Journal of Scleroderma and Related Disorders最新文献

{"title":"Nailfold video capillaroscopy in patients with suspected systemic sclerosis: a comparison of one-finger method versus eight-finger method.","authors":"Lars Doornewaard, Dieneke Haverkort, Brigit E Kersten, Madelon C Vonk","doi":"10.1136/jsrd-2026-000015","DOIUrl":"10.1136/jsrd-2026-000015","url":null,"abstract":"<p><strong>Objective: </strong>We evaluated whether nailfold video capillaroscopy (NVC) of one finger is an adequate alternative to detect scleroderma pattern in patients suspected of systemic sclerosis (SSc) compared with the usual eight-finger method.</p><p><strong>Methods: </strong>In this study, NVC images of 87 patients suspected of SSc were analysed for capillary density, capillary dimension, capillary morphology, the presence of microhaemorrhages and pattern. Each individual finger was compared with the eight-finger method using Cohen's kappa agreement. Finally, a two-finger and four-finger combination was analysed using the fingers with highest agreement.</p><p><strong>Results: </strong>After inclusion of 76 patients, NVC of a single finger showed weak to moderate agreement with the eight-finger method in detecting a scleroderma pattern with a Cohen's kappa value ranging from K=0.23 (right digit V) to K=0.47 (right digit IV). NVC of digit IV of both hands provided moderate agreement (K=0.63), whereas a four-finger combination of digit III and IV of both hands provided strong agreement (K=0.82). For detection of giant capillaries and capillary density <7 /mm, the four-finger method provided K=0.82 and K=0.52, respectively. The digit IV of the right hand provided the highest agreement among all the individual fingers for NVC pattern, giant capillaries and capillary density.</p><p><strong>Conclusion: </strong>NVC of a single finger does not represent a reliable alternative for the eight-finger method because of a weak to moderate level of agreement. A four-finger method using digit III and IV of both hands shows strong agreement and could be considered in clinical practice to optimise time efficiency.</p>","PeriodicalId":17036,"journal":{"name":"Journal of Scleroderma and Related Disorders","volume":"11 1","pages":"e000015"},"PeriodicalIF":1.5,"publicationDate":"2026-05-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13231727/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148198918","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":"Lips in health and in systemic sclerosis: relevant physiology, pathogenesis, existing therapeutic approaches and unmet needs.","authors":"Mustafa Mahmood, Michael Hughes, Zsuzsanna McMahan, Lee Shapiro","doi":"10.1136/jsrd-2026-000004","DOIUrl":"10.1136/jsrd-2026-000004","url":null,"abstract":"<p><strong>Introduction: </strong>Systemic sclerosis (SSc), or scleroderma, is a chronic autoimmune disease marked by excessive collagen deposition and vasculopathy. Oro-facial manifestations of SSc, particularly involving the lips, are common including microcheilia, microstomia, and the classic \"mask-like\" face, all of which greatly affect patient quality of life. This review explores the lips in both health and SSc. We examine factors affecting lip health, methods of assessment, SSc-specific impacts, and discuss available therapeutic approaches.</p><p><strong>Methods: </strong>A comprehensive literature search was conducted via PubMed and Embase. All article types were considered from inception of the databases, and the search was last run on April 20, 2025.</p><p><strong>Results: </strong>The lips play a critical role in facial expression, communication, and appearance. Anatomy is complex and relevant to SSc pathogenesis. Relevant factors include gender differences, aging patterns, ethnicity, and lifestyle. The pathogenesis of lip involvement in SSc is multifactorial. In SSc, dermal fillers, autologous fat transfer, and platelet-rich plasma therapy have been shown to at least transiently volumize the lips and alleviate tightness. There is a lack of data on long-term benefits as well as standardized assessment tools for lip involvement in SSc.</p><p><strong>Conclusion: </strong>The impact of lip involvement in SSc presents significant unmet clinical needs. There is a critical need to benchmark natural lip variation due to ethnicity, aging, and gender to facilitate research. Future research agendas should focus on defining patient priorities, standardizing imaging techniques to assist in understanding pathogenesis and treatment response, and developing evidence based educational resources for patients and healthcare professionals.