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Early IL-1 Inhibition in Still's Disease: A Window of Opportunity for Improving Outcomes. 斯蒂尔氏病早期IL-1抑制:改善预后的机会之窗
IF 2.8
ACR open rheumatology Pub Date : 2025-10-01 DOI: 10.1002/acr2.70106
Sara Bindoli, Cristina Cadore, Irina Guidea, Andrea Doria, Roberta Ramonda, Paolo Sfriso
{"title":"Early IL-1 Inhibition in Still's Disease: A Window of Opportunity for Improving Outcomes.","authors":"Sara Bindoli, Cristina Cadore, Irina Guidea, Andrea Doria, Roberta Ramonda, Paolo Sfriso","doi":"10.1002/acr2.70106","DOIUrl":"10.1002/acr2.70106","url":null,"abstract":"<p><strong>Objective: </strong>Still's disease is a complex, multisystemic disorder requiring prompt diagnosis and treatment. This study provides a general assessment of a Still's disease cohort aiming to evaluate the efficacy of early intervention with interleukin-1 inhibitors (IL-1i) in achieving clinical inactive disease (CID) and reducing glucocorticoid use in patients with Still's disease.</p><p><strong>Methods: </strong>We retrospectively analyzed 42 adult patients diagnosed with Still's disease after 2010 and treated with IL-1i. Patients were categorized as early treated (IL-1i started before six months from symptom onset) or late treated (IL-1i started after 6 months). CID achievement, glucocorticoid use, flares, and imaging findings were assessed. The chi-square test was employed as a statistical test, and P < 0.05 was considered significant. GraphPad Prism 8 was used for statistical analysis.</p><p><strong>Results: </strong>The median time from symptom onset to IL-1i introduction was six months (interquartile range: 2-19). Overall, 55% of patients achieved CID at six months. Early treated patients showed a trend toward higher proportion of CID achievement compared to that of late-treated patients (67% vs 38%; P = 0.17). A meta-analysis including our cohort and two other observational studies showed that early treatment was associated with an odds ratio of 6.73 (95% confidence interval: 2.31-19.64) in comparison to late treatment. All our patients initially received glucocorticoids, with 48% of those achieving CID able to discontinue glucocorticoids within six months. Ten major flares were observed, with 62% occurring after IL-1i suspension or spacing. At baseline, F-fluorodeoxyglucose positron emission tomography (PET)-computed tomography (CT) or magnetic resonance (MR) scans revealed hypermetabolic areas primarily in the bone marrow, spleen, and lymph nodes. After IL-1i introduction, these hypermetabolic regions generally showed a reduction in activity.</p><p><strong>Conclusions: </strong>Early introduction of IL-1i in patients with Still's disease may lead to higher rates of CID achievement and glucocorticoid discontinuation. PET-CT/MR imaging may be valuable in assessing disease activity and guiding treatment decisions. These findings support the concept of a \"window of opportunity\" in Still's disease management, emphasizing the importance of prompt diagnosis and treatment initiation.</p>","PeriodicalId":93845,"journal":{"name":"ACR open rheumatology","volume":"7 10","pages":"e70106"},"PeriodicalIF":2.8,"publicationDate":"2025-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12491934/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145214708","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}
引用次数: 0
Correction to "Gout Remission With Pegloticase-Induced Intensive Urate-Lowering Therapy: A Post Hoc Clinical Trial Analysis". 更正“pegloticase诱导的强化降尿酸治疗缓解痛风:事后临床试验分析”。
IF 2.8
ACR open rheumatology Pub Date : 2025-10-01 DOI: 10.1002/acr2.70128
{"title":"Correction to \"Gout Remission With Pegloticase-Induced Intensive Urate-Lowering Therapy: A Post Hoc Clinical Trial Analysis\".","authors":"","doi":"10.1002/acr2.70128","DOIUrl":"https://doi.org/10.1002/acr2.70128","url":null,"abstract":"","PeriodicalId":93845,"journal":{"name":"ACR open rheumatology","volume":"7 10","pages":"e70128"},"PeriodicalIF":2.8,"publicationDate":"2025-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145240375","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Dual Malignancies in IgG4-Related Disease: A Diagnostic and Therapeutic Challenge. igg4相关疾病的双重恶性肿瘤:诊断和治疗的挑战
