Journal of Scleroderma and Related Disorders最新文献

{"title":"A rare case of scleredema associated with breast cancer.","authors":"Jenifer Centeno Gavica, Rupak Thapa","doi":"10.1177/23971983251370102","DOIUrl":"10.1177/23971983251370102","url":null,"abstract":"<p><p>SclerEDEMA is a rare condition marked by diffuse skin thickening, often confused with sclerODERMA. It is linked with infections, malignancies, and diabetes. Although scleredema is associated with hematologic malignancies like multiple myeloma, it has not been reported with breast cancer, as suspected in this patient. We present a case of a patient diagnosed with scleredema, confirmed through skin biopsy. Subsequent cancer screenings identified a 6 mm breast nodule, which was confirmed as grade 3 ductal carcinoma. This newfound association between scleredema and breast cancer highlights the importance of vigilant health maintenance and regular cancer screenings in these patients.</p>","PeriodicalId":17036,"journal":{"name":"Journal of Scleroderma and Related Disorders","volume":" ","pages":"NP1-NP4"},"PeriodicalIF":1.5,"publicationDate":"2025-08-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12398460/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144958656","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 associations of worsening physical function as measured by HAQ-DI scores in systemic sclerosis.","authors":"Jessica L Fairley, Dylan Hansen, Susanna Proudman, Joanne Sahhar, Gene-Siew Ngian, Jennifer Walker, Diane Apostolopoulos, Lauren V Host, Wendy Stevens, Mandana Nikpour, Laura Ross","doi":"10.1177/23971983251360883","DOIUrl":"10.1177/23971983251360883","url":null,"abstract":"<p><strong>Background: </strong>Functional disability is a major concern for individuals with systemic sclerosis (SSc). The Health Assessment Questionnaire-Disability Index (HAQ-DI) measures the ability to perform activities of daily living, with higher scores indicating poorer function.</p><p><strong>Objective: </strong>To define the frequency and clinical associations of minimum clinically important difference (MCID) change in HAQ-DI scores in SSc.</p><p><strong>Methods: </strong>Australian Scleroderma Cohort Study participants with two or more HAQ-DI scores ⩽ 2 visits apart were included. Generalised estimating equations were used to model the correlates of the MCID improvement (-0.125 points) and MCID worsening (+0.14 points) of HAQ-DI scores. Subgroup analysis in those with incident (⩽5 years SSc duration at recruitment) and diffuse SSc (dcSSc) were performed.</p><p><strong>Results: </strong>Of 1117 participants, 712 (64%) recorded worsening of HAQ-DI scores. Of 827 participants with baseline HAQ-DI ⩾ 0.125 units, 585 (71%) had recorded improvement. Across 3229 study visits, older age (odds ratio (OR) 1.1, 95% confidence interval (CI) 1.1-1.1, <i>p</i> < 0.01), higher skin score (OR 1.1, 95% CI 1.0-1.2, <i>p</i> = 0.01), digital ulcers (OR 1.3, 95% CI 1.0-1.5, <i>p</i> = 0.02), raised C-reactive protein (CRP; OR 1.3, 95% CI 1.1-1.6, <i>p</i> < 0.01) and patient-reported worsening Raynaud's phenomenon (RP; OR 1.2, 95% CI 1.0-1.4, <i>p</i> = 0.04) and dyspnoea (OR 1.3, 95% CI 1.1-1.6, <i>p</i> < 0.01) were associated with worsening of HAQ-DI scores. In those with incident SSc, raised CRP and patient-reported worsening RP and dyspnoea were associated with worsening of HAQ-DI scores. In dcSSc, only raised CRP was associated with worsening HAQ-DI scores, while in lcSSc higher baseline HAQ-DI score, older age, higher skin score, proximal weakness and worsening dyspnoea were associated with worsening scores. Only higher baseline HAQ-DI score was associated with improvement in HAQ-DI scores in the overall cohort (OR 1.3, 95% CI 1.2-1.5, <i>p</i> < 0.01).</p><p><strong>Conclusions: </strong>Two-thirds of a large SSc cohort demonstrated significant change in physical function. Worsening symptom burden and elevated CRP were important determinants of worsening function.</p>","PeriodicalId":17036,"journal":{"name":"Journal of Scleroderma and Related Disorders","volume":" ","pages":"545-555"},"PeriodicalIF":1.5,"publicationDate":"2025-08-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12367724/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144958669","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":"Treatment recommendations for the systemic pharmacological treatment of systemic sclerosis digital ulcers: Results from the World Scleroderma Foundation Ad Hoc Committee.","authors":"Nancy Maltez, Laura Ross, Murray Baron, Alessia Alunno, Corrado Campochiaro, Yossra A Suliman, Jan W Schoones, Yannick Allanore, Francesco Del Galdo, Christopher P Denton, Oliver Distler, Tracy Frech, Daniel E Furst, Dilia Giuggioli, Dinesh Khanna, Thomas Krieg, Masataka Kuwana, Marco Matucci-Cerinic, Pia Moinzadeh, Janet Pope, Lorinda Chung, Michael Hughes","doi":"10.1177/23971983251340559","DOIUrl":"10.1177/23971983251340559","url":null,"abstract":"<p><strong>Introduction: </strong>Digital ulcers are an important disease manifestation of systemic sclerosis and are associated with significant morbidity. As such, there is an urgent need for the development of evidence-based recommendations to guide clinicians in the treatment of digital ulcers.