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A case of VEXAS syndrome presenting with unusual bone marrow granulomas: a diagnostic dilemma. 一例伴有异常骨髓肉芽肿的VEXAS综合征:诊断困境。
IF 2.2 Q3 RHEUMATOLOGY Pub Date : 2023-07-10 DOI: 10.1186/s41927-023-00343-w
Khiem T Vu, Rachel M Wolfe, Jonathan E Lambird, Danielle L V Maracaja

Background: VEXAS is a recently described inflammatory disease caused by mutations in the UBA1 gene. Symptoms are diverse and include fevers, cartilaginous inflammation, lung inflammation, vasculitis, neutrophilic dermatoses, and macrocytic anemia. Cytoplasmic inclusions in myeloid and erythroid progenitors in the bone marrow are a hallmark feature. Here we report the first case of VEXAS with non-caseating granulomas in the bone marrow.

Case presentation: A 62-year-old Asian male presented with fevers, erythema nodosum, inflammatory arthritis, and periorbital inflammation. Labs were significant for persistently elevated inflammatory markers and macrocytic anemia. Over the years his symptoms and inflammatory markers only improved with glucocorticoids and recurred when prednisone dose was lowered below 15-20 mg daily. He underwent bone marrow biopsy showing non-caseating granulomas and PET scan showing hilar/mediastinal lymphadenopathy. He was initially diagnosed with IgG4-related disease (treated with rituximab) and later sarcoidosis (treated with infliximab). After failing these agents, the possibility of VEXAS was considered and later confirmed by molecular testing.

Conclusions: To the best of our knowledge, this is the first observation of non-caseating granulomas in VEXAS, a cautionary reminder of its non-specificity since misinterpretation can lead to diagnostic delay. VEXAS should be in the differential in patients with symptoms of chronic inflammation responding positively to steroids (but not to B-cell depletion or TNF inhibition), which is in line with previous literature.

背景:VEXAS是最近发现的由UBA1基因突变引起的炎症性疾病。症状多种多样,包括发热、软骨炎症、肺部炎症、血管炎、中性粒细胞性皮肤病和大细胞性贫血。骨髓中髓系和红系祖细胞的细胞质包涵体是一个标志性的特征。我们在此报告首例伴骨髓非干酪化肉芽肿的VEXAS病例。病例介绍:一名62岁亚洲男性,表现为发热、结节性红斑、炎症性关节炎和眶周炎症。实验室显示炎症标志物持续升高和大细胞性贫血。多年来,他的症状和炎症指标仅在使用糖皮质激素时得到改善,当泼尼松剂量降至每日15- 20mg以下时复发。骨髓活检显示非干酪样肉芽肿,PET扫描显示肺门/纵隔淋巴结病变。他最初被诊断为igg4相关疾病(用利妥昔单抗治疗),后来被诊断为结节病(用英夫利昔单抗治疗)。在这些药物失败后,考虑了VEXAS的可能性,后来通过分子检测证实了这一点。结论:据我们所知,这是在VEXAS中首次观察到非干酪化肉芽肿,这是对其非特异性的警示,因为误解可能导致诊断延迟。有慢性炎症症状的患者对类固醇反应阳性(但对b细胞耗竭或TNF抑制反应不阳性),这与先前的文献一致。
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引用次数: 0
Physical function and severe side effects matter most to patients with RA (< 5 years): a discrete choice experiment assessing preferences for personalized RA treatment. 身体功能和严重的副作用对RA患者(< 5年)最重要:一项评估个性化RA治疗偏好的离散选择实验。
IF 2.2 Q3 RHEUMATOLOGY Pub Date : 2023-07-03 DOI: 10.1186/s41927-023-00341-y
Karin Schölin Bywall, Bente Appel Esbensen, Marie Heidenvall, Inger Erlandsson, Marta Lason, Mats Hansson, Jennifer Viberg Johansson

Aim: Early assessment of patient preferences has the potential to support shared decisions in personalized precision medicine for patients with rheumatoid arthritis (RA). The aim of this study was to assess treatment preferences of patients with RA (< 5 years) with previous experience of inadequate response to first-line monotherapy.

