Journal of Scleroderma and Related Disorders最新文献

{"title":"Occupation as a gendered-role and outcome in systemic sclerosis.","authors":"Fatema Alkhamees,&nbsp;Oriana Hoi Yun Yu,&nbsp;Mianbo Wang,&nbsp;Marie Hudson","doi":"10.1177/23971983221143599","DOIUrl":"https://doi.org/10.1177/23971983221143599","url":null,"abstract":"<p><strong>Objective: </strong>Sex and gender are of growing scientific interest in disease onset and course. While sex differences have been shown to exist in systemic sclerosis, there is a paucity of data on gender. Our objective was to examine the association between occupation, a gender-related role and outcomes in systemic sclerosis.</p><p><strong>Methods: </strong>An occupation score ranging from 0 to 100, with lower scores representing occupations traditionally held by men and higher scores traditionally held by women, was constructed using the National Occupational Classification 2016 and data from Statistics Canada. Subjects in the Canadian Scleroderma Research Group registry were assigned an occupation score based on self-reported occupation. Multivariate models, adjusted for sex, age, smoking and education were used to estimate the independent effect of occupation score on systemic sclerosis outcomes.</p><p><strong>Results: </strong>We included 1104 subjects, of which 961 were females (87%) and 143 (13%) males. There were differences between females versus males: disease duration (9.9 vs 7.6 years, <i>p</i> = 0.002), diffuse disease (35% vs 54%, <i>p</i> < 0.001), interstitial lung disease (28% vs 37%, <i>p</i> = 0.021) and pulmonary hypertension (10% vs 4%, <i>p</i> = 0.033), but not pain, response to treatment and mortality. The median occupation scores differed between females and males (84.3 (interquartile range 56.8, 89.4) vs 24.9 (4.3, 54.1), <i>p</i> < 0.001). The Spearman correlation between sex and occupation score was 0.44, indicating a weak correlation. In adjusted analyses, occupation score was not an independent predictor of disease subset (diffuse vs limited), interstitial lung disease, pulmonary hypertension, pain, response to treatment or mortality.</p><p><strong>Conclusion: </strong>We did not find independent associations between an occupation score, a gender-related role and outcomes in systemic sclerosis. These results should be interpreted with caution as occupation may be a poor measure of gender. Future research using a validated measure of gender will be needed to generate robust data on the effect of gender in systemic sclerosis.</p>","PeriodicalId":17036,"journal":{"name":"Journal of Scleroderma and Related Disorders","volume":null,"pages":null},"PeriodicalIF":2.0,"publicationDate":"2023-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/4c/02/10.1177_23971983221143599.PMC10242688.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9599243","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":"Relapsing polychondritis in systemic sclerosis: A rare vasculitic mimic.","authors":"Carolina Teles,&nbsp;Chiranthi Kongala Liyanage,&nbsp;Geoffrey Chow,&nbsp;Christopher P Denton,&nbsp;Voon Ong","doi":"10.1177/23971983221141599","DOIUrl":"https://doi.org/10.1177/23971983221141599","url":null,"abstract":"<p><strong>Introduction: </strong>Relapsing polychondritis is a rare, immune-mediated disease characterised by inflammation of cartilaginous structures. Auricular chondritis, sparing the fatty lobule, is the most typical feature, followed by nose and laryngotracheal involvement. Albeit rare, neurologic involvement is reported with relapsing polychondritis. Cranial nerve involvement is the most frequent neurologic manifestation and is probably due to an underlying vasculitic process. Approximately one-third of relapsing polychondritis patients can overlap with other systemic diseases, including other autoimmune connective tissue diseases, but association with systemic sclerosis has very rarely been described.</p><p><strong>Case description: </strong>A 63-year-old woman presented with acute new-onset severe dysphagia, accompanied by hoarseness and preceded by pain, swelling and erythema of the left pinna, unresponsive to antibiotics. She had a history of long-standing limited cutaneous systemic sclerosis. Cranial nerve examination revealed right-sided palatal palsy, and left vocal cord palsy was found on fibreoptic nasendoscopy. Magnetic resonance imaging of the head and neck showed bilateral enhancement of an extracranial segment of the glossopharyngeal and vagus nerves. Clinical features and imaging findings were consistent with relapsing polychondritis, which successfully responded to high-dose steroids.