</p>","PeriodicalId":17036,"journal":{"name":"Journal of Scleroderma and Related Disorders","volume":"11 1","pages":""},"PeriodicalIF":1.5,"publicationDate":"2026-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13224322/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148150225","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":"Expert recommendations for the management of fecal incontinence in systemic sclerosis: A report from the World Scleroderma Foundation (WSF) Gastrointestinal \"Ad hoc committee\".","authors":"Zsuzsanna H McMahan, Maria Cristina De Santis, Anna Lo Cricchio, Luis G Alcala-Gonzalez, John Clarke, Alexander O'Connor, Silvia Bellando-Randone, Christopher P Denton, Ilaria Galetti, Alain Lescoat, Marco Matucci-Cerinic, Pietro Matucci-Cerinic, John McLaughlin, Charles Murray, Elizabeth R Volkmann, Alessia Alunno, Giulia Bandini, Michael Hughes","doi":"10.1136/jsrd-2026-000022","DOIUrl":"10.1136/jsrd-2026-000022","url":null,"abstract":"<p><strong>Background: </strong>Fecal incontinence (FI) affects up to 50% of patients with systemic sclerosis (SSc), significantly impairing quality of life and daily functioning. Despite its prevalence, patients may not disclose their symptoms and there is limited guidance on evaluation and management. Given the complexity of SSc and delays in gastroenterology referrals, rheumatologists often initiate care. To address this gap, an expert panel was convened to develop practical, consensus-based recommendations for assessing and managing SSc-FI.</p><p><strong>Methods: </strong>An international Steering Board (n=19) was assembled under the auspices of the World Scleroderma Foundation GI ad hoc committee, including clinicians from rheumatology, gastroenterology, and GI surgery, as well as a methodology expert and a patient representative. A working definition of SSc-FI was established. Draft recommendations were developed through a literature review and expert consensus. Two rounds of online voting were conducted, requiring ≥75% agreement in round one and ≥60% in round two for inclusion.</p><p><strong>Results: </strong>In the first and second voting rounds, participation rates were 63.2% and 84.2%, respectively. All 22 draft recommendations were approved, with most (21) reaching consensus in the first round. Recommendations span five domains: general management approach (n=3); clinical assessment and non-pharmacological interventions (n=7 and n=3, respectively); management of FI in the context of diarrhea (n=5); and alternative interventions and strategies (n=4).</p><p><strong>Conclusion: </strong>These are the first practical recommendations for managing SSc-related FI. They emphasize a structured, multidisciplinary approach to care, highlight unmet clinical needs, and lay the foundation for a research agenda to advance the understanding and treatment of this underrecognized complication in patients with SSc.</p>","PeriodicalId":17036,"journal":{"name":"Journal of Scleroderma and Related Disorders","volume":"11 1","pages":""},"PeriodicalIF":1.5,"publicationDate":"2026-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13274632/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148277824","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":"Expert recommendations for the management of refractory gastroesophageal reflux disease in systemic sclerosis: A report from the World Scleroderma Foundation (WSF) Gastrointestinal \"Ad hoc committee\".","authors":"Michael Hughes, Anna Lo Cricchio, Maria Cristina De Santis, Luis G Alcala-Gonzalez, John Clarke, Alexander O'Connor, Silvia Bellando-Randone, Christopher P Denton, Ilaria Galetti, Alain Lescoat, Marco Matucci-Cerinic, Pietro Matucci-Cerinic, John McLaughlin, Charles Murray, Pankaj J Pasricha, Elizabeth R Volkmann, Alessia Alunno, Giulia Bandini, Zsuzsanna H McMahan","doi":"10.1136/jsrd-2026-000024","DOIUrl":"10.1136/jsrd-2026-000024","url":null,"abstract":"<p><strong>Background: </strong>Gastro-esophageal reflux disease (GERD) occurs in most (~75%) patients with systemic sclerosis (SSc) and significantly impacts quality of life. Current recommendations lack depth and therefore, our aim was to produce practical, consensus-based recommendations for refractory SSc-related GERD.