Method: Patients were recruited (March-June 2021) via four clinics in Sweden. Potential respondents (N = 933) received an invitation to answer a digital survey. The survey included an introductory part, a discrete choice experiment (DCE) and demographic questions. Each respondent answered 11 hypothetical choice questions as part of the DCE. Patient preferences and preference heterogeneity were estimated using random parameter logit models and latent class analysis models.

Results: Patients (n = 182) assessed the most important treatment attributes out of physical functional capacity, psychosocial functional capacity, frequency of mild side effects and likelihood of severe side effects. In general, patients preferred a greater increase in functional capacity and decreased side effects. However, a substantial preference heterogeneity was identified with two underlying preference patterns. The most important attribute in the first pattern was the 'likelihood of getting a severe side effect'. Physical functional capacity was the most important attribute in the second pattern.

Conclusion: Respondents focused their decision-making mainly on increasing their physical functional capacity or decreasing the likelihood of getting a severe side effect. These results are highly relevant from a clinical perspective to strengthen communication in shared decision making by assessing patients' individual preferences for benefits and risks in treatment discussions.

目的:对患者偏好的早期评估有可能支持类风湿关节炎(RA)患者个性化精准医疗的共同决策。本研究的目的是评估RA患者的治疗偏好(方法:通过瑞典的四个诊所招募患者(2021年3月至6月)。潜在的受访者(N = 933)收到了回答数字调查的邀请。该调查包括一个介绍部分,一个离散选择实验(DCE)和人口统计问题。作为DCE的一部分,每位受访者都要回答11个假设性选择题。使用随机参数logit模型和潜在类分析模型估计患者偏好和偏好异质性。结果:患者(n = 182)评估了最重要的治疗属性,包括身体功能能力、社会心理功能能力、轻微副作用的频率和严重副作用的可能性。一般来说,患者更倾向于功能能力的提高和副作用的减少。然而,一个实质性的偏好异质性被确定为两个潜在的偏好模式。第一种模式中最重要的属性是“产生严重副作用的可能性”。身体功能能力是第二种模式中最重要的属性。结论:受访者的决策主要集中在提高他们的身体功能能力或减少产生严重副作用的可能性。从临床角度来看,通过评估患者在治疗讨论中的利益和风险偏好,加强共同决策中的沟通,这些结果具有高度相关性。
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引用次数: 0
Determinants of health-related quality of life among patients with systemic lupus erythematosus in Hanoi, Vietnam. 越南河内系统性红斑狼疮患者健康相关生活质量的决定因素
IF 2.2 Q3 RHEUMATOLOGY Pub Date : 2023-06-22 DOI: 10.1186/s41927-023-00339-6
Aya Mizukami, Minh Trang Trinh, Thi Phuong Hoang, Akira Shibanuma, Ken Ing Cherng Ong, Masamine Jimba

Background: Systemic lupus erythematosus (SLE) is a chronic autoimmune disease which impacts patients' lives. Many studies in high-income countries have focused on their health-related quality of life (HRQoL). However, evidence of awareness of SLE and HRQoL in low- and middle-income countries is lacking. Therefore, this study aimed to identify the determinants of HRQoL of SLE patients in Vietnam, a lower-middle income country.

Methods: This cross-sectional study was conducted at the National Hospital of Dermatology and Venereology in 2019. A pre-tested structured questionnaire was used to collect data. It consisted of Short Form-36 to assess HRQoL which comprised physical and mental component summaries, Multidimensional Scale of Perceived Social Support, Satisfaction with Life Scale, and Mental Adjustment to SLE. Multiple linear regression was used to identify the determinants of HRQoL.

