg, Daniel S Knight, John G Coghlan, Voon H Ong, Christopher P Denton","doi":"10.1177/23971983231188824","DOIUrl":"10.1177/23971983231188824","url":null,"abstract":"<p><p>Systemic sclerosis is an autoimmune disease characterized by fibrosis and small vessel vasculopathy, which affects various organ systems, such as the heart. Takotsubo cardiomyopathy is a transient cardiomyopathy in reaction to an emotional or physical trigger. There may be clinical and pathogenetic overlap between Takotsubo cardiomyopathy and primary systemic sclerosis heart disease, and some patients with systemic sclerosis have been diagnosed with recurrent Takotsubo cardiomyopathy. Our large systemic sclerosis clinical cohort was reviewed to identify cases diagnosed with Takotsubo cardiomyopathy. The clinical features, laboratory and imaging results were reviewed and evaluated to perform a comparison between cases. We identified five patients with systemic sclerosis, all female (age 68.6 ± 5.7 years), who were diagnosed with Takotsubo cardiomyopathy. Two of these patients had recurrent episodes: one case with a history of multiple episodes and the other with one recurrence. Typical features included repolarization abnormalities on the electrocardiogram and transient left ventricular dysfunction observed using echocardiography or cardiac magnetic resonance imaging. Our findings build upon previous reports and observations that systemic sclerosis may cause Takotsubo cardiomyopathy. To our knowledge, this is the largest case series of Takotsubo syndrome in patients with systemic sclerosis. This association may provide novel insights into the aetiopathogenesis of Takotsubo cardiomyopathy as part of primary systemic sclerosis heart involvement.</p>","PeriodicalId":17036,"journal":{"name":"Journal of Scleroderma and Related Disorders","volume":null,"pages":null},"PeriodicalIF":2.0,"publicationDate":"2024-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10848928/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"43924728","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":"Intensive receptor blockade and plasma exchange to treat refractory scleroderma renal crisis in patients with agonistic autoantibodies targeting the angiotensin II type 1 and endothelin-1 type A receptors.","authors":"Björn Hegner, Julia Callaghan, Ralf Schindler, Harald Heidecke, Gabriela Riemekasten, Aurélie Philippe, Rusan Catar","doi":"10.1177/23971983231168193","DOIUrl":"10.1177/23971983231168193","url":null,"abstract":"<p><p>Scleroderma renal crisis is a rare complication of systemic sclerosis characterized by a rapid decline in kidney function due to acute renal vascular injury. Recently, activating autoantibodies targeting the angiotensin II type 1 receptor and the endothelin-1 type A receptor have been implicated in the pathophysiology of scleroderma renal crisis by sensitizing the angiotensin II type 1 receptor and endothelin-1 type A receptor in renal resistance arteries to their natural ligands. Here, we describe a cohort of 10 patients with scleroderma renal crisis refractory to standard treatment, including blockade of the renin-angiotensin system. Multimodal therapy was initiated, targeting at the removal of anti-angiotensin II type 1 receptor and anti-endothelin-1 type A receptor autoantibodies by plasma exchange and the reduction of vasoconstrictive activity. Further treatment options included angiotensin II type 1 receptor and endothelin-1 type A receptor blockade, iloprost, intravenous immunoglobulins, and immunosuppression. Six patients were hypertensive. On kidney biopsy, concentric intimal sclerosis was present in all patients, whereas acute vascular injury was evident in eight. Levels of anti-angiotensin II type 1 receptor and anti-endothelin-1 type A receptor autoantibodies were significantly reduced by multimodal treatment. Kidney function improved in three patients with histological signs of severe acute renal vascular damage. This report demonstrates that intensive multimodal therapy taking account of potentially pathogenic anti-angiotensin II type 1 receptor and anti-endothelin-1 type A receptor autoantibodies in concert with other vasodilatory interventions provides a salvage option for patients with refractory scleroderma renal crisis.</p>","PeriodicalId":17036,"journal":{"name":"Journal of Scleroderma and Related Disorders","volume":null,"pages":null},"PeriodicalIF":2.0,"publicationDate":"2024-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10848926/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"45758023","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":"Clinical and ultrasonogrpahic evaluation of efficacy and safety of intralesional injection of autologous platelet-rich plasma in morphea: A comparative case series","authors":"E. Elnaquip, M. Makki, Mofreh Mansour, A. Moshrif","doi":"10.1177/23971983231222664","DOIUrl":"https://doi.org/10.1177/23971983231222664","url":null,"abstract":"To evaluate the efficacy and safety of platelet-rich plasma to restore skin changes in morphea by ultrasound and Localized Scleroderma Cutaneous Assessment Tool. Nine morphea patients (21 lesions) were diagnosed clinically and by histopathology. Intradermal platelet-rich plasma was injected into morphea lesion once weekly for 12 sessions. The disease severity and damage were evaluated at baseline, after the last session (3 months later), and at 6 months follow-up using the LoSCAT and a high-resolution ultrasound. The healthy corresponding side was considered as a control. The Localized Scleroderma Cutaneous Assessment Tool score showed a significant improvement starting from 13 ± 7.28 up to 7.33 ± 6.82 after the therapeutic endpoint, reaching to 6.44 ± 7.09 after 6 months of follow-up with p value = 0.008 and 0.014, respectively. There was a significant positive correlation between the duration of the lesion and the improvement assessed by the ultrasound, with p value = 0.01. Regarding adverse effects, all patients reported having pain during platelet-rich plasma injection; transient edema of the face was reported by four patients (45%), and only two patients showed transient erythema. Autologous platelet-rich plasma is a safe technique with great aesthetic outcomes for filling up the contour defects and correcting both hyper and hypopigmentation, in addition to softening the indurated lesions.","PeriodicalId":17036,"journal":{"name":"Journal of Scleroderma and Related Disorders","volume":null,"pages":null},"PeriodicalIF":2.0,"publicationDate":"2024-01-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139605887","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":"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":null,"pages":null},"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}