</p><p><strong>Methods: </strong>An international, multidisciplinary Steering Board (SB) was assembled (n=19) consisting of clinicians (rheumatologists, gastroenterologists, GI surgeons), an expert methodologist, and patient representation. After reviewing the latest definitions of GERD and refractory GERD in the published literature, the SB collectively devised a practical definition of SSc refractory-GERD. Initial recommendations were drafted/agreed upon by the SB based on review of the existing guidelines, published literature, and expert opinion. Two online voting rounds were conducted to determine whether items should be accepted, with >75% and >60% SB agreement required for first and second rounds, respectively.</p><p><strong>Results: </strong>There was a good response/completion rate for the initial (74%) and final (84%) voting rounds. The SB agreed on all 36 draft recommendations. The majority (n=34) were approved in the first round. Recommendations were organized according to the following categories: general approach to management (n=5), assessment (n=6), non-pharmacological (n=2) and pharmacological (n=10) management, endoscopic and surgical treatment (n=3), and special circumstances, including refractory GERD and SSc-ILD (n=3), myositis and SSc CTD-overlap (n=3) and peri-lung transplant (n=4).</p><p><strong>Conclusion: </strong>The group of experts has drafted extended and practical recommendations for the management of SSc refractory-GERD. The necessity of a structured approach and a patient assessment is highlighted in order to provide a multi-disciplinary approach to the tailored choice of the treatment. Our work also has also identified significant unmet needs and has provided a research agenda to advance the field.</p>","PeriodicalId":17036,"journal":{"name":"Journal of Scleroderma and Related Disorders","volume":"11 1","pages":""},"PeriodicalIF":1.5,"publicationDate":"2026-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13271578/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148271997","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":"Incidence and prevalence of juvenile SSc in the United States: An analysis by age group.","authors":"John Mattson, Haikun Bao, Mary Flack, Karen Coeytaux","doi":"10.1177/23971983251370871","DOIUrl":"10.1177/23971983251370871","url":null,"abstract":"<p><strong>Objectives: </strong>Systemic sclerosis is a rare autoimmune connective tissue disease characterized by progressive skin and organ fibrosis, and diffuse fibro-proliferative vascular modifications. Roughly 2%-10% of systemic sclerosis cases develop during juvenile years (under 18 years of age); however, there are limited data on the incidence and prevalence of juvenile systemic sclerosis. This analysis assessed the incidence and prevalence of juvenile systemic sclerosis in the United States, overall and by age group.</p><p><strong>Methods: </strong>Juvenile systemic sclerosis patients with ⩾2 medical diagnostic claims for systemic sclerosis within a 1-year period were identified from the Optum de-identified Clinformatics^® Data Mart Database between 2007 and 2021, using International Classification of Diseases diagnostic codes. Incidence and prevalence rates were estimated in the overall population and in a subgroup of patients with at least one filled prescription or infusion of methotrexate, mycophenolate mofetil or cyclophosphamide. Estimates were sex- and/or age-adjusted according to the 2020 US census.</p><p><strong>Results: </strong>Forty-eight incident and 103 prevalent cases were identified. Overall, the age- and sex-adjusted incidence and prevalence of juvenile systemic sclerosis were 2.4 per million person-years and 12.0 per million, respectively. When stratified by age group, incidence and prevalence rates increased with age in the overall and subgroup analyses.</p><p><strong>Conclusion: </strong>In this analysis, incidence and prevalence rates of juvenile systemic sclerosis increased with age. Increasing incidence and prevalence were observed from 10 years old and from 6 years old, respectively. While the overall data are broadly consistent with previous reports, these data provide new information on the estimated incidence and prevalence of juvenile systemic sclerosis by age group.