Results: One hundred thirty four patients with SLE participated in this study. The majority of the patients were women (n = 126, 94.0%). The mean age of all participants was 37.9 years old (standard deviation [SD] 12.5). Of 134 participants, 104 (77.6%) were married. Older patients were more likely to have a lower score of mental component summary (B=-0.45, 95% CI -0.73, -0.17). Patients with more children were more likely to have a lower score of physical component summary (B=-5.14, 95% CI -9.27, -1.00). Patients who felt more helplessness or hopelessness were more likely to have lower scores of physical and mental component summaries (B=-1.85, 95% CI -2.80, -0.90; B=-1.69, 95% CI -2.57, -0.81). Also, patients who felt more anxious were more likely to have a lower score of mental component summary (B=-1.04, 95% CI -1.77, -0.32). Patients who were more satisfied with their lives were more likely to have higher scores of physical and mental component summaries (B = 1.07, 95% CI 0.50, 1.64; B = 1.08, 95% CI 0.55, 1.61).

Conclusion: Factors associated with lower HRQoL in Vietnam were feelings of helplessness or hopelessness, and burdens of parenting roles. However, social support can contribute to a higher HRQoL, such as information support, self-support groups, and daycare services provided at the community level.

背景:系统性红斑狼疮(SLE)是一种影响患者生活的慢性自身免疫性疾病。在高收入国家进行的许多研究侧重于他们与健康有关的生活质量(HRQoL)。然而,低收入和中等收入国家缺乏对SLE和HRQoL认识的证据。因此,本研究旨在确定越南这个中低收入国家SLE患者HRQoL的决定因素。方法:本横断面研究于2019年在国立皮肤性病医院进行。采用预先测试的结构化问卷收集数据。HRQoL由短表36评估,包括身体和心理部分总结、感知社会支持多维度量表、生活满意度量表和SLE心理适应。采用多元线性回归确定HRQoL的影响因素。结果:134例SLE患者参与了本研究。患者以女性为主(126例,占94.0%)。所有参与者的平均年龄为37.9岁(标准差[SD] 12.5)。在134名参与者中,有104人(77.6%)已婚。老年患者的心理成分总结得分较低(B=-0.45, 95% CI -0.73, -0.17)。孩子多的患者身体成分总结得分越低(B=-5.14, 95% CI -9.27, -1.00)。感到更无助或绝望的患者更有可能在身体和精神成分总结中得分较低(B=-1.85, 95% CI -2.80, -0.90;B=-1.69, 95% ci -2.57, -0.81)。此外,焦虑程度越高的患者心理成分总结得分越低(B=-1.04, 95% CI -1.77, -0.32)。生活满意度越高的患者,身体和精神成分总结得分越高(B = 1.07, 95% CI 0.50, 1.64;B = 1.08, 95% ci 0.55, 1.61)。结论:越南儿童HRQoL较低的相关因素是无助感或绝望感和父母角色负担。然而,社会支持有助于提高HRQoL,如信息支持、自我支持小组和社区层面提供的日托服务。
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引用次数: 0
What are the core recommendations for gout management in first line and specialist care? Systematic review of clinical practice guidelines. 在一线和专科护理中,痛风管理的核心建议是什么?临床实践指南的系统回顾。
IF 2.1 Q3 RHEUMATOLOGY Pub Date : 2023-06-15 DOI: 10.1186/s41927-023-00335-w
Brooke Conley, Samantha Bunzli, Jonathan Bullen, Penny O'Brien, Jennifer Persaud, Tilini Gunatillake, Michelle M Dowsey, Peter F Choong, Mandana Nikpour, Rebecca Grainger, Ivan Lin

Background: Gout is the most common inflammatory arthritis, increasing in prevalence and burden. Of the rheumatic diseases, gout is the best-understood and potentially most manageable condition. However, it frequently remains untreated or poorly managed. The purpose of this systematic review is to identify Clinical Practice Guidelines (CPG) regarding gout management, evaluate their quality, and to provide a synthesis of consistent recommendations in the high-quality CPGs.

