Journal of Scleroderma and Related Disorders最新文献

{"title":"Hand functional capacity in women with systemic sclerosis using the Glittre-ADL-Shelf test: Relationship with demographics, body composition, hand function, physical function, digital dexterity, muscle strength and lung function.","authors":"Fátima de Sousa Paiva Duarte, Isabelle da Nobrega Ferreira, Alessandro Dos Santos Beserra, Nicolly Oliveira Barbosa, Laura Lima da Silva, Viviane Cristina Viana de Souza, Valter Gomes, Agnaldo José Lopes","doi":"10.1177/23971983251341515","DOIUrl":"10.1177/23971983251341515","url":null,"abstract":"<p><strong>Objective: </strong>Hand functionality in systemic sclerosis is assessed using patient-reported measures. However, the Glittre-ADL test (TGlittre) provides an objective assessment of functional capacity using activities of daily living, including the hand shelf task. The aim of this study was to assess hand functional capacity in women with systemic sclerosis using the TGlittre-Shelf and to correlate it with demographics, body composition, hand function, physical function, digital dexterity, muscle strength, and lung function.</p><p><strong>Methods: </strong>This was a cross-sectional study in which 41 women with systemic sclerosis and 41 healthy controls underwent the TGlittre-S. They also underwent the following assessments: Cochin Hand Functional Scale, modified Rodnan skin score, Health Assessment Questionnaire Disability Index, 9-hole peg test, handgrip strength, and pulmonary function tests.</p><p><strong>Results: </strong>The mean age was 51.9 ± 13.7 and 45.9 ± 9.3 years in women with systemic sclerosis and healthy controls, respectively (<i>p</i> = 0.09). Body mass index was 24.4 ± 4.9 and 29.4 ± 5.3 kg/m² in women with systemic sclerosis and healthy controls, respectively (<i>p</i> < 0.0001). TGlittre-S time was higher in women with systemic sclerosis than in healthy controls [60 (55-74) vs 44 (41-49) sec, <i>p</i> < 0.0001]. In addition, 9-hole peg test was higher in women with systemic sclerosis than in healthy controls [24 (22-26) vs 20 (18-22) sec, <i>p</i> < 0.0001]. Similarly, Cochin Hand Functional Scale was higher in women with systemic sclerosis than in healthy controls [6 (0.5-25) vs 0 (0-0) points, <i>p</i> < 0.0001]. In women with systemic sclerosis, TGlittre-S time was significantly correlated with the following variables: handgrip strength (<i>r_s</i>  = -0.511, <i>p</i> = 0.0006), Health Assessment Questionnaire Disability Index (<i>r_s</i>  = 0.510, <i>p</i> = 0.0006), 9-hole peg test (<i>r_s</i>  = 0.398, <i>p</i> = 0.009), and Cochin Hand Functional Scale (<i>r_s</i>  = 0.351, <i>p</i> = 0.024).</p><p><strong>Conclusion: </strong>In women with systemic sclerosis, there is a deterioration in impaired hand functional capacity as measured by TGlittre-S. In these patients, the longer the TGlittre-S time, the worse the handgrip strength, digital dexterity, and physical function. As TGlittre-S is easy to perform and does not require much space, its incorporation into clinical practice is promising and may be considered as an outcome measure for future studies in systemic sclerosis.</p>","PeriodicalId":17036,"journal":{"name":"Journal of Scleroderma and Related Disorders","volume":" ","pages":"23971983251341515"},"PeriodicalIF":1.4,"publicationDate":"2025-05-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12102079/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144142855","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":"Frequency and determinants of use of immunosuppressants in the Australian Scleroderma Cohort Study.","authors":"Jessica L Fairley, Dylan Hansen, Susanna Proudman, Joanne Sahhar, Gene-Siew Ngian, Diane Apostolopoulos, Jennifer Walker, Lauren V Host, Wendy Stevens, Laura Ross, Mandana Nikpour","doi":"10.1177/23971983251342690","DOIUrl":"10.1177/23971983251342690","url":null,"abstract":"&lt;p&gt;&lt;strong&gt;Objectives: &lt;/strong&gt;To assess the frequency and determinants of immunosuppressant medication use in systemic sclerosis and changes in prescribing patterns over time.&lt;/p&gt;&lt;p&gt;&lt;strong&gt;Methods: &lt;/strong&gt;The Australian Scleroderma Cohort Study participants meeting the American College of Rheumatology/European Alliance of Associations for Rheumatology criteria for systemic sclerosis with recorded treatment data were included. The Chi-square, two-sample &lt;i&gt;t&lt;/i&gt;-tests or Wilcoxon rank-sum tests were used for between-group comparison as appropriate. Multivariable logistic regression models were used to establish the determinants of the use of immunosuppressants.