</p>","PeriodicalId":17036,"journal":{"name":"Journal of Scleroderma and Related Disorders","volume":" ","pages":"303-310"},"PeriodicalIF":1.5,"publicationDate":"2025-10-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12531193/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145329261","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 eHealth literacy among people with systemic sclerosis: A Scleroderma Patient-centred Intervention Network (SPIN) Cohort cross-sectional study.","authors":"Natalie Co, Claire Adams, Marie-Eve Carrier, Meira Golberg, Elsa-Lynn Nassar, Linda Kwakkenbos, Susan J Bartlett, Catherine Fortuné, Amy Gietzen, Geneviève Guillot, Amanda Lawrie-Jones, Vanessa L Malcarne, Maureen D Mayes, Michelle Richard, Luc Mouthon, Andrea Benedetti, Brett D Thombs","doi":"10.1177/23971983251376428","DOIUrl":"10.1177/23971983251376428","url":null,"abstract":"<p><strong>Introduction/objective: </strong>eHealth literacy reflects the ability to obtain, understand, and evaluate health information from electronic sources and apply this information to health problems. Our objective was to evaluate sociodemographic (age, sex, race or ethnicity, education, marital status, country, residence location) and disease factors (duration, subtype) associations with eHealth literacy among individuals with systemic sclerosis (SSc).</p><p><strong>Methods: </strong>Scleroderma Patient-centred Intervention Network (SPIN) Cohort participants completed the 8-item eHealth Literacy Scale (eHEALS) from January 17 to February 18, 2025. Multivariable linear regression was used to assess associations of sociodemographic and disease characteristics with eHealth literacy.</p><p><strong>Results: </strong>The 333 participants were from France (N = 116, 35%), Canada (N = 90, 27%), the United States (N = 85, 26%), the United Kingdom (N = 32, 10%), and Australia, Mexico, or Spain (N = 10, 3%). Most participants were female (N = 295, 89%), White (N = 268, 80%), and had limited SSc (N = 206, 62%). Compared to the United States, participants from Canada (-2.2 points, 95% CI -4.2 to -0.1; standardized mean difference (SMD) = -0.33) and France (-4.2 points, 95% CI -6.2 to -2.3; SMD = -0.64) had significantly lower eHEALS scores. Age, sex, race or ethnicity, marital status, education level, time since first non-Raynaud's symptom onset, and disease subtype were not associated with eHEALS scores.</p><p><strong>Conclusion: </strong>eHealth literacy in SSc was not associated with age and education level as in some other studies but was associated with country. Future research should examine country-level differences in eHealth literacy for individuals with SSc.</p>","PeriodicalId":17036,"journal":{"name":"Journal of Scleroderma and Related Disorders","volume":" ","pages":"588-596"},"PeriodicalIF":1.5,"publicationDate":"2025-09-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12479466/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145206635","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":"Pain and fatigue in systemic sclerosis: Practical strategies for non-pharmacological management.","authors":"Francesco Bonomi, Khadija El Aoufy, Silvia Bellando Randone, Marco Matucci-Cerinic, Michael Hughes","doi":"10.1177/23971983251369577","DOIUrl":"10.1177/23971983251369577","url":null,"abstract":"<p><strong>Objective: </strong>Pain and fatigue are among the most prevalent and disabling symptoms in patients with systemic sclerosis, significantly impairing quality of life and daily functioning. Despite their high burden, these symptoms are frequently underrecognized and undertreated by clinicians. This narrative review aims to synthesize current evidence and practical approaches to support multidisciplinary care in the assessment and non-pharmacological management of pain and fatigue in systemic sclerosis.</p><p><strong>Methods: </strong>We conducted a comprehensive review of the literature focusing on the multifactorial causes, diagnostic workup, and non-pharmacological treatment strategies for pain and fatigue in systemic sclerosis. This narrative review integrates evidence-based recommendations, expert consensus statements, and reflects best current clinical practice.</p><p><strong>Results: </strong>Pain and fatigue in patients with systemic sclerosis are underestimated and often multifactorial. Pain in systemic sclerosis may arise from musculoskeletal, neuropathic, vascular, gastrointestinal, and iatrogenic sources, while fatigue is driven by cardiopulmonary involvement, malnutrition, endocrine dysfunction, poor sleep quality, and psychological factors. Accurate symptom assessment requires a combination of patient-reported outcome measures, clinical examination, imaging modalities, and laboratory testing. Non-pharmacological interventions, including physical rehabilitation, cognitive behavioural therapy, nutritional support, and mind-body practices, demonstrate particularly promising benefits in symptom relief and functional improvement, although high-quality evidence in systemic sclerosis remains limited.