</p><p><strong>Conclusions: </strong>This is a case of relapsing polychondritis mimicking progression of systemic sclerosis, showcasing its challenging features. It emphasises the importance of early diagnosis and prompt management with potential impact on the outcome, while highlighting the complex interplay between these two disease entities and vasculitic mechanisms, which may reflect the shared network of genetic predisposition across autoimmune rheumatic diseases.</p>","PeriodicalId":17036,"journal":{"name":"Journal of Scleroderma and Related Disorders","volume":null,"pages":null},"PeriodicalIF":2.0,"publicationDate":"2023-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10242694/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9599245","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":"COVID-19 and protection of vaccination in patients with systemic sclerosis-associated interstitial lung disease.","authors":"Stylianos Panopoulos,&nbsp;Vasilios Tzilas,&nbsp;Vasiliki-Kalliopi Bournia,&nbsp;Anastasios Karamanakos,&nbsp;Katerina Laskari,&nbsp;Demosthenes Bouros,&nbsp;Maria Tektonidou,&nbsp;Petros P Sfikakis","doi":"10.1177/23971983221143252","DOIUrl":"https://doi.org/10.1177/23971983221143252","url":null,"abstract":"<p><strong>Objectives: </strong>Data on COVID-19 in patients with interstitial lung disease are scarce and whether SARS-CoV-2 may trigger interstitial lung disease progression remains unknown. We aimed to analyze outcomes of COVID-19 in patients with systemic sclerosis-associated interstitial lung disease, including possible thoracic radiographic progression.</p><p><strong>Patients and methods: </strong>All 43 patients with systemic sclerosis-associated interstitial lung disease followed in our center (mean ± SD, 55.2 ± 11.6 years, 36 female) with confirmed SARS-CoV2 infection up to 1 September 2022 were analyzed. Individual interstitial lung disease extent on high resolution CT (HRCT) performed before (up to 3 months) and after COVID-19 (2-5 months) was compared.</p><p><strong>Results: </strong>At SARS-CoV-2 infection, 9/43 patients were unvaccinated, whereas 5, 26, and 3 had received 2, 3, or 4 doses of an mRNA vaccine, respectively. Thirty-one patients were either on monotherapy with immunosuppressives (mycophenolate, <i>n</i> = 7; cyclophosphamide, <i>n</i> = 2; methotrexate, <i>n</i> = 10; tocilizumab, <i>n</i> = 7; rituximab, <i>n</i> = 1; etanercept, <i>n</i> = 1), or their combinations (<i>n</i> = 3). Eight patients (20%), of whom four unvaccinated, required hospitalization for pneumonia and three (7%) died of acute respiratory failure (<i>n</i> = 2, both unvaccinated) or cardiac arrest. Lack of vaccination was the only independent predictor for hospitalization (OR = 7.98, 95% CI: 1.25-51.09) and marginally for death (OR = 32.7, 95% CI: 0.97-1110.98), regardless of the presence of diffuse systemic sclerosis, interstitial lung disease extent greater than 20% or immunosuppressive treatment. In 22 patients with available HRCT pairs (vaccinated = 20), the interstitial lung disease extent before COVID-19 (20.4%± 17.8%) remained unchanged (22.4% ± 18.5%) in all but one patient.</p><p><strong>Conclusion: </strong>SARS-CoV-2 vaccination is of outmost importance for every systemic sclerosis patient with interstitial lung disease. COVID-19 does not seem to promote progression of systemic sclerosis-associated interstitial lung disease in vaccinated patients, but further studies are warranted.</p>","PeriodicalId":17036,"journal":{"name":"Journal of Scleroderma and Related Disorders","volume":null,"pages":null},"PeriodicalIF":2.0,"publicationDate":"2023-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9755035/pdf/10.1177_23971983221143252.