IF 2.8
ACR open rheumatology Pub Date : 2025-10-01 DOI: 10.1002/acr2.70114
Ji Li, Ming Zhang, Juanjuan Song, Lina Zhang, Sheng-Guang Li
{"title":"Dual Malignancies in IgG4-Related Disease: A Diagnostic and Therapeutic Challenge.","authors":"Ji Li, Ming Zhang, Juanjuan Song, Lina Zhang, Sheng-Guang Li","doi":"10.1002/acr2.70114","DOIUrl":"10.1002/acr2.70114","url":null,"abstract":"<p><p>We report a rare case of IgG4-related disease (IgG4-RD) presenting with retroperitoneal fibrosis and autoimmune pancreatitis, followed by sequential development of papillary thyroid carcinoma and diffuse large B cell lymphoma. Histopathologic and positron emission tomography-computed tomography findings were essential for diagnosis and guided timely treatment. This case highlights the potential oncogenic link between chronic IgG4-driven inflammation and B cell malignancy, emphasizing the need for vigilant cancer surveillance in patients with IgG4-RD.</p>","PeriodicalId":93845,"journal":{"name":"ACR open rheumatology","volume":"7 10","pages":"e70114"},"PeriodicalIF":2.8,"publicationDate":"2025-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12495398/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145226267","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}
引用次数: 0
Discontinuation of Oral Glucocorticoids and Achievement of Remission in Patients With Eosinophilic Granulomatosis With Polyangiitis Treated With Benralizumab or Mepolizumab. 停止口服糖皮质激素治疗嗜酸性肉芽肿合并多血管炎患者的缓解效果
IF 2.8
ACR open rheumatology Pub Date : 2025-09-01 DOI: 10.1002/acr2.70096
Michael E Wechsler, Nancy Agmon-Levin, David R W Jayne, Christian Pagnoux, Ulrich Specks, Lena Börjesson Sjö, Sofia Necander, Anat Shavit, Claire Walton, Peter A Merkel
{"title":"Discontinuation of Oral Glucocorticoids and Achievement of Remission in Patients With Eosinophilic Granulomatosis With Polyangiitis Treated With Benralizumab or Mepolizumab.","authors":"Michael E Wechsler, Nancy Agmon-Levin, David R W Jayne, Christian Pagnoux, Ulrich Specks, Lena Börjesson Sjö, Sofia Necander, Anat Shavit, Claire Walton, Peter A Merkel","doi":"10.1002/acr2.70096","DOIUrl":"10.1002/acr2.70096","url":null,"abstract":"<p><strong>Objective: </strong>In the phase 3 head-to-head MANDARA study (NCT04157348), benralizumab demonstrated noninferiority to mepolizumab in inducing remission (defined as Birmingham Vasculitis Activity Score [BVAS] of 0 and oral glucocorticoid [OGC] dosage ≤4 mg/day at weeks 36 and 48) in patients with eosinophilic granulomatosis with polyangiitis (EGPA). This analysis investigated a more stringent definition of remission that included discontinuation of OGCs and being relapse-free.</p><p><strong>Methods: </strong>Patients aged ≥18 years with documented relapsing or refractory EGPA receiving OGCs at ≥7.5 mg/day with or without immunosuppressive therapy for ≥4 weeks before enrollment were randomized (1:1) to benralizumab at 30 mg or mepolizumab at 300 mg subcutaneously every 4 weeks for 52 weeks. The proportion of patients achieving remission off OGCs, defined as BVAS of 0, OGC dose of 0 mg/day (at weeks 36 and 48) and no relapses during the double-masked period, was assessed.</p><p><strong>Results: </strong>Patients (n = 140) were randomized to benralizumab (n = 70) or mepolizumab (n = 70). The adjusted percentage of patients with remission off OGCs was 23.5% (n = 16) with benralizumab versus 11.1% (n = 8) with mepolizumab (difference 12.47 [95% confidence interval 0.46-24.48], P = 0.042). Of those who achieved remission off OGCs, 100% of benralizumab-treated patients and 98.6% of mepolizumab-treated patients achieved remission within the first 36 weeks of treatment.</p><p><strong>Conclusion: </strong>The administration of anti-interleukin-5/receptor (IL-5/R) therapies, benralizumab and mepolizumab, enable discontinuation of OGCs in some patients with EGPA, while avoiding relapses. These findings suggest that adding anti-IL-5/R therapy to standard primary treatment for patients with EGPA may improve response.</p>","PeriodicalId":93845,"journal":{"name":"ACR open rheumatology","volume":"7 9","pages":"e70096"},"PeriodicalIF":2.8,"publicationDate":"2025-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12398947/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144982085","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}