</p><p><strong>Methods: </strong>A steering committee of international experts was established. A systematic review of the literature pertaining to the use of pharmacologic treatments in the management of digital ulcers was performed to inform the development of treatment recommendations for systemic sclerosis digital ulcers. Consensus methodology was used to develop the final treatment recommendations.</p><p><strong>Results: </strong>The World Scleroderma Foundation committee agreed on 8 overarching treatment principles and 10 pharmacologic treatment recommendations for the management of systemic sclerosis digital ulcers. Phosphodiesterase 5 inhibitors and intravenous iloprost were recommended for the management of acute digital ulcers. Bosentan was recommended for prevention of digital ulcers.</p><p><strong>Conclusion: </strong>This study has yielded pragmatic treatment recommendations to direct treatment decisions for the management of systemic sclerosis digital ulcers. In addition, results have highlighted areas in need of future research in order to improve patient outcomes.</p>","PeriodicalId":17036,"journal":{"name":"Journal of Scleroderma and Related Disorders","volume":" ","pages":"267-275"},"PeriodicalIF":1.5,"publicationDate":"2025-08-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12367710/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144957890","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":"Evaluation of the type D personality and its association with cardiovascular risk and disease-related parameters in patients with systemic sclerosis.","authors":"Ayşegül Yetişir, Volkan Deniz, Gizem Varkal, İpek Türk, Aylin Sariyildiz","doi":"10.1177/23971983251362595","DOIUrl":"10.1177/23971983251362595","url":null,"abstract":"<p><strong>Objective: </strong>The objective of the study was to assess the type D personality, social inhibition, and negative affectivity and their association with cardiovascular risk and disease-related parameters in patients with systemic sclerosis.</p><p><strong>Materials and methods: </strong>This cross-sectional study included patients with systemic sclerosis (n = 84) and controls (n = 74). We recorded the sociodemographic parameters, blood pressure (mmHg), and laboratory parameters (mg/dL) of the study groups, as well as disease-related parameters in patients with systemic sclerosis, along with the modified Rodnan skin score. The type D Scale-14 for type D personality (social inhibition and negative affectivity) and the Framingham risk score for 10-year cardiovascular disease risk to the study groups were administered.</p><p><strong>Results: </strong>The mean age, type D Scale-14 total score, Framingham risk score, and median total modified Rodnan skin score were 55.7 ± 11.6 years, 28.2 ± 13.6, 9.1± 8.1, and 17.5 (13), respectively, in patients with systemic sclerosis. The frequency of type D personality, social inhibition, and negative affectivity was higher in patients than in controls, at 59.5% vs 14.9%, 65.5% vs 23%, and 78.6% vs 32.4%, respectively (p < 0.001 for all). The median Framingham risk score was statistically similar for patients, controls, and patients with and without type D personality (p > 0.05 for all). Compared to those without social inhibition, patients with social inhibition had higher total modified Rodnan skin scores (p = 0.011). Social inhibition exhibited a weakly positive correlation with the total modified Rodnan skin score (Spearman's ρ = 0.223), yet regression analysis did not achieve significance.</p><p><strong>Conclusions: </strong>More than half of patients with systemic sclerosis display type D personality traits. Patients with serious skin fibrosis experience more social inhibition, so it is important to provide psychological and social support to them.