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9593195","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":"Gender differences in juvenile systemic sclerosis patients: Results from the international juvenile scleroderma inception cohort.","authors":"Ivan Foeldvari, Jens Klotsche, Ozgur Kasapcopur, Amra Adrovic, Maria Teresa Terreri, Ana Paula Sakamoto, Valda Stanevicha, Jordi Anton, Brian M Feldman, Flavio Sztajnbok, Raju Khubchandani, Ekaterina Alexeeva, Maria Katsicas, Sujata Sawhney, Vanessa Smith, Simone Appenzeller, Tadej Avcin, Mikhail Kostik, Thomas Lehman, Edoardo Marrani, Dieneke Schonenberg-Meinema, Walter-Alberto Sifuentes-Giraldo, Natalia Vasquez-Canizares, Mahesh Janarthanan, Monika Moll, Dana Nemcova, Anjali Patwardhan, Maria Jose Santos, Cristina Battagliotti, Lillemor Berntson, Blanca Bica, Jürgen Brunner, Rolando Cimaz, Patricia Costa-Reis, Despina Eleftheriou, Liora Harel, Gerd Horneff, Sindhu R Johnson, Daniela Kaiser, Tilmann Kallinich, Dragana Lazarevic, Kirsten Minden, Susan Nielsen, Farzana Nuruzzaman, Siri Opsahl Hetlevik, Yosef Uziel, Nicola Helmus, Kathryn S Torok","doi":"10.1177/23971983221143244","DOIUrl":"10.1177/23971983221143244","url":null,"abstract":"<p><strong>Objective: </strong>To compare organ involvement and disease severity between male and female patients with juvenile onset systemic sclerosis.</p><p><strong>Methods: </strong>Demographics, organ involvement, laboratory evaluation, patient-reported outcomes and physician assessment variables were compared between male and female juvenile onset systemic sclerosis patients enrolled in the prospective international juvenile systemic sclerosis cohort at their baseline visit and after 12 months.</p><p><strong>Results: </strong>One hundred and seventy-five juvenile onset systemic sclerosis patients were evaluated, 142 females and 33 males. Race, age of onset, disease duration, and disease subtypes (70% diffuse cutaneous) were similar between males and females. Active digital ulceration, very low body mass index, and tendon friction rubs were significantly more frequent in males. Physician global assessment of disease severity and digital ulcer activity was significantly higher in males. Composite pulmonary involvement was also more frequent in males, though not statistically significantly. After 12 months, they are the pattern of differences changed female patients had significantly more frequent pulmonary involvement.</p><p><strong>Conclusion: </strong>In this cohort, juvenile onset systemic sclerosis had a more severe course in males at baseline and but the pattern changed after 12 months. Some differences from adult findings persisted, there is no increased signal of pulmonary arterial hypertension or heart failure in male pediatric patients. While monitoring protocols of organ involvement in juvenile onset systemic sclerosis need to be identical for males and females.</p>","PeriodicalId":17036,"journal":{"name":"Journal of Scleroderma and Related Disorders","volume":null,"pages":null},"PeriodicalIF":2.0,"publicationDate":"2023-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10242693/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9653004","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":"Scleromyxedema in an adult following Sinopharm BBIBP-CorV vaccination: An extremely rare phenomenon.","authors":"Amirhossein Parsaei, Somayeh Sadat Rezaei, Alireza Rahmani, Maryam Masoumi, Hojat Dehghanbanadaki, Somayyeh Norouzi, Mohammad Mehdi Riyahi, Reihane Tabaraii, Seyed Mohammad Hashem Montazeri","doi":"10.1177/23971983221107321","DOIUrl":"10.1177/23971983221107321","url":null,"abstract":"<p><strong>Introduction: </strong>The Sinopharm BBIBP-CorV vaccine produces a variety of cutaneous adverse effects. Scleromyxedema is a mucinous connective tissue disorder that causes skin thickness and sclerodermoid changes. According to our findings, this is the first case of scleromyxedema induced by the Sinopharm immunization.</p><p><strong>Case description: </strong>We discuss the case of a 75-year-old woman who acquired progressive thickening of the skin in her limbs and trunk after getting the Sinopharm vaccination. Examination, laboratory testing, and a biopsy were used to verify scleromyxedema diagnosis. Intravenous immunoglobulins, mycophenolate mofetil, and prednisolone were used in the treatment of the patient. The outcomes from the 4-month follow-up were reassuring.</p><p><strong>Conclusion: </strong>This study emphasizes the need of considering scleromyxedema as a connective tissue pathology in patients who have recently received Sinopharm vaccine and have similar cutaneous signs.