引用次数: 0
Impaired Brachial Artery Reactivity in Young Adults With Clinically Quiescent Juvenile Dermatomyositis. 患有临床静止性幼年皮肌炎的年轻成人肱动脉反应性受损。
IF 2.8
ACR open rheumatology Pub Date : 2025-09-01 DOI: 10.1002/acr2.70099
Lauren M Pachman, Gabrielle A Morgan, Claudia E Korcar, Maria Amoruso, James H Stein
{"title":"Impaired Brachial Artery Reactivity in Young Adults With Clinically Quiescent Juvenile Dermatomyositis.","authors":"Lauren M Pachman, Gabrielle A Morgan, Claudia E Korcar, Maria Amoruso, James H Stein","doi":"10.1002/acr2.70099","DOIUrl":"10.1002/acr2.70099","url":null,"abstract":"<p><strong>Objective: </strong>This study characterized brachial artery reactivity, an ultrasound indicator of endothelial function, in young adults who had clinically quiescent juvenile dermatomyositis (JDM) compared with matched controls.</p><p><strong>Methods: </strong>Twenty young adults with previous documentation of JDM 14.2 ± 3.9 years (mean ± SD) after onset of their JDM symptoms, who were no longer taking medications were enrolled, with 20 healthy control participants matched for age, race, sex and body mass index. They obtained ultrasound brachial artery reactivity testing and nailfold end row capillary loop count.</p><p><strong>Results: </strong>Participants were 21.8 ± 4.2 years old (70% female) and were aged 7.6 ± 3.7 years at JDM onset. The JDM Disease Activity Score (DAS) was 1.9 ± 2.5 and primarily based on active skin symptoms. Compared to controls, participants with JDM had fewer end row capillary loops/mm (mean ± SD 6.35 ± 1.29 vs 7.40 ± 0.58; P = 0.002) and were shorter in stature (mean ± SD 163.68 ± 9.69 cm vs 170.49 ± 9.60 cm; P = 0.032). Their end row capillary loop count was associated with their DAS (r = -0.401, P = 0.011). After adjusting for height, age, sex, and resting brachial artery diameter, the brachial artery reactivity in the adults with JDM was significantly lower than that in controls (β = 2.51%, 95% CI -4.62 to -0.40, P = 0.026).</p><p><strong>Conclusion: </strong>This pilot study provides new evidence of endothelial dysfunction, as assessed by brachial artery reactivity, in asymptomatic adults who had classic JDM symptoms in childhood.</p>","PeriodicalId":93845,"journal":{"name":"ACR open rheumatology","volume":"7 9","pages":"e70099"},"PeriodicalIF":2.8,"publicationDate":"2025-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12464089/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145152333","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}
引用次数: 0
Falls Among Individuals With Systemic Lupus Erythematosus: An Observational Study. 系统性红斑狼疮患者跌倒:一项观察性研究。
IF 2.8
ACR open rheumatology Pub Date : 2025-09-01 DOI: 10.1002/acr2.70098
Chelsea R Perfect, C Barrett Bowling, S Sam Lim, Charmayne Dunlop-Thomas, Courtney Hoge, Jinoos Yazdany, Patricia P Katz, Laura C Plantinga
{"title":"Falls Among Individuals With Systemic Lupus Erythematosus: An Observational Study.","authors":"Chelsea R Perfect, C Barrett Bowling, S Sam Lim, Charmayne Dunlop-Thomas, Courtney Hoge, Jinoos Yazdany, Patricia P Katz, Laura C Plantinga","doi":"10.1002/acr2.70098","DOIUrl":"10.1002/acr2.70098","url":null,"abstract":"<p><strong>Objective: </strong>Systemic lupus erythematosus (SLE) is thought to accelerate the aging process. However, there is limited research on geriatric syndromes, such as falls, in this potentially vulnerable population. This study aimed to describe the prevalence of and contributors to falls in the SLE population.</p><p><strong>Methods: </strong>Participants were recruited from an ongoing population-based cohort of individuals with validated SLE. Falls (number of falls and fall-related injuries requiring medical attention over the past year) and perceived contributing factors were self-reported. Descriptive statistics were calculated, and age-, sex-, and race-adjusted odds ratios (aORs) of participant characteristics with falls were estimated using multivariable logistic regression.