&lt;/p&gt;&lt;p&gt;&lt;strong&gt;Results: &lt;/strong&gt;Of 2019 participants, 60% received immunosuppressants, including 81% of those with diffuse systemic sclerosis and 52% of those with limited systemic sclerosis (p &lt; 0.001). Forty-six percent of patients received prednisolone and 40% disease-modifying anti-rheumatic drugs. Immunosuppressant use was more common in those with severe or inflammatory systemic sclerosis features, including interstitial lung disease, synovitis or myositis. Comparing prescribing patterns early in incident systemic sclerosis from 2007-2014 to 2015-2024, disease-modifying anti-rheumatic drug use increased (35% vs 56%, p &lt; 0.001), while prednisolone use decreased (24% vs 17%, p = 0.046). Immunosuppressants were commenced earlier in incident systemic sclerosis in 2015-2024 versus 2007-2014 (1.8 (interquartile range = 1.0-3.2) vs 2.4 (interquartile range = 1.2-4.0) years, p = 0.011). In multivariable modelling, prednisolone use was associated with diffuse systemic sclerosis (odds ratio = 1.8, 95% confidence interval = 1.4-2.2, p &lt; 0.001), interstitial lung disease (odds ratio = 2.1, 95% confidence interval = 1.7-2.5, p &lt; 0.001), myositis (odds ratio = 2.7, 95% confidence interval = 1.8-4.0, p &lt; 0.001), synovitis (odds ratio = 2.2, 95% confidence interval = 1.8-2.6, p &lt; 0.001) and systemic sclerosis heart involvement (odds ratio = 1.4, 95% confidence interval = 1.0-2.0, p = 0.044). Disease-modifying anti-rheumatic drug exposure was associated with diffuse systemic sclerosis (odds ratio = 2.7, 95% confidence interval = 2.1-3.4, p &lt; 0.001), interstitial lung disease (odds ratio = 2.2, 95% confidence interval = 1.7-2.7, p &lt; 0.001), myositis (odds ratio = 3.6, 95% confidence interval = 2.4-5.5, p &lt; 0.001) and synovitis (odds ratio = 4.2, 95% confidence interval = 3.5-5.2, p &lt; 0.001) and inversely associated with age (odds ratio = 0.7, 95% confidence interval = 0.5-0.8, p &lt; 0.01) and pulmonary arterial hypertension (odds ratio = 0.5, 95% confidence interval = 0.4-0.7, p &lt; 0.001). In subgroups with diffuse systemic sclerosis and limited systemic sclerosis and different autoantibody profiles, findings were generally similar, with interstitial lung disease, synovitis and myositis tending to be associated with prednisolone and/or disease-modifying anti-rheumatic drug use, as was systemic sclero","PeriodicalId":17036,"journal":{"name":"Journal of Scleroderma and Related Disorders","volume":" ","pages":"23971983251342690"},"PeriodicalIF":1.4,"publicationDate":"2025-05-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12102085/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144142854","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 systematic literature review of Janus kinase inhibitors for the treatment of systemic sclerosis.","authors":"Seher Sener, Yusuf Ziya Sener, Ezgi Deniz Batu, Alper Sari, Ali Akdogan","doi":"10.1177/23971983251342697","DOIUrl":"10.1177/23971983251342697","url":null,"abstract":"<p><strong>Objectives: </strong>The use of Janus kinase inhibitors is increasing in systemic sclerosis, a complex autoimmune disease characterized by fibrosis, vasculopathy, and immune dysregulation. In this review, we aimed to examine the studies in the literature reporting on patients with systemic sclerosis treated with Janus kinase inhibitors.</p><p><strong>Methods: </strong>We performed a search on MEDLINE and Scopus for articles involving patients with systemic sclerosis treated with Janus kinase inhibitors from the inception of these databases through 1 August 2024.</p><p><strong>Results: </strong>Our literature search revealed 18 articles describing 87 systemic sclerosis patients treated with Janus kinase inhibitors. The median (min-max) age of these patients was 48.5 (13-78) years (F/M = 4.9). The most commonly used Janus kinase inhibitors in systemic sclerosis were tofacitinib (82.8%), followed by baricitinib (13.8%). Janus kinase inhibitors were most commonly used to treat the combination of interstitial lung disease and skin involvement (44.9%), and the combination of gastrointestinal system and skin involvement (38.8%). Improvement rates with Janus kinase inhibitors were 87.5%, and 5.9% of patients on Janus kinase inhibitors relapsed. Adverse events were observed in 50% of systemic sclerosis patients treated with Janus kinase inhibitors. Various infections (21.1%) were the most common adverse event reported following Janus kinase inhibitor use.