</p>","PeriodicalId":17036,"journal":{"name":"Journal of Scleroderma and Related Disorders","volume":null,"pages":null},"PeriodicalIF":2.0,"publicationDate":"2023-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10242695/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9971678","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":"A purple plaque in a patient with systemic sclerosis.","authors":"Sheena Ramyead, Christopher P Denton, Catherine H Orteu, Victoria Swale, Jorge Mayor-Jerez, Emma Gardette","doi":"10.1177/23971983231152342","DOIUrl":"10.1177/23971983231152342","url":null,"abstract":"<p><p>We present the case of a 43-year old woman with anti-U3 ribonucleoprotein antibody-positive systemic sclerosis presenting with an enlarging purple plaque on the left upper arm. The skin was not sclerotic; however, there had been a cluster of long-standing telangiectases preceding the plaque. Histology and immunohistochemistry confirmed an angiosarcoma. There are five reported cases in the literature about angiosarcoma arising in the skin of patients with systemic sclerosis; however, to our knowledge, this is the first to have arisen from non-sclerotic skin. We would urge clinicians to adopt a high index of suspicion for atypical vascular tumours presenting in patients with systemic sclerosis.</p>","PeriodicalId":17036,"journal":{"name":"Journal of Scleroderma and Related Disorders","volume":null,"pages":null},"PeriodicalIF":2.0,"publicationDate":"2023-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10242689/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9955050","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":"Factors associated with life satisfaction in systemic sclerosis: Examining the moderating roles of social support and spiritual well-being.","authors":"Yen T Chen, Susan L Murphy, Daniel E Furst, Philip Clements, Suzanne Kafaja, Joel Tsevat, Vanessa Malcarne, Dinesh Khanna","doi":"10.1177/23971983221146366","DOIUrl":"10.1177/23971983221146366","url":null,"abstract":"<p><strong>Objectives: </strong>Systemic sclerosis often has a significant impact on an individual's quality of life. Life satisfaction is a subjective expression of well-being and a key component of quality of life. We examined the associations between functional limitations, social support, and spiritual well-being with life satisfaction and investigated the moderating roles of social support and spiritual well-being on the relationship between functional limitations and life satisfaction in people with systemic sclerosis.</p><p><strong>Methods: </strong>Data were drawn from the baseline University of California Los Angeles Scleroderma Quality of Life Study. Participants completed questionnaires that included demographics, depressive symptoms, functional limitations, social support, and spiritual well-being. The Satisfaction with Life Scale was used to evaluate overall life satisfaction. Data were analyzed using a hierarchical linear regression.</p><p><strong>Results: </strong>Of 206 participants (84% female, 74% White, 52% limited cutaneous subtype, 51% early disease), 38% reported being dissatisfied with their lives. Functional limitations (β = -0.19, <i>p</i> = 0.006), social support (β = 0.18, <i>p</i> = 0.006), and spiritual well-being (β = 0.40, <i>p</i> < 0.001) were associated with life satisfaction, with spiritual well-being emerging as the strongest statistical contributor. However, social support and spiritual well-being did not significantly moderate the relationship between functional limitations and life satisfaction (<i>p</i> = 0.882 and <i>p</i> = 0.339, respectively).</p><p><strong>Conclusion: </strong>Spiritual well-being is particularly important in understanding life satisfaction in people with systemic sclerosis. Future longitudinal research is needed to assess and examine spiritual well-being and its impact on life satisfaction in a larger and more diverse systemic sclerosis sample.