</p><p><strong>Results: </strong>Nearly one-third (30.7%) of participants (overall N = 447; 40.9% aged ≥50 years, 91.7% female, and 82.6% Black) reported falling in the past year; 19.2% fell twice or more. Loss of balance (78.1% of falls), slipping/tripping (64.2%), and weakness (53.3%) were the most commonly reported contributing factors. Age and sex were not associated with falls, but higher physical performance (aOR, 0.78; 95% confidence interval [CI], 0.71-0.87) was associated with lower odds of falls. Higher SLE activity (aOR, 1.78; 95% CI, 1.44-2.21) and damage (aOR, 1.22; 95% CI, 1.00-1.49), greater depressive symptoms (aOR, 1.43; 95% CI, 1.14,1-80), and taking fall risk-increasing drugs (antidepressants: aOR, 1.82; 95% CI, 1.13-2.93; pain medications: aOR, 2.60; 95% CI, 1.68-4.00; opioids: aOR, 4.52; 95% CI, 2.39-8.56) were associated with higher odds of falls.</p><p><strong>Conclusion: </strong>Falls were common in our cohort, regardless of age. Our results suggest potential interventions for reducing falls, like better control of SLE, physical therapy, depression screening, and medication review.</p>","PeriodicalId":93845,"journal":{"name":"ACR open rheumatology","volume":"7 9","pages":"e70098"},"PeriodicalIF":2.8,"publicationDate":"2025-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12405055/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144982106","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}
引用次数: 0
Preoperative Factors Associated With Falls Following Total Hip Arthroplasty in Older Adults. 老年人全髋关节置换术后跌倒的术前相关因素。
IF 2.8
ACR open rheumatology Pub Date : 2025-09-01 DOI: 10.1002/acr2.70091
Craig P Hensley, Abel N Kho, Andrew Kritselis, Jing Song, Jungwha Lee, Alison H Chang
{"title":"Preoperative Factors Associated With Falls Following Total Hip Arthroplasty in Older Adults.","authors":"Craig P Hensley, Abel N Kho, Andrew Kritselis, Jing Song, Jungwha Lee, Alison H Chang","doi":"10.1002/acr2.70091","DOIUrl":"10.1002/acr2.70091","url":null,"abstract":"<p><strong>Objective: </strong>Falls are common after total hip arthroplasty (THA) and can cause serious complications. Understanding preoperative factors linked to falls post-THA can inform discharge planning and management to lower fall risk. We aimed to identify preoperative factors associated with falls occurring within one year following THA in older adults.</p><p><strong>Methods: </strong>We performed a retrospective cohort study using records within a large hospital system. Adults aged ≥65 years with a recorded THA between March 2012 and March 2022 were included. The primary outcome was a fall occurrence (yes vs no) within one year post-THA. A fall was identified through International Classification of Diseases, Ninth/Tenth Revision codes, documentation for fall-related emergency room visit(s), or mention of a fall in the medical record. Purposeful selection of variables in logistic regression models identified preoperative factors associated with a fall.</p><p><strong>Results: </strong>Among 4,501 patients (age 72.7 ± 6.0 years), 683 (15.2%) experienced at least one fall within one year post-THA. The strongest risk factors were a fall within one year pre-THA (odds ratio [OR] = 2.67, 95% confidence interval [CI] = 2.22-3.22) and depression (OR = 1.95, 95% CI = 1.62-2.35). Other factors included neurologic conditions, insomnia, revision or conversion hip arthroplasty, knee pain, lumbar conditions, body mass index (BMI) <20 kg/m<sup>2</sup>, and older age. A BMI of 25 to 29.9 was protective against falls.</p><p><strong>Conclusion: </strong>This longitudinal cohort study identified multiple independent factors associated with fall occurrences within one year post-THA. Findings highlight the opportunity to address modifiable factors and provide individuals with tailored discharge planning and management strategies (eg, fall prevention programs) to reduce fall risk.</p>","PeriodicalId":93845,"journal":{"name":"ACR open rheumatology","volume":"7 9","pages":"e70091"},"PeriodicalIF":2.8,"publicationDate":"2025-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12423943/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145034837","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}