</p>","PeriodicalId":17036,"journal":{"name":"Journal of Scleroderma and Related Disorders","volume":" ","pages":"633-639"},"PeriodicalIF":1.5,"publicationDate":"2025-08-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12334412/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144817031","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":"Esophageal dysmotility in systemic sclerosis: Relationship with extra-gastrointestinal manifestations and complementary utility of thoracic imaging.","authors":"Inês Santos, Carlos Marques-Gomes, Mariana Diz-Lopes, Georgina Terroso, Lúcia Costa, Raquel Miriam Ferreira","doi":"10.1177/23971983251362586","DOIUrl":"10.1177/23971983251362586","url":null,"abstract":"<p><strong>Introduction: </strong>The relationship between manometric changes and esophageal dilation on chest high-resolution computed tomography is well established in systemic sclerosis, but its association with extra-esophageal manifestations is inconsistent. This study aims to characterize manometric findings in systemic sclerosis patients and to determine potential associations with esophageal body dysmotility.</p><p><strong>Methods: </strong>Retrospective single-center study including adult systemic sclerosis patients who underwent conventional or high-resolution esophageal manometry. Demographic and clinical data were collected. Associations between esophageal motility and disease duration, immunologic profile, cutaneous and pulmonary involvement, as well as endoscopic or tomographic esophageal alterations were evaluated.</p><p><strong>Results: </strong>A total of 76 patients were included. Conventional manometry was performed in 60 (78.9%), with aperistalsis observed in 23 (38.3%) and a normotonic lower esophageal sphincter in 45 (75.0%). Sixteen patients (21.1%) underwent high-resolution esophageal manometry, showing normal motility in 9 (56.3%), normotonic lower esophageal sphincter in 9 (56.3%), and hypotonic lower esophageal sphincter in 7 (43.8%). Overall, 46 patients (60.5%) had esophageal body dysmotility and 54 (71.1%) had normotonic lower esophageal sphincter. Most patients (84.2%) had limited cutaneous disease. Median disease duration was 5 years (interquartile range = 11) with mean age 54.1 ± 12.4 years. Seventy-one patients (93.4%) were females. Potential associations with manometric esophageal involvement were compared between patients with normal motility and dysmotility. Esophageal dilation on chest high-resolution computed tomography was more frequent among those with dysmotility (p = 0.005). No significant differences were found regarding disease duration, immunologic profile, modified Rodnan skin score, esophagitis, Barrett's esophagus, interstitial lung disease, forced vital capacity, or single-breath carbon monoxide diffusing capacity.</p><p><strong>Discussion: </strong>Esophageal involvement was frequent in our sample, although the lower esophageal sphincter was more commonly spared. An association between esophageal dysmotility and its dilation on chest high-resolution computed tomography was found, highlighting the utility of chest high-resolution computed tomography for identification of upper gastrointestinal involvement in systemic sclerosis. No association was found between manometric changes and extra-esophageal manifestations.</p>","PeriodicalId":17036,"journal":{"name":"Journal of Scleroderma and Related Disorders","volume":" ","pages":"452-457"},"PeriodicalIF":1.5,"publicationDate":"2025-08-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12334408/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144817030","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 serum levels of specific autoantibodies in systemic sclerosis predict a more severe skin involvement.","authors":"Hannah Dengler, Maya Vonow-Eisenring, Mike Oliver Becker, Rucsandra Dobrota, Carina Mihai, Sinziana Muraru, Anna-Maria Hoffmann-Vold, Oliver Distler, Cosimo Bruni, Muriel Elhai","doi":"10.1177/23971983251357991","DOIUrl":"10.1177/23971983251357991","url":null,"abstract":"<p><strong>Background: </strong>Systemic sclerosis is a severe autoimmune disease characterized by fibrosis of the skin and internal organs. Systemic sclerosis is associated with the presence of three specific autoantibodies: anti-topoisomerase I, anti-centromere, and anti-RNA polymerase III autoantibodies, which have also been identified as prognostic factors. However, it remains unknown whether the prognosis also varies based on their serum levels.</p><p><strong>Objectives: </strong>We aimed to assess the value of serum levels of systemic sclerosis-specific autoantibodies as biomarkers of disease severity and progression in systemic sclerosis.</p><p><strong>Design: </strong>We conducted a post hoc longitudinal analysis of data of systemic sclerosis patients included in the Zurich EUSTAR cohort, who were positive for at least one of the three systemic sclerosis-specific autoantibodies.</p><p><strong>Methods: </strong>The association between the levels of systemic sclerosis-specific autoantibodies and disease severity at baseline and during the follow-up was assessed by univariable and multivariable logistic and linear regressions.