</p>","PeriodicalId":17036,"journal":{"name":"Journal of Scleroderma and Related Disorders","volume":null,"pages":null},"PeriodicalIF":2.0,"publicationDate":"2023-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10202482/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9590893","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":"Comprehensive description of the prevalence, serological and clinical characteristics, and visceral involvement of systemic sclerosis (scleroderma) in a large cohort from the United Arab Emirates Systemic Sclerosis Registry.","authors":"Rajaie Namas, Mohamed Elarabi, Saniya Khan, Asia Mubashir, Esat Memisoglu, Mahmoud El-Kaissi, Abhay Joshi, Jeffrey Chapman, Imad Jassim, Hiba Khogali, Nada Hassan, Hani Sabbour, Khaled Saleh, Khalid A Alnaqbi, Ahmed S Zayat, Sehriban Diab, Zyiad Awir, Nehad Abu Taha, Amel Ginawi, Atheer Al Ansari, Hazem Rifaai, Zaid Alrawi, Afra Al Dhaheri, Gamal Ibrahim, Ahmed Abogamal, Waleed Al Shehhi, Jamal Teir, Tahir Khan, Maisam Musgrave, Beena Hameed, Bhavna Khan, Nagwa Mosallam, Nahla Hussien, Iman Hussein, Abeer Abdulelhamid, Ahmed Ali, Suad Hannawi, Mustafa Al Izzi, Humeira Badsha, Jamal Al Saleh","doi":"10.1177/23971983221145788","DOIUrl":"10.1177/23971983221145788","url":null,"abstract":"<p><p>Systemic sclerosis is an autoimmune condition characterized by a wide range of clinical presentations. Registries may serve to expand understanding about systemic sclerosis and aid in patient care and follow-up. The objective of this study was to analyze the prevalence of systemic sclerosis in a large cohort from the United Arab Emirates Systemic Sclerosis Registry and find the significant similarities and differences between the different subsets. All scleroderma patients in the United Arab Emirates were included in this multicenter national retrospective analysis. Data on demographics, comorbidities, serological characteristics, clinical aspects, and treatment were collected and analyzed, highlighting the most common traits identified. A total of 167 systemic scleroderma patients from diverse ethnic backgrounds were enrolled. Overall, 54.5% (91/167) of the patients were diagnosed with diffuse cutaneous systemic sclerosis, and 45.5% (76/167) with limited cutaneous systemic sclerosis. The prevalence of systemic sclerosis was 1.66 per 100,000 for the total registry and 7.78 per 100,000 for United Arab Emirates patients. Almost all patients in the diffuse cutaneous systemic sclerosis and limited cutaneous systemic sclerosis groups tested positive for the immunofluorescence antinuclear antibody. Antibodies against Scl-70 were significantly more associated with diffuse cutaneous systemic sclerosis, whereas anticentromere antibodies were significantly more associated with the limited cutaneous systemic sclerosis group (<i>p</i> < 0.001). Sclerodactyly, shortness of breath, and digital ulcers were more common in diffuse cutaneous systemic sclerosis patients compared with the limited cutaneous systemic sclerosis subtype in terms of clinical symptoms and organ involvement. Telangiectasia was much more common in the limited cutaneous systemic sclerosis group. Furthermore, diffuse cutaneous systemic sclerosis patients had more lung fibrosis (interstitial lung disease) than limited cutaneous systemic sclerosis patients (70.5% vs 45.7%), and pulmonary arterial hypertension was twice as common in limited cutaneous systemic sclerosis patients as it was in diffuse cutaneous systemic sclerosis patients. Local registries are paramount to understanding the clinical/serological characteristics of scleroderma. This study emphasizes the importance of raising disease awareness and distinguishing between the various systemic sclerosis subsets to implement patient-tailored strategies for early detection, better management, and higher quality of care.</p>","PeriodicalId":17036,"journal":{"name":"Journal of Scleroderma and Related Disorders","volume":null,"pages":null},"PeriodicalIF":2.0,"publicationDate":"2023-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10242692/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9971681","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":"Worsening premature death burden gap from systemic sclerosis in men and black persons: A US nationwide population-based study.","authors":"Ram Raj Singh,&nbsp;Devanshu R Singh,&nbsp;Eric Y Yen","doi":"10.1177/23971983221140538","DOIUrl":"https://doi.org/10.1177/23971983221140538","url":null,"abstract":"<p><strong>Objective: </strong>Male sex and black race incur poor prognosis in systemic sclerosis (SSc). There is no nationwide population-based assessment of premature SSc death burden by sex and race.