Methods: Gout management CPGs were eligible for inclusion if they were (1) written in English and published between January 2015-February 2022; focused on adults aged ≥ 18 years of age; and met the criteria of a CPG as defined by the Institute of Medicine; and (2) were rated as high quality on the Appraisal of Guidelines for Research and Evaluation (AGREE) II instrument. Gout CPGs were excluded if they required additional payment to access; only addressed recommendations for the system/organisation of care and did not include interventional management recommendations; and/or included other arthritic conditions. OvidSP MEDLINE, Cochrane, CINAHL, Embase and Physiotherapy Evidence Database (PEDro) and four online guideline repositories were searched.

Results: Six CPGs were appraised as high quality and included in the synthesis. Clinical practice guidelines consistently recommended education, commencement of non-steroidal anti-inflammatories, colchicine or corticosteroids (unless contraindicated), and assessment of cardiovascular risk factors, renal function, and co-morbid conditions for acute gout management. Consistent recommendations for chronic gout management were urate lowering therapy (ULT) and continued prophylaxis recommended based on individual patient characteristics. Clinical practice guideline recommendations were inconsistent on when to initiate ULT and length of ULT, vitamin C intake, and use of pegloticase, fenofibrate and losartan.

Conclusion: Management of acute gout was consistent across CPGs. Management of chronic gout was mostly consistent although there were inconsistent recommendations regarding ULT and other pharmacological therapies. This synthesis provides clear guidance that can assist health professionals to provide standardised, evidence-based gout care.

Trial registration: The protocol for this review was registered with Open Science Framework (DOI https://doi.org/10.17605/OSF.IO/UB3Y7 ).