</p><p><strong>Results: </strong>The serum levels of anti-topoisomerase I autoantibodies [β = 0.032 (95% confidence interval = 0.014 to 0.049), p < 0.001], anti-centromere [β = 0.002 (95% confidence interval = 0.001 to 0.003), p < 0.001], and anti-RNA polymerase III autoantibodies [β = 0.143 (95% confidence interval = 0.066 to 0.220), p < 0.001] were associated with the modified Rodnan Skin Score in univariable analysis at baseline. For anti-centromere [β = 0.002 (95% confidence interval = 0.001 to 0.003), p < 0.001] and anti-RNA polymerase III autoantibodies [β = 0.135 (95% confidence interval = 0.053 to 0.217), p = 0.002], this association also remained significant in multivariable analysis. In the longitudinal analysis, the levels of the three systemic sclerosis-specific autoantibodies did not predict changes in mRSS over 1 year.</p><p><strong>Conclusion: </strong>Increased serum levels of all three autoantibodies predicted a more severe skin fibrosis. The results underscore the relevance of measuring the levels of systemic sclerosis-specific autoantibodies to enhance risk stratification in systemic sclerosis, with particular focus on skin involvement.</p>","PeriodicalId":17036,"journal":{"name":"Journal of Scleroderma and Related Disorders","volume":" ","pages":"342-349"},"PeriodicalIF":1.5,"publicationDate":"2025-07-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12307331/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144760458","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":"Effects of a 12-week physiotherapist-supervised home-based rehabilitation program on the functional exercise capacity in people with systemic sclerosis.","authors":"Samantha Gomes de Alegria, Matheus Mello da Silva, Beatriz Luiza Pinheiro Alves Azevedo, Jéssica Gabriela Messias Oliveira, Iasmim Maria Pereira Pinto Fonseca, Isabelle da Nóbrega Ferreira, Patrícia Frascari Litrento, Cláudia Henrique da Costa, Agnaldo José Lopes","doi":"10.1177/23971983251360912","DOIUrl":"10.1177/23971983251360912","url":null,"abstract":"&lt;p&gt;&lt;strong&gt;Objective: &lt;/strong&gt;There is still a wide variation in the rehabilitation programs developed for systemic sclerosis, which makes it urgent to develop feasible and easy-to-use protocols using tools capable of measuring the response to rehabilitation. The Glittre-ADL test assesses functional exercise capacity using activities of daily living and is useful due to the hands involvement. This study aimed to evaluate the effects of a physiotherapist-supervised home-based rehabilitation program on functional exercise capacity in women with systemic sclerosis using the Glittre-ADL test. A secondary aim was to evaluate the association between the Glittre-ADL test and handgrip strength, hand function, physical function, quality of life, and lung function.&lt;/p&gt;&lt;p&gt;&lt;strong&gt;Methods: &lt;/strong&gt;This is a quasi-experimental study in which 33 women with systemic sclerosis underwent a physiotherapist-supervised home-based rehabilitation program for 12 weeks. The following pre- and post-physiotherapist-supervised home-based rehabilitation program assessments were performed: Glittre-ADL test, Health Assessment Questionnaire Disability Index, Cochin Hand Functional Scale, Short Form 36 Health Survey, handgrip strength, and spirometry.&lt;/p&gt;&lt;p&gt;&lt;strong&gt;Results: &lt;/strong&gt;There was a significant increase in Glittre-ADL test time after physiotherapist-supervised home-based rehabilitation program (&lt;i&gt;p&lt;/i&gt; = 0.036). There was a significant decrease in manual time of the Glittre-ADL test between pre- and post-physiotherapist-supervised home-based rehabilitation program (&lt;i&gt;p&lt;/i&gt; = 0.001). For Health Assessment Questionnaire Disability Index, there was a significant decrease from 1.13 (0.5-1.8) points before physiotherapist-supervised home-based rehabilitation program to 1.0 (0.4-1.3) points after physiotherapist-supervised home-based rehabilitation program (&lt;i&gt;p&lt;/i&gt; = 0.004). For the Cochin Hand Functional Scale, there was a decrease from 14 (5-35) points before physiotherapist-supervised home-based rehabilitation program to 7 (3-21) points after physiotherapist-supervised home-based rehabilitation program (&lt;i&gt;p&lt;/i&gt; = 0.0002). There was a significant increase in handgrip strength between pre- and post-PHPR (&lt;i&gt;p&lt;/i&gt; = 0.008). The delta for Glittre-ADL test time (%predicted) was significantly correlated with the delta for handgrip strength (&lt;i&gt;r&lt;sub&gt;s&lt;/sub&gt;&lt;/i&gt;  = -0.360, &lt;i&gt;p&lt;/i&gt; = 0.039).