</p><p><strong>Methods: </strong>This is a population-based study comprising all recorded SSc deaths across the United States. We constructed histograms depicting the number of SSc deaths for each age by sex and race, and calculated the cumulative percent death at each age and the median age of death. We determined the odds ratios for the risk of premature death from SSc by sex and race. We then calculated the percent of total SSc deaths for different age groups by sex and race from 1970 to 2015. We performed chi-square test with Yates's correction and quantified the odds ratio (OR) with 95% confidence interval (CI).</p><p><strong>Results: </strong>The median age of SSc death was 63 years in males versus 68 years in females, and 57 years in blacks versus 70 years in whites. The odds for SSc death before 65 years age was 1.8 (95% CI, 1.6-2.0) for males compared with females and 5.1 (95% CI, 4.4-6.0) for blacks compared with whites. The higher odds for premature death in males than in females was similar for both races. Differences in the proportions of premature deaths from 1970 to 2015 increased between males and females (-5% to 17%) and between blacks and whites (14% to 36%).</p><p><strong>Conclusion: </strong>Males and black persons die of SSc at younger ages. The worsening premature death burden gap between the two sexes and races over the last five decades is troublesome.</p>","PeriodicalId":17036,"journal":{"name":"Journal of Scleroderma and Related Disorders","volume":null,"pages":null},"PeriodicalIF":2.0,"publicationDate":"2023-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9896199/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9648573","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":"Percutaneous revascularization for the treatment of refractory digital ischemia in systemic sclerosis.","authors":"Lily A Romero-Karam,&nbsp;Kevin A Honan,&nbsp;Salman A Arain,&nbsp;Maureen D Mayes","doi":"10.1177/23971983221116669","DOIUrl":"https://doi.org/10.1177/23971983221116669","url":null,"abstract":"<p><strong>Objective: </strong>The objective of this study is to explore the role of adjunctive percutaneous revascularization of the hand in the management of patients with systemic sclerosis-associated refractory digital ischemia.</p><p><strong>Methods: </strong>We present our initial experience of using percutaneous upper extremity interventions to treat patients with systemic sclerosis and symptomatic Raynaud's phenomenon who presented with either refractory digital ischemia or non-healing ulcers. We discuss patient characteristics, procedural findings, and short-term clinical outcomes of these interventions.</p><p><strong>Results: </strong>We performed 14 interventions in 6 patients with non-healing digital ulcers or refractory ischemia secondary to systemic sclerosis. Angioplasty was performed at or below the wrist in conjunction with intravenous prostaglandin therapy, started prior to or immediately after the revascularization procedure. All patients experienced symptomatic relief and demonstrated accelerated wound healing. Two patients required an additional procedure to treat recurrent ischemia (without new ulceration) in the treated digit. Three of the patients underwent multiple procedures during the study period to treat new ischemic lesions or Raynaud's phenomenon symptoms, highlighting the progressive nature of the vascular occlusions in systemic sclerosis. There were no adverse events related to the interventions.</p><p><strong>Conclusions: </strong>Our retrospective analysis suggests that percutaneous revascularization in combination with vasodilator therapy in systemic sclerosis-associated digital ischemia is safe and can facilitate the healing of long-standing ulcers. Its role in the management of refractory digital ischemia in patients with systemic sclerosis should be explored further.</p>","PeriodicalId":17036,"journal":{"name":"Journal of Scleroderma and Related Disorders","volume":null,"pages":null},"PeriodicalIF":2.0,"publicationDate":"2023-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/06/e1/10.1177_23971983221116669.PMC9896190.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9648567","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}