</p><p><strong>Conclusion: </strong>A personalized, holistic approach is essential to effectively address pain and fatigue in patients with systemic sclerosis. Interdisciplinary care involving rheumatologists, nurses, physiotherapists, nutritionists, psychologists, and occupational therapists is key to optimizing outcomes. Our data show that some unmet needs await to be addressed and therefore an agenda for future research is proposed to improve quality of life in patients with systemic sclerosis.</p>","PeriodicalId":17036,"journal":{"name":"Journal of Scleroderma and Related Disorders","volume":" ","pages":"237-250"},"PeriodicalIF":1.5,"publicationDate":"2025-09-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12457323/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145149580","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":"Criteria for defining and dating onset of organ system involvement in systemic sclerosis.","authors":"Marie Hudson, John D Pauling, Tatiana S Rodriguez-Reyna, Julie J Paik, Ami A Shah, Tracy M Frech, Francesco Boin, Wendy Stevens, Luc Mouthon, Melanie C Baniña, Thomas A Medsger","doi":"10.1177/23971983251374385","DOIUrl":"10.1177/23971983251374385","url":null,"abstract":"<p><strong>Objectives: </strong>This study was undertaken to develop standardized definitions of individual organ system involvement in systemic sclerosis and their date of onset, with the overall goal of increasing the internal validity and comparability of future clinical trials and observational studies.</p><p><strong>Methods: </strong>Under the auspices of the Scleroderma Clinical Trials Consortium, an international working group of experts and patient partners formed nine sub-groups for each of the following organ systems: skin, peripheral vascular, joints/tendons, skeletal muscle, gastrointestinal tract, parenchymal lung, pulmonary arterial hypertension, heart, and kidney. Lists of elements that could be considered as potential criteria for involvement of each organ system were compiled. A three-round Delphi exercise was conducted among Scleroderma Clinical Trials Consortium and the European Scleroderma Trials and Research group members in order to achieve consensus with the larger systemic sclerosis community. Informed by the results of the Delphi exercise, sub-groups proposed composite criteria for organ system involvement, and the international working group approved the final criteria.</p><p><strong>Results: </strong>This consensus-driven project involved over 160 systemic sclerosis experts from around the world, organ system specialists, and patient participants. We developed criteria for nine individual organ systems commonly involved in systemic sclerosis, made recommendations for the date of onset thereof, and provided explanatory notes.</p><p><strong>Conclusions: </strong>We believe these standardized criteria for systemic sclerosis organ system involvement and date of onset provide useful guidelines for investigators and will enhance the comparability of future clinical trials and observational studies in this disease.</p>","PeriodicalId":17036,"journal":{"name":"Journal of Scleroderma and Related Disorders","volume":" ","pages":"293-302"},"PeriodicalIF":1.5,"publicationDate":"2025-09-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12460267/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145186031","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":"Ultrasonographic assessment of digital ulcers in systemic sclerosis: Quantitative and qualitative insights for evaluation and follow-up.","authors":"Luca Idolazzi, Riccardo Bixio, Denise Rotta, Francesca Pistillo, Giulia Zanetti, Antonio Carletto, Maurizio Rossini, Ombretta Viapiana","doi":"10.1177/23971983251343459","DOIUrl":"10.1177/23971983251343459","url":null,"abstract":"<p><strong>Objective: </strong>The objective of this study is to examine the efficacy of ultrasonography in evaluating and tracking the healing process of digital ulcers in patients with systemic sclerosis.</p><p><strong>Methods: </strong>The study cohort comprised 23 patients with systemic sclerosis and digital ulcers. Gray Scale and Power Doppler ultrasonography was employed for the assessment of lesions. Subsequently, a sharp debridement was administered, followed by the application of hydrocolloid dressings. The healing process was then monitored through follow-up and using ultrasonography.