</p><p><strong>Conclusion: </strong>Although Janus kinase inhibitors seem to be very effective, especially in patients with systemic sclerosis who have resistant/progressive skin disease, some side effects should not be ignored. Therefore, controlled clinical trials in larger populations are needed on Janus kinase inhibitors use in systemic sclerosis.</p>","PeriodicalId":17036,"journal":{"name":"Journal of Scleroderma and Related Disorders","volume":" ","pages":"23971983251342697"},"PeriodicalIF":1.4,"publicationDate":"2025-05-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12102077/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144142852","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 impact of systemic sclerosis on hospitalized COVID-19 patients: Analysis of the US nationwide inpatient sample (2021).","authors":"Nattanicha Chaisrimaneepan, Ben Thiravetyan, Pannathorn Nakaphan, Chanokporn Puchongmart","doi":"10.1177/23971983251342065","DOIUrl":"10.1177/23971983251342065","url":null,"abstract":"<p><strong>Background: </strong>The study was conducted to investigate the impact of systemic sclerosis (SSc) on hospitalized COVID-19 patients.</p><p><strong>Method: </strong>This retrospective observational study analyzed data from the National Inpatient Survey (NIS) in 2021. Patients hospitalized with COVID-19 were categorized into SSc and non-SSc groups. Characteristics of patients and comorbidities were compared. The primary outcome was the mortality rate. Secondary outcomes included resource utilization and acute in-hospital complications of SSc. Multivariate logistic regression analyses were conducted, with <i>p</i>-values < 0.05 considered statistically significant.</p><p><strong>Result: </strong>Of all, 1865 patients hospitalized with COVID-19 had SSc. Patients with SSc had a higher mortality risk (aOR = 1.37 [1.03-1.82]; <i>p</i> = 0.032). The average cost of hospitalization was significantly higher in the SSc group (<i>p</i> = 0.048), with no difference in LOS (9.4 ± 0.65 days vs 8.4 ± 0.03 days; <i>p</i> = 0.260). COVID-19 patients with SSc significantly had a higher risk for DIC (aOR 2.82 [1.06-7.53]; <i>p</i> = 0.038), left-sided HF (aOR 1.76 [1.16-2.67]; <i>p</i> = 0.008), ventricular arrhythmia (aOR 3.17 [1.01-9.89]; <i>p</i> = 0.047), oxygen dependence (aOR 2.41 [1.64-3.55]; <i>p</i> < 0.001), cardiac arrest (aOR 2.61 [1.63-4.18]; <i>p</i> < 0.001), and ileus (aOR 2.61 [1.45-4.69]; <i>p</i> = 0.001).</p><p><strong>Conclusion: </strong>Hospitalized COVID-19 patients with SSc were more likely to develop in-hospital complications and had a higher mortality risk.</p>","PeriodicalId":17036,"journal":{"name":"Journal of Scleroderma and Related Disorders","volume":" ","pages":"23971983251342065"},"PeriodicalIF":1.4,"publicationDate":"2025-05-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12092399/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144127903","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":"Recent advances in non-invasive imaging of systemic sclerosis-related digital ulcers.","authors":"Ariane L Herrick, Michael Hughes, Andrea Murray","doi":"10.1177/23971983251339703","DOIUrl":"10.1177/23971983251339703","url":null,"abstract":"<p><p>Digital ulcers are a major source of pain and disability in patients with systemic sclerosis. Current treatments are not ideal, yet drug development for digital ulcers is hampered by a lack of objective outcome measures to facilitate clinical trials. Advances in non-invasive imaging could provide a way forward. This review article first describes the rationale for non-invasive imaging of digital ulcers in both clinical practice and research. In clinical practice, magnetic resonance imaging allows early diagnosis of underlying osteomyelitis, and smartphone imaging allows early (remote) identification of digital ulcers. In research, non-invasive imaging provides new insights into pathophysiology, as well as the ability to measure precisely digital ulcer surface area and volume. The imaging techniques discussed include mobile phone photography, ultrasound, laser Doppler methods and emerging technologies (multispectral imaging and polarisation-sensitive optical coherence tomography). Between them, these methods hold promise as outcome measures for early and later phase trials, but first require full validation.