背景:痛风是最常见的炎症性关节炎,患病率和负担都在增加。在风湿性疾病中,痛风是最容易理解的,也可能是最容易控制的疾病。然而,它经常得不到治疗或管理不善。本系统综述的目的是确定有关痛风管理的临床实践指南(CPG),评估其质量,并在高质量的CPG中提供一致建议的综合;专注于老年人 ≥ 18岁;并符合医学研究所定义的CPG标准;和(2)在研究与评估指南评估(AGREE)II工具中被评为高质量。如果Gout CPG需要额外付费才能访问,则将其排除在外;仅针对护理系统/组织提出建议,不包括介入管理建议;和/或包括其他关节炎状况。检索OvidSP MEDLINE、Cochrane、CINAHL、Embase和物理治疗证据数据库(PEDro)和四个在线指南库。结果:6个CPG被鉴定为高质量,并被纳入合成。临床实践指南一贯建议进行教育,开始服用非甾体抗炎药、秋水仙碱或皮质类固醇(除非有禁忌症),并评估心血管风险因素、肾功能和合并疾病,以进行急性痛风治疗。慢性痛风管理的一致建议是降低尿酸盐治疗(ULT),并根据个别患者的特点建议继续预防。临床实践指南建议在何时开始ULT、ULT的持续时间、维生素C的摄入以及使用pegloticase、非诺贝特和氯沙坦方面不一致。结论:不同CPG对急性痛风的治疗是一致的。慢性痛风的治疗基本上是一致的,尽管关于ULT和其他药物治疗的建议不一致。这一综合提供了明确的指导,可以帮助卫生专业人员提供标准化的、循证的痛风护理。试验注册:本次审查的方案已在开放科学框架(DOIhttps://doi.org/10.17605/OSF.IO/UB3Y7)。
{"title":"What are the core recommendations for gout management in first line and specialist care? Systematic review of clinical practice guidelines.","authors":"Brooke Conley, Samantha Bunzli, Jonathan Bullen, Penny O'Brien, Jennifer Persaud, Tilini Gunatillake, Michelle M Dowsey, Peter F Choong, Mandana Nikpour, Rebecca Grainger, Ivan Lin","doi":"10.1186/s41927-023-00335-w","DOIUrl":"10.1186/s41927-023-00335-w","url":null,"abstract":"<p><strong>Background: </strong>Gout is the most common inflammatory arthritis, increasing in prevalence and burden. Of the rheumatic diseases, gout is the best-understood and potentially most manageable condition. However, it frequently remains untreated or poorly managed. The purpose of this systematic review is to identify Clinical Practice Guidelines (CPG) regarding gout management, evaluate their quality, and to provide a synthesis of consistent recommendations in the high-quality CPGs.</p><p><strong>Methods: </strong>Gout management CPGs were eligible for inclusion if they were (1) written in English and published between January 2015-February 2022; focused on adults aged ≥ 18 years of age; and met the criteria of a CPG as defined by the Institute of Medicine; and (2) were rated as high quality on the Appraisal of Guidelines for Research and Evaluation (AGREE) II instrument. Gout CPGs were excluded if they required additional payment to access; only addressed recommendations for the system/organisation of care and did not include interventional management recommendations; and/or included other arthritic conditions. OvidSP MEDLINE, Cochrane, CINAHL, Embase and Physiotherapy Evidence Database (PEDro) and four online guideline repositories were searched.</p><p><strong>Results: </strong>Six CPGs were appraised as high quality and included in the synthesis. Clinical practice guidelines consistently recommended education, commencement of non-steroidal anti-inflammatories, colchicine or corticosteroids (unless contraindicated), and assessment of cardiovascular risk factors, renal function, and co-morbid conditions for acute gout management. Consistent recommendations for chronic gout management were urate lowering therapy (ULT) and continued prophylaxis recommended based on individual patient characteristics. Clinical practice guideline recommendations were inconsistent on when to initiate ULT and length of ULT, vitamin C intake, and use of pegloticase, fenofibrate and losartan.</p><p><strong>Conclusion: </strong>Management of acute gout was consistent across CPGs. Management of chronic gout was mostly consistent although there were inconsistent recommendations regarding ULT and other pharmacological therapies. This synthesis provides clear guidance that can assist health professionals to provide standardised, evidence-based gout care.</p><p><strong>Trial registration: </strong>The protocol for this review was registered with Open Science Framework (DOI https://doi.org/10.17605/OSF.IO/UB3Y7 ).</p>","PeriodicalId":9150,"journal":{"name":"BMC Rheumatology","volume":"7 1","pages":"15"},"PeriodicalIF":2.1,"publicationDate":"2023-06-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10268528/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9630732","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
Prediction of risk of ischemic heart disease in first-diagnosed systemic lupus erythematosus patients in taiwan: is air pollution exposure a risk factor? 台湾初诊系统性红斑狼疮患者缺血性心脏病风险预测:空气污染暴露是危险因素吗?
IF 2.2 Q3 RHEUMATOLOGY Pub Date : 2023-06-08 DOI: 10.1186/s41927-023-00337-8
Pei-Yun Chen, Yu-Tse Tsan, Chao-Tung Yang, Yun-Mei Lee, Li-Li Chen, Wen-Chao Ho, Shu-Hua Lu

Background: Air pollution is a key public health factor with the capacity to induce diseases. The risk of ischemia heart disease (IHD) in those suffering from systemic lupus erythematosus (SLE) from air pollution exposure is ambiguous. This study aimed to: (1) determine the hazard ratio (HR) of IHD after the first-diagnosed SLE and (2) examine the effects of air pollution exposure on IHD in SLE for 12 years.

Methods: This is a retrospective cohort study. Taiwan's National Health Insurance Research Database and Taiwan Air Quality Monitoring data were used in the study. Cases first diagnosed with SLE in 2006 cases without IHD were recruited as the SLE group. We randomly selected an additional sex-matched non-SLE cohort, four times the size of the SLE cohort, as the control group. Air pollution indices by residence city per period were calculated as the exposure. Life tables and Cox proportional risk models of time-dependent covariance were used in the research.

Results: This study identified patients for the SLE group (n = 4,842) and the control group (n = 19,368) in 2006. By the end of 2018, the risk of IHD was significantly higher in the SLE group than in the control group, and risks peaked between the 6th and 9th year. The HR of incidence IHD in the SLE group was 2.42 times that of the control group. Significant correlations with risk of developing IHD were noted for sex, age, CO, NO2, PM10, and PM2.5, of which PM10 exposure had the highest risk of IHD incidence.