&lt;/p&gt;&lt;p&gt;&lt;strong&gt;Conclusion: &lt;/strong&gt;After physiotherapist-supervised home-based rehabilitation program, women with systemic sclerosis require less time to complete the Glittre-ADL test tasks. Physiotherapist-supervised home-based rehabilitation program improves manual function, physical function, handgrip strength, lung function, and quality of life. There is a relationship between Glittre-ADL test time and handgrip strength. These results suggest that a 12-week physiotherapist-supervised home-based rehabilitation program could be a beneficial non-pharmacological therapy that reduces","PeriodicalId":17036,"journal":{"name":"Journal of Scleroderma and Related Disorders","volume":" ","pages":"576-587"},"PeriodicalIF":1.5,"publicationDate":"2025-07-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12307330/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144760457","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":"Transcriptomic profiling of scleroderma monocytes reveals links with cardiovascular complications, implicating Notch and interferon pathways.","authors":"Mehmed T Dinc, Fatima El-Adili, Justin K Lui, Subedi Kripesh, Michael York, Marcin Trojanowski, Giovanni Ligresti, Robert Lafyatis, Maria Trojanowska, Andreea M Bujor","doi":"10.1177/23971983251356123","DOIUrl":"10.1177/23971983251356123","url":null,"abstract":"<p><strong>Objectives: </strong>Recent research has highlighted the critical role of monocytes and macrophages in driving both inflammatory and fibrotic processes in systemic sclerosis. This study seeks to elucidate the gene expression profiles of systemic sclerosis monocytes and their potential links to disease complications, with the ultimate goal of uncovering novel therapeutic targets.</p><p><strong>Methods: </strong>A total of 48 systemic sclerosis patients and 15 controls were recruited and monocytes were isolated using CD14+ magnetic beads. Total RNA was extracted and bulk RNA-seq analysis was performed. Differential gene expression followed by unsupervised hierarchical clustering and pathway analysis was conducted, and correlations with clinical features were analyzed. Interferon signature score (IFN6) was calculated using the log transformed values of six genes (IFIT3, IFIT2, MX1, IFIH1, STAT2, and NCF1).</p><p><strong>Results: </strong>We identified four distinct patient subgroups, relative to normal, two with inflammatory and two with non-inflammatory gene profiles. The inflammatory subgroups exhibited high expression of interferon-related genes and included all systemic sclerosis patients with pulmonary hypertension and most with cardiac involvement. In these patients, IFN6 was markedly elevated and showed a significant correlation with global longitudinal strain (GLS; r = -0.5, p = 0.006), a key indicator of cardiac function. Furthermore, pathway analysis identified an enrichment of the Notch signaling pathway among genes whose overexpression correlated with impaired global longitudinal strain.</p><p><strong>Conclusion: </strong>These findings unveil a potential new mechanistic link between interferon activity, Notch signaling, and cardiac complications in systemic sclerosis, offering new insights into disease pathogenesis and potential therapeutic targets.</p>","PeriodicalId":17036,"journal":{"name":"Journal of Scleroderma and Related Disorders","volume":" ","pages":"472-482"},"PeriodicalIF":1.5,"publicationDate":"2025-07-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12301231/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144742326","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":"Sexual dysfunction and perceptions of rheumatologist engagement on this issue in patients with systemic sclerosis.","authors":"Liza M Morales, Robert F Spiera, Jessica K Gordon, Deanna Jannat-Khah, Kimberly S Lakin","doi":"10.1177/23971983251357732","DOIUrl":"10.1177/23971983251357732","url":null,"abstract":"<p><strong>Background: </strong>The purpose of this study is to define the prevalence of, and risk factors associated with sexual dysfunction among individuals with systemic sclerosis, evaluate the frequency of sexual health discussions between patients and rheumatologists, identify patient-perceived barriers to these discussions, and assess screening tools for detecting systemic sclerosis-associated sexual dysfunction.</p><p><strong>Methods: </strong>The Sexual Function in SSc Questionnaire anonymously surveys individuals with self-reported SSc about their sexual function and sexual health discussions with their rheumatologist. Participants were asked to complete the Sexual Function in SSc Questionnaire and the Female Sexual Function Index or International Index of Erectile Function Questionnaire for females and males, respectively. Comparisons were made between those with versus without self-reported sexual dysfunction (measured by the Sexual Function in SSc Questionnaire) using Fisher's exact and t-test, as appropriate. Logistic regression was used to identify predictors of sexual dysfunction. Agreement to statements about barriers to sexual health conversations were compared between those who reported feeling satisfied versus not satisfied with sexual health discussions using Fisher's exact test.