</p><p><strong>Results: </strong>Of the 46 ulcers observed, 34 demonstrated complete healing, while 12 did not. The use of Power Doppler ultrasonography was found to be a highly significant indicator of healing. Significant statistical differences were observed in lesion dimensions (length, width, depth) between the healed and non-healed groups and a strong correlation was observed between the reduction in lesion dimensions and the healing process. The results of the point-biserial correlation analysis indicated a robust positive correlation between healing and alterations in length (r = 0.600) and width (r = 0.576), and a moderate positive correlation with depth (r = 0.400).</p><p><strong>Conclusion: </strong>The findings of this study indicate that the ultrasonography is a feasible and objective method to assess digital ulcers and their healing. Reduction of length and width and presence of Power Doppler ultrasonography are strong indicators of wound repair. Furthermore, the presence of necrotic tissue is not an indicator for a poor outcome. Ultrasonography offers comprehensive qualitative and quantitative data that facilitate a deeper comprehension and monitoring of the healing process.</p>","PeriodicalId":17036,"journal":{"name":"Journal of Scleroderma and Related Disorders","volume":" ","pages":"425-433"},"PeriodicalIF":1.5,"publicationDate":"2025-09-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12436318/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145081048","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":"Characteristics of patients with systemic sclerosis with aspiration: A single-center study.","authors":"Tatsuaki Naganawa, Takako Hashimoto, Naomi Ikeda, Haruna Takase, Naoki Dosoden, Kodai Ito, Marika Sawada, Yumi Ito, Natsuko Watanabe, Ai Umeda, Konomi Akamatsu, Megumi Kurumizawa, Jo Nishino, Shusaku Fukaya, Yoko Inamoto, Seiko Shibata, Yohei Otaka, Hidekata Yasuoka","doi":"10.1177/23971983251370883","DOIUrl":"10.1177/23971983251370883","url":null,"abstract":"<p><strong>Objectives: </strong>To investigate the prevalence of aspiration and its association with GI involvement and clinical features in systemic sclerosis (SSc).</p><p><strong>Methods: </strong>Fifty patients with SSc who underwent the videofluoroscopic (VF) swallowing study were included. Aspiration was identified by the presence of aspiration and/or penetration defined by the Penetration-Aspiration Scale. The radiological findings including the residue in the oral cavity, pharynx and esophagus, which reflect oral, pharyngeal, and esophageal involvement, were also evaluated.</p><p><strong>Results: </strong>Twenty-three patients (46%) had aspiration and/or penetration. Patients with aspiration and/or laryngeal penetration had more pharyngeal vallecular residue (None 22%, Trace 44%, Mild 56%; <i>p</i> = 0.072) and esophageal residue (None 0%, Mild 20%, Moderate 40%, Severe 61%; <i>p</i> = 0.010). Esophageal, pharyngeal, and lower GI involvement were found in 98%, 83%, and 54%, respectively. Notably, the group with esophageal and pharyngeal plus lower GI involvement had a higher frequency of aspiration and/or penetration (79% vs 28%; <i>p</i> = 0.003), higher FSSG scores (18.4 ± 11.8 vs 5.4 ± 5.7; <i>p</i> = 0.002), and higher UCLA GIT-2.0 total scores (None-to-Mild 47% vs 100%, Moderate 42% vs 0%, Severe-to-Very severe 11% vs 0%; <i>p</i> = 0.001) compared to those limited esophageal and pharyngeal involvement. By multivariate analysis, aspiration and/or penetration was associated with pharyngeal vallecular residue (Odds ratio (OR) 3.71; <i>p</i> = 0.012) and esophageal residue (OR 2.92; <i>p</i> = 0.011), and higher UCLA GIT-2.0 scores for diarrhea (OR 3.68; <i>p</i> = 0.028) and the total score (OR 4.21; <i>p</i> = 0.046).</p><p><strong>Conclusion: </strong>In our study, about half of the patients had aspiration and/or penetration in SSc. Aspiration was associated with the extent of radiographic abnormalities of pharyngeal and esophageal involvement on the VF swallowing study. Patients with lower GI have aspiration and high UCLA GIT-2.0 scores suggesting that these patients had more severe GI manifestation.</p>","PeriodicalId":17036,"journal":{"name":"Journal of Scleroderma and Related Disorders","volume":" ","pages":"458-471"},"PeriodicalIF":1.5,"publicationDate":"2025-09-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12425942/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145064851","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}