Conclusions: Subjects with SLE were at a higher risk of IHD, especially those in the 6th to 9th year after SLE diagnosis. The advanced cardiac health examinations and health education plan should be recommended for SLE patients before the 6th year after SLE diagnosed.

背景:空气污染是一个重要的公共卫生因素,具有诱发疾病的能力。暴露于空气污染的系统性红斑狼疮(SLE)患者患缺血性心脏病(IHD)的风险尚不明确。本研究旨在:(1)确定首次诊断SLE后IHD的危险比(HR),(2)研究12年的SLE空气污染暴露对IHD的影响。方法:回顾性队列研究。本研究采用台湾全民健保研究资料库及台湾空气品质监测资料。2006年首次诊断为SLE的无IHD患者被纳入SLE组。我们随机选择了另外一个性别匹配的非SLE队列,其规模是SLE队列的四倍,作为对照组。以居住城市为单位计算各时期空气污染指数作为暴露量。研究采用生命表和Cox时间相关协方差比例风险模型。结果:本研究确定了2006年SLE组(n = 4,842)和对照组(n = 19,368)的患者。截至2018年底,SLE组的IHD风险明显高于对照组,风险在第6年至第9年达到峰值。SLE组IHD发生率的HR是对照组的2.42倍。性别、年龄、CO、NO2、PM10和PM2.5与IHD发病风险显著相关,其中PM10暴露与IHD发病风险最高。结论:SLE患者发生IHD的风险较高,尤其是SLE诊断后6 - 9年的患者。SLE患者在确诊后6年前应进行心脏健康检查和健康教育计划。
{"title":"Prediction of risk of ischemic heart disease in first-diagnosed systemic lupus erythematosus patients in taiwan: is air pollution exposure a risk factor?","authors":"Pei-Yun Chen,&nbsp;Yu-Tse Tsan,&nbsp;Chao-Tung Yang,&nbsp;Yun-Mei Lee,&nbsp;Li-Li Chen,&nbsp;Wen-Chao Ho,&nbsp;Shu-Hua Lu","doi":"10.1186/s41927-023-00337-8","DOIUrl":"https://doi.org/10.1186/s41927-023-00337-8","url":null,"abstract":"<p><strong>Background: </strong>Air pollution is a key public health factor with the capacity to induce diseases. The risk of ischemia heart disease (IHD) in those suffering from systemic lupus erythematosus (SLE) from air pollution exposure is ambiguous. This study aimed to: (1) determine the hazard ratio (HR) of IHD after the first-diagnosed SLE and (2) examine the effects of air pollution exposure on IHD in SLE for 12 years.</p><p><strong>Methods: </strong>This is a retrospective cohort study. Taiwan's National Health Insurance Research Database and Taiwan Air Quality Monitoring data were used in the study. Cases first diagnosed with SLE in 2006 cases without IHD were recruited as the SLE group. We randomly selected an additional sex-matched non-SLE cohort, four times the size of the SLE cohort, as the control group. Air pollution indices by residence city per period were calculated as the exposure. Life tables and Cox proportional risk models of time-dependent covariance were used in the research.</p><p><strong>Results: </strong>This study identified patients for the SLE group (n = 4,842) and the control group (n = 19,368) in 2006. By the end of 2018, the risk of IHD was significantly higher in the SLE group than in the control group, and risks peaked between the 6th and 9th year. The HR of incidence IHD in the SLE group was 2.42 times that of the control group. Significant correlations with risk of developing IHD were noted for sex, age, CO, NO<sub>2</sub>, PM<sub>10</sub>, and PM<sub>2.5</sub>, of which PM<sub>10</sub> exposure had the highest risk of IHD incidence.</p><p><strong>Conclusions: </strong>Subjects with SLE were at a higher risk of IHD, especially those in the 6th to 9th year after SLE diagnosis. The advanced cardiac health examinations and health education plan should be recommended for SLE patients before the 6th year after SLE diagnosed.</p>","PeriodicalId":9150,"journal":{"name":"BMC Rheumatology","volume":"7 1","pages":"14"},"PeriodicalIF":2.2,"publicationDate":"2023-06-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10249222/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9596775","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
Radiographic involvement of cervical facet joints in ankylosing spondylitis: a longitudinal analysis in correlation with vertebral body lesions. 强直性脊柱炎患者颈椎面关节的影像学受累情况:与椎体病变相关的纵向分析。
IF 2.1 Q3 RHEUMATOLOGY Pub Date : 2023-06-07 DOI: 10.1186/s41927-023-00334-x
Tae-Han Lee, Seunghun Lee, Bon San Koo, Kyung Bin Joo, Tae-Hwan Kim