</p>","PeriodicalId":17036,"journal":{"name":"Journal of Scleroderma and Related Disorders","volume":" ","pages":"23971983251339703"},"PeriodicalIF":1.4,"publicationDate":"2025-05-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12095265/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144142857","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":"Isolated scleroderma of the lower extremities misdiagnosed as lymphedema and presenting with scleroderma renal crisis.","authors":"Hammad Ali, Lais Lopes Almeida Gomes, Touraj Khosravi-Hafshejani, Aretha On, Xiwei Yang, Shae Chambers, Victoria P Werth","doi":"10.1177/23971983251334505","DOIUrl":"10.1177/23971983251334505","url":null,"abstract":"<p><p>Systemic sclerosis (SSc) is a chronic autoimmune disorder characterized by progressive fibrosis, vasculopathy, and immune dysregulation. The disease commonly presents with Raynaud's phenomenon and skin thickening, commonly of the upper limb. Isolated lower extremity presentation is uncommon and often misdiagnosed. This diagnostic uncertainty may lead to delayed recognition and increased morbidity, particularly when SSc mimics lymphedema in the early stages. We report a case of a 61-year-old female who initially presented with lower extremity swelling and was misdiagnosed with primary lymphedema. Despite treatment with diuretics and compression therapy, her symptoms progressed to involve her upper extremities, prompting further evaluation. Physical examination revealed non-pitting lower extremity scleroderma, sclerodactyly, puffy hands, and nailfold capillary abnormalities. Laboratory workup was positive for anti-RNA polymerase III antibodies, a marker associated with an increased risk of scleroderma renal crisis. The patient developed scleroderma renal crisis after a delayed diagnosis, necessitating hospital admission and initiation of angiotensin-converting enzyme inhibitors. This case highlights the challenges in distinguishing early lower extremity SSc from lymphedema. Early identification of atypical SSc presentations is critical to be cognizant of life-threatening complications such as scleroderma renal crisis. Clinicians should maintain a high index of suspicion for SSc in patients with persistent non-pitting lower extremity swelling, skin thickening, and abnormal capillaroscopy findings, even in the absence of initial upper limb involvement.</p>","PeriodicalId":17036,"journal":{"name":"Journal of Scleroderma and Related Disorders","volume":" ","pages":"23971983251334505"},"PeriodicalIF":1.4,"publicationDate":"2025-05-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12081387/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144094136","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":"Nanofat and lipofilling for cutaneous fibrosis in scleroderma: Current evidence and future directions.","authors":"Giuseppe Di Toro, Angelo Alito, Giulia Leonardi, Fiorenza Giulia Di Toro, Simona Portaro","doi":"10.1177/23971983251341502","DOIUrl":"10.1177/23971983251341502","url":null,"abstract":"<p><p>Systemic sclerosis is a rare autoimmune disease characterized by progressive fibrosis, microvascular dysfunction, and chronic inflammation, leading to significant functional and aesthetic impairments, particularly in the face and hands. Current therapeutic strategies are limited in their ability to reverse established skin fibrosis, prompting interest in regenerative approaches such as autologous fat grafting. Lipofilling and nanofat techniques, originally developed for volume restoration and skin rejuvenation, have demonstrated promising outcomes in improving skin texture, elasticity, and vascularization through mechanisms involving adipose-derived stem cells and stromal vascular fraction. This editorial reviews the available randomized controlled trials evaluating nanofat and lipofilling in the treatment of cutaneous manifestations of systemic sclerosis. A literature search identified four randomized controlled trials meeting inclusion criteria, primarily focused on facial fat grafting with or without platelet-rich plasma in corticosteroid-resistant patients. Although subjective improvements in skin quality were reported, methodological variability, small sample sizes, and non-standardized techniques limit the generalizability of findings. Given the autologous and minimally invasive nature of fat grafting, it represents a potentially valuable adjunct treatment for systemic sclerosis. However, further research, including multicenter registries and standardized protocols, is essential to better define its clinical utility and optimize outcomes in this complex disease.