引用次数: 0
A Tale of Many Canadas: Associations of Ancestry With Juvenile Idiopathic Arthritis Categories and Disease Severity at Presentation to Care. 许多加拿大人的故事:祖先与幼年特发性关节炎类别和疾病严重程度的关系。
IF 2.8
ACR open rheumatology Pub Date : 2025-09-01 DOI: 10.1002/acr2.70092
Stephanie K A Wong, Lori B Tucker, Kristin Houghton, David A Cabral, Mercedes Chan, Kimberly A Morishita, Rae S M Yeung, Kiem Oen, Ciaran M Duffy, Roberta A Berard, Gaëlle Chédeville, Thomas Loughin, Jaime Guzman
{"title":"A Tale of Many Canadas: Associations of Ancestry With Juvenile Idiopathic Arthritis Categories and Disease Severity at Presentation to Care.","authors":"Stephanie K A Wong, Lori B Tucker, Kristin Houghton, David A Cabral, Mercedes Chan, Kimberly A Morishita, Rae S M Yeung, Kiem Oen, Ciaran M Duffy, Roberta A Berard, Gaëlle Chédeville, Thomas Loughin, Jaime Guzman","doi":"10.1002/acr2.70092","DOIUrl":"10.1002/acr2.70092","url":null,"abstract":"<p><strong>Objective: </strong>To assess associations of ancestry with juvenile idiopathic arthritis (JIA) categories and clinical Juvenile Arthritis Disease Activity Scores (cJADAS10) at presentation to pediatric rheumatology care in a multicultural country with universal health care.</p><p><strong>Methods: </strong>Parents reported their child's ancestry in the Research in Arthritis in Canadian Children Emphasizing Outcomes (ReACCh-Out) cohort. For each ancestry reported for ≥30 children, we compared JIA category distribution and median cJADAS10 scores among three groups: only that ancestry, with that and other ancestries, and without that ancestry. Chi-square, Fisher's exact, and Kruskal-Wallis tests compared the three groups and multivariable linear regression assessed factors associated with cJADAS10 scores.</p><p><strong>Results: </strong>Among 1,407 participants, 629 (44.7%) reported more than one ancestry. British ancestry was associated with higher median cJADAS10 scores (7.5) and higher frequency of enthesitis-related arthritis (18.7%) and psoriatic arthritis (10.0%), French ancestry was associated with lower cJADAS10 scores (5.8) and higher oligoarthritis (51.2%), Indigenous ancestry was associated with higher cJADAS10 scores (11.0) and higher rheumatoid factor-positive polyarthritis (21.9%), Black ancestry was associated with higher rheumatoid factor-positive polyarthritis (16.0%), and Eastern European ancestry was associated with lower cJADAS10 scores (3.6). Associations of ancestry with cJADAS10 scores were largely explained by differences in JIA categories (total R<sup>2</sup> = 0.28, with R<sup>2</sup> = 0.25 for JIA category alone). Black ancestry was associated with longer time from symptom onset to diagnosis (27 vs 18.9 weeks).</p><p><strong>Conclusions: </strong>British and French ancestries had distinct associations with JIA categories and cJADAS10 scores, and many children had multiple ancestries, questioning the use of a single \"European\" reference group. Higher cJADAS10 scores were largely explained by differences in JIA categories across ancestries.</p>","PeriodicalId":93845,"journal":{"name":"ACR open rheumatology","volume":"7 9","pages":"e70092"},"PeriodicalIF":2.8,"publicationDate":"2025-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12399783/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144982063","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}
引用次数: 0
Anti-RuvBL1/2 Antibodies in Scleromyositis: A Clinicopathological Report of Two Cases. 抗ruvbl1 /2抗体在硬化肌炎中的应用:2例临床病理报告
IF 2.8
ACR open rheumatology Pub Date : 2025-09-01 DOI: 10.1002/acr2.70113
Yuki Imai, Masaru Takeshita, Yasushi Kondo, Jun Kikuchi, Junko Kuramoto, Shiro Matsubara, Yuko Kaneko