Background: The inability to assess structural changes in facet joints is a limitation of established radiographic scoring systems for ankylosing spondylitis (AS). We compared radiographic evidence of ankylosis in cervical facet joints and cervical vertebral bodies in patients with AS.

Methods: We analysed longitudinal data collected from 1106 AS patients and assessed 4984 spinal radiographs obtained up to 16 years of follow-up. Comparisons between cervical facet joints and cervical vertebral bodies focused on the presence of ankylosis, which was defined by at least one facet joint exhibiting complete ankylosis (according to the method of de Vlam) or at least one vertebral body with a bridging syndesmophyte (according to the modified Stoke Ankylosing Spondylitis Spinal Score [mSASSS]). Ankylosis was assessed over time using spinal radiographs collected during follow-up periods stratified in 4-year increments.

Results: Patients with cervical facet joint ankylosis had higher cervical mSASSS, sacroiliitis grades, and inflammatory markers, with more prevalent hip involvement and uveitis. Overall, the numbers of spinal radiographs indicating ankylosis were comparable between cervical facet joints (17.8%) and cervical vertebral bodies (16.8%), and they usually presented together (13.5%). We observed similar proportions of radiographs with ankylosis only in cervical facet joints (4.3%) and cervical vertebral bodies (3.3%). As damage progressed, configurations with both cervical facet joint ankylosis and bridging syndesmophytes became more predominant with longer follow-up times, while configurations with cervical facet joint ankylosis only or bridging syndesmophytes only were less frequently observed.

Conclusions: Evidence of cervical facet joint ankylosis appears as often as bridging syndesmophytes on routine AS spinal radiographs. Presence of cervical facet joint ankylosis should be considered because it may have a higher disease burden.

背景:强直性脊柱炎(AS)既有的放射学评分系统无法评估面关节的结构变化,这是一个局限性。我们比较了强直性脊柱炎患者颈椎面关节和颈椎体强直的影像学证据:我们分析了从1106名强直性脊柱炎患者中收集的纵向数据,并对随访16年的4984张脊柱X光片进行了评估。颈椎面关节和颈椎体之间的比较主要集中在是否存在强直,强直的定义是至少有一个面关节表现出完全强直(根据 de Vlam 的方法)或至少有一个椎体出现桥接联合骨赘(根据修改后的斯托克强直性脊柱炎脊柱评分[mSASSS])。随访期间收集的脊柱X光片对强直情况进行评估,每4年为一个阶段:结果:颈椎切面关节强直的患者颈椎 mSASSS、骶髂关节炎等级和炎症标志物较高,髋关节受累和葡萄膜炎的发病率较高。总体而言,颈椎面关节(17.8%)和颈椎椎体(16.8%)的脊柱X光片显示强直的数量相当,而且它们通常同时出现(13.5%)。我们观察到,仅在颈椎面关节(4.3%)和颈椎体(3.3%)出现强直的 X 光片比例相似。随着损伤的进展,随访时间越长,同时伴有颈椎面关节强直和桥状联合韧带增生的病例越多,而仅伴有颈椎面关节强直或桥状联合韧带增生的病例较少:结论:在AS脊柱常规X光片上,颈椎面关节强直的证据与桥状联合骨赘一样常见。颈椎面关节强直可能会造成更大的疾病负担,因此应予以重视。
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引用次数: 0
Attitudes and beliefs towards COVID-19 and COVID-19 vaccination among rheumatology patients in a Los Angeles County safety net clinic. 洛杉矶县安全网诊所风湿病患者对COVID-19和COVID-19疫苗接种的态度和信念
IF 2.2 Q3 RHEUMATOLOGY Pub Date : 2023-06-01 DOI: 10.1186/s41927-023-00338-7
Nicole K Zagelbaum Ward, Suman Pal, Katherine Ruddy, Stavros Savvas