</p>","PeriodicalId":17036,"journal":{"name":"Journal of Scleroderma and Related Disorders","volume":" ","pages":"23971983251341502"},"PeriodicalIF":1.4,"publicationDate":"2025-05-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12081377/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144094139","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":"Recommendations for the local management of digital ulcers in systemic sclerosis: A report from the World Scleroderma Foundation (WSF) 'Ad hoc committee'.","authors":"Corrado Campochiaro, Yossra A Suliman, Dilia Giuggioli, Pia Moinzadeh, Alessia Alunno, Jan W Schoones, Murray Baron, Lorinda Chung, Laura Ross, Nancy Maltez, Begonya Alcacer-Pitarch, Khadija El-Aoufy, Yannick Allanore, Francesco Del Galdo, Christopher P Denton, Oliver Distler, Tracy Frech, Daniel E Furst, Dinesh Khanna, Thomas Krieg, Masataka Kuwana, Marco Matucci-Cerinic, Janet Pope, Michael Hughes","doi":"10.1177/23971983251339821","DOIUrl":"10.1177/23971983251339821","url":null,"abstract":"<p><strong>Introduction: </strong>Digital ulcers (DUs) stand out as one of the most prevalent and clinically meaningful manifestations of systemic sclerosis (SSc) and are associated with significant morbidity. While systemic (pharmacological) therapy is currently established as the 'standard of care', effective local ulcer management remains crucial for all cases of DUs. This is particularly true for patients who cannot tolerate systemic treatments or in the case of refractory SSc-DUs. On this background, there is a pressing demand for the formulation of evidence-based guidelines to assist clinicians and patients in navigating the local treatment options for DUs.</p><p><strong>Methods: </strong>A steering committee of international experts was established by the World Scleorderma Foundation (WSF) Digital Ulcer (DU) ad hoc committee. Two systematic literature reviews on local non-surgical and surgical treatments for the management of SSc-DUs were performed to inform the development of local treatment recommendations for SSc-DUs. Consensus methodology was used to develop the final treatment recommendations.</p><p><strong>Results: </strong>Six overarching treatment principles and eight local treatment recommendations (five non-surgical and three surgical) were agreed upon for the management of SSc-DU. Among topical non-surgical options, botulin toxin can be conditionally recommended for refractory and/or severe DUs. Among surgical treatments, autologous adipose tissue grafting might be recommended for DU healing when combined with background systemic treatments.</p><p><strong>Conclusion: </strong>These recommendations are specifically tailored to guide treatment decisions concerning both local and non-pharmacological approaches to managing SSc-related DUs. Our work has highlighted a notable quality gap in comparison to systemic treatments, underscoring the scarcity of high-quality studies concerning this topic.</p>","PeriodicalId":17036,"journal":{"name":"Journal of Scleroderma and Related Disorders","volume":" ","pages":"23971983251339821"},"PeriodicalIF":1.4,"publicationDate":"2025-05-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12084219/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144094102","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 evaluation of hand and wrist joints in patients with systemic sclerosis.","authors":"Zeynep Ermancık, Müçteba Enes Yayla, Ahmet İlbay, Nilgün Göveç Gıynaş, Serdar Sezer, Emine Uslu, Aşkın Ateş, Murat Turgay","doi":"10.1177/23971983251337227","DOIUrl":"10.1177/23971983251337227","url":null,"abstract":"<p><strong>Introduction: </strong>The objective of this investigation was to reveal abnormal musculoskeletal ultrasonographic (US) findings in the hand and wrist joints of systemic sclerosis (SSc) patients compared to healthy controls and to determine the severity of synovitis and tenosynovitis, as well as to disclose the demographic, clinical, and serologic characteristics of patients with SSc with synovitis.</p><p><strong>Methods: </strong>The current study is a cross-sectional study comparing 50 SSc patients and 50 healthy controls between February and September 2023. Metacarpophalangeal (MCP), proximal interphalangeal (PIP), distal interphalangeal (DIP), and wrist joints were evaluated with US.