</p><p><strong>Results: </strong>A total of 41 participants completed the Sexual Function in SSc Questionnaire, and 30 (73%) also completed the Female Sexual Function Index or International Index of Erectile Function. Sexual dysfunction was self-reported in 26 (63%) participants. Age, sex, SSc subtype, and time since systemic sclerosis diagnosis were not significant predictors of sexual dysfunction. Twenty-five percent of participants who self-reported sexual dysfunction did not meet the criteria based on the Female Sexual Function Index/International Index of Erectile Function scored assessment. Thirty-six of 41 participants (88%) reported they had never discussed sexual health with their rheumatologist. Among them, 23 (64%) self-reported sexual dysfunction.</p><p><strong>Conclusion: </strong>Sexual dysfunction is common in systemic sclerosis, yet most participants had never discussed sexual health with their rheumatologist. Discrepancies between self-reported dysfunction and validated questionnaire scores suggest that existing tools may not capture systemic sclerosis-specific concerns. Future studies developing better screening methods and promoting education related to sexual health in SSc care are needed.</p>","PeriodicalId":17036,"journal":{"name":"Journal of Scleroderma and Related Disorders","volume":" ","pages":"502-509"},"PeriodicalIF":1.5,"publicationDate":"2025-07-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12289617/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144731935","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":"Pharmacological management of pain in digital ulcers of systemic sclerosis: A PRISMA-driven systematic review.","authors":"Inês Almeida, Anita Dourado, Luís Agualusa","doi":"10.1177/23971983251352340","DOIUrl":"10.1177/23971983251352340","url":null,"abstract":"<p><p>Digital ulcers represent a severe manifestation of digital vasculopathy in systemic sclerosis, occasionally with the contribution of microtrauma and calcinosis. These ulcers are often challenging to heal and extremely painful. Regarding analgesic approaches, there is a lack of consensus on best practices, leading to physician experience-guided approaches rather than standardized protocols. To assess the published evidence on the effectiveness of oral and topical analgesics and local anaesthetics for pain reduction in digital ulcer of systemic sclerosis, we conducted a PRISMA-driven systematic review. A search in PubMed and Cochrane databases was performed using the keyword search terms 'digital ulcers', 'finger ulcers', 'systemic sclerosis', 'scleroderma', 'pain management', 'analgesics', 'anaesthetics' and 'painkillers', with the Boolean terms 'AND' and 'OR'. Original studies and case reports were considered for inclusion. Results were screened by title, abstract and full-text. We identified a total of 26 results, of which 5 original papers were included: 2 prospective observational studies, 1 prospective case series, 1 retrospective case series and 1 case report. The evaluated pharmacological strategies were digital nerve block with lidocaine and mepivacaine (one study), oral extended-release oxycodone (two studies), local lidocaine (one study), local lidocaine and prilocaine, local morphine and sublingual morphine (one study). Overall, digital nerve block appeared to provide complete or near-complete pain relief. Oral and topical strategies resulted in reduced pain intensity, although levels remained in the mild-to-moderate range. Concerning adverse outcomes, all strategies were generally well tolerated, with only mild effects reported across studies. Despite limitations, the available evidence suggests that the evaluated analgesics and anaesthetics may provide pain relief for digital ulcer in patients with systemic sclerosis. Future larger-scale studies employing standardized protocols are necessary to further assess the efficacy, optimal dosing and long-term outcomes of these interventions.</p>","PeriodicalId":17036,"journal":{"name":"Journal of Scleroderma and Related Disorders","volume":" ","pages":"200-211"},"PeriodicalIF":1.5,"publicationDate":"2025-07-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12289615/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144731934","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}