{"title":"Anti-RuvBL1/2 Antibodies in Scleromyositis: A Clinicopathological Report of Two Cases.","authors":"Yuki Imai, Masaru Takeshita, Yasushi Kondo, Jun Kikuchi, Junko Kuramoto, Shiro Matsubara, Yuko Kaneko","doi":"10.1002/acr2.70113","DOIUrl":"10.1002/acr2.70113","url":null,"abstract":"<p><p>We report two patients with scleromyositis with anti-RuvBL1/2 antibodies, recently identified rare autoantibodies associated with older age at onset, diffuse sclerodactyly, skeletal and cardiac myositis, and interstitial lung disease (ILD). Both patients showed sclerodactyly and skeletal myositis. Muscle biopsy revealed lymphocytes and macrophage infiltration in an 83-year-old woman with cardiac involvement and immune-mediated necrotizing myopathy in a 68-year-old woman with ILD, guiding diagnosis and treatment decisions. Glucocorticoids and immunosuppressants led to clinical improvement. These cases highlight the clinical and pathologic spectrum of anti-RuvBL1/2 antibody-associated disease and the value of antibody detection and tissue analysis.</p>","PeriodicalId":93845,"journal":{"name":"ACR open rheumatology","volume":"7 9","pages":"e70113"},"PeriodicalIF":2.8,"publicationDate":"2025-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12423767/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145034903","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}
引用次数: 0
Limited Utility of Screening Electrocardiograms in Systemic Sclerosis: Data from the Canadian Scleroderma Research Group. 心电图筛查在系统性硬化症中的有限应用:来自加拿大硬皮病研究小组的数据。
IF 2.8
ACR open rheumatology Pub Date : 2025-09-01 DOI: 10.1002/acr2.70030
Sophie Wojcik, Christos Galatas, Alaa Dekis, Licia Iacoviello, Augusto Di Castelnuovo, Simona Costanzo, Mianbo Wang, Marvin J Fritzler, Marie Hudson, Thao Huynh, Murray Baron
{"title":"Limited Utility of Screening Electrocardiograms in Systemic Sclerosis: Data from the Canadian Scleroderma Research Group.","authors":"Sophie Wojcik, Christos Galatas, Alaa Dekis, Licia Iacoviello, Augusto Di Castelnuovo, Simona Costanzo, Mianbo Wang, Marvin J Fritzler, Marie Hudson, Thao Huynh, Murray Baron","doi":"10.1002/acr2.70030","DOIUrl":"10.1002/acr2.70030","url":null,"abstract":"<p><strong>Objective: </strong>Recommendations have been made to use electrocardiograms (EKGs) to screen for cardiac disease in systemic sclerosis (SSc). The objective of this study was to compare the prevalence of EKG abnormalities in SSc and controls to help determine if the EKG should be used as a screening tool.</p><p><strong>Methods: </strong>EKGs from patients with SSc were compared with those from a random sample of age- and gender-matched controls. Two cardiologists read all EKGs using a standardized approach. The groups were compared using t-tests, chi-squared tests, and Fisher exact tests.</p><p><strong>Results: </strong>Patients with SSc (n = 833, mean ± SD disease duration 11.3 ± 9.3 years; 39.4% had diffuse cutaneous SSc) and controls (n = 832) were included. The prevalence of conduction and rhythm abnormalities were similar in the SSc and control groups. More patients with SSc than controls had possible right atrial enlargement (5% vs 0.1%, P < 0.001), right axis deviation (3.2% vs 0.4%, P < 0.001), left atrial enlargement (9.2% vs 1.6%, P < 0.001), poor/abnormal R progression (5.6% vs 2.2%, P < 0.001) and nonspecific T wave abnormalities (6.1% vs 3.4%, P = 0.008).</p><p><strong>Conclusion: </strong>Our findings suggest that conduction abnormalities are not more prevalent in those with SSc than in controls. Evidence of right heart stress on EKG in SSc may be secondary to pulmonary hypertension and left atrial enlargement, and poor R wave progression in precordial leads may indicate myocardial damage. Future studies are required to determine if these EKG abnormalities represent underlying structural heart disease, and, until that is proven, EKGs should not be considered a screening tool for cardiac abnormalities in SSc.</p>","PeriodicalId":93845,"journal":{"name":"ACR open rheumatology","volume":"7 9","pages":"e70030"},"PeriodicalIF":2.8,"publicationDate":"2025-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12440803/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145076942","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}
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