</p><p><strong>Results: </strong>Synovitis (24 (48%) vs 6 (12%), <i>p</i> < .001), synovial hypertrophy (19 (38%) vs 1 (2%), <i>p</i> < .001), tenosynovitis (22 (44%) vs 5 (10%), <i>p</i> < .001), and calcinosis (7 (14%) vs 0, <i>p</i> = .012) were more common in SSc patients than in healthy controls. The presence of osteophytes was also more frequent in SSc patients but not statistically significant (36 (72%) vs 27 (54%), <i>p</i> = .062). The frequencies of moderate to severe synovitis and tenosynovitis were similar in the two groups, whereas mild synovitis (44% vs 12%, <i>p</i> < .001) and tenosynovitis (38% vs 8%, <i>p</i> < .001) were more common in SSc patients. The wrist was the most affected joint. Patients with synovitis had a higher mean age (<i>p</i> = .007) and erythrocyte sedimentation rate (ESR; <i>p</i> = .025). The presence of high c-reactive protein (CRP; <i>p</i> = .039) and hydroxychloroquine use (<i>p</i> = .024) were more common in these patients. There was no difference between the frequency of arthralgia in patients with and without synovitis.</p><p><strong>Conclusions: </strong>Synovitis, tenosynovitis, and synovial hypertrophy seem to be quite common in SSc patients with or without arthralgia. Patients with synovitis are older, and acute phase markers may be higher in these patients.</p>","PeriodicalId":17036,"journal":{"name":"Journal of Scleroderma and Related Disorders","volume":" ","pages":"23971983251337227"},"PeriodicalIF":1.4,"publicationDate":"2025-05-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12084211/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144094103","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":"Hypogammaglobulinemia in patients affected by limited cutaneous systemic sclerosis: Case series and review of the literature.","authors":"Maria Giovanna Danieli, Cristina Mezzanotte, Alberto Paladini, Devis Benfaremo, Giuseppe Murdaca, Gianluca Moroncini","doi":"10.1177/23971983251333851","DOIUrl":"https://doi.org/10.1177/23971983251333851","url":null,"abstract":"<p><strong>Background: </strong>Hypogammaglobulinemia is a condition that can be related to both primary and secondary immunodeficiencies. While the role of primary immunodeficiency in immune-mediated diseases is well known, its occurrence in systemic sclerosis is not reported.</p><p><strong>Objectives: </strong>This study aims to describe the clinical features associated with hypogammaglobulinemia in a cohort of limited cutaneous systemic sclerosis patients.</p><p><strong>Methods: </strong>We retrospectively reviewed medical records of systemic sclerosis patients from two Italian referral centres (2010-2024). Included patients had limited cutaneous systemic sclerosis and presented reduced serum concentrations of one or more Ig isotypes (IgG < 700 mg/dL, IgA < 70 mg/dL or IgM < 50 mg/dL) in at least two separate measurements. Patients with secondary causes of hypogammaglobulinemia were excluded. Data collected included demographics, clinical features, Ig levels, infection history and comorbidities.</p><p><strong>Results: </strong>We identified 30 systemic sclerosis patients (93% female, mean age 62 years) with limited cutaneous involvement and hypogammaglobulinemia. Most patients were positive for anti-centromere antibodies and received periodic intravenous infusions of prostaglandin analogues. No patient received immunosuppressive therapy. Median (interquartile range) serum IgG levels 519.5 (175) mg/dL, median IgA 65.5 (48) mg/dL and median IgM 71.5 (49) mg/dL. Four patients who met the European Society for Immunodeficiencies (ESID) criteria for common variable immunodeficiency experienced recurrent infections and had associated immune-mediated diseases. Five patients had selective IgA deficiency, with frequent immune-mediated comorbidities (thyroiditis, Sjögren's syndrome, arthritis, psoriasis). The other patients exhibited mild IgG deficiency without a significant infectious history.</p><p><strong>Conclusions: </strong>This is the first study describing a cohort of patients with limited cutaneous systemic sclerosis and hypogammaglobulinemia. Our population presented a high prevalence of immune-mediated comorbidities but low infection rates. Further research is needed to explore the underlying mechanisms and clinical significance of hypogammaglobulinemia in these patients.</p>","PeriodicalId":17036,"journal":{"name":"Journal of Scleroderma and Related Disorders","volume":" ","pages":"23971983251333851"},"PeriodicalIF":1.4,"publicationDate":"2025-05-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12052914/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144000492","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}