Background: The novel Coronavirus disease (COVID-19) pandemic has represented an evolving global threat with high morbidity and mortality. Patients with autoimmune rheumatic diseases and on immune-suppressing medications may be at increased risk to more severe disease, hospitalization, and death. Vaccines are essential to combat the COVID-19 pandemic and curb the spread of infection. Rheumatology patients may be more fearful to receive the vaccine compared to the general population. The Los Angeles County rheumatology patients are primarily Hispanic and represent a unique and possibly particularly vulnerable cohort warranting further exploration into barriers to receive the COVID-19 vaccine. We aimed to explore the willingness of COVID-19 vaccine acceptance among patients with rheumatic disease.

Methods: We conducted a cross-sectional survey to assess the perceptions and barriers to COVID-19 vaccine acceptance in our Los Angeles County rheumatology clinics between July 2021 to September 2021 and received responses from 116 patients.

Results: The majority of respondents were female (83.9%), 41-60 years of age (59.8%), Hispanic (89.2%), with high school or lower level of education (68.7%), and had Rheumatoid Arthritis (56.9%). We found most (88.4%) patients received at least one dose of the COVID-19 vaccine. We identified no differences in vaccine acceptance related to age, education, race, and ethnicity. Most respondents agreed that their health condition puts them at high risk of COVID-19 complications. In addition, individuals reported that they valued being engaged by their rheumatologists in discussions of the risk and benefits of the vaccine prior to receiving it.

Conclusion: We found that the majority of patients were already vaccinated or willing to be vaccinated, at higher levels than general United States population and that a conversation initiated by a rheumatologist can have positive effect on patients' health behaviors related to COVID-19.

背景:新型冠状病毒病(COVID-19)大流行是一种不断演变的全球威胁,具有高发病率和高死亡率。自身免疫性风湿病患者和使用免疫抑制药物的患者可能面临更严重疾病、住院和死亡的风险增加。疫苗对于抗击COVID-19大流行和遏制感染传播至关重要。与一般人群相比,风湿病患者可能更害怕接种疫苗。洛杉矶县的风湿病患者主要是西班牙裔,代表了一个独特的、可能特别脆弱的群体,需要进一步探索接种COVID-19疫苗的障碍。目的探讨风湿病患者接受新冠肺炎疫苗的意愿。方法:我们进行了一项横断面调查,以评估2021年7月至2021年9月期间洛杉矶县风湿病诊所对COVID-19疫苗接受的看法和障碍,并收到116名患者的反馈。结果:大多数受访者为女性(83.9%),41-60岁(59.8%),西班牙裔(89.2%),高中及以下文化程度(68.7%),类风湿关节炎(56.9%)。我们发现大多数(88.4%)患者至少接种了一剂COVID-19疫苗。我们发现疫苗接受度在年龄、教育、种族和民族方面没有差异。大多数受访者认为,他们的健康状况使他们面临COVID-19并发症的高风险。此外,个人报告说,他们重视风湿病医生在接种疫苗之前与他们讨论疫苗的风险和益处。结论:我们发现,大多数患者已经接种或愿意接种疫苗,接种水平高于美国普通人群,风湿病学家发起的对话可以对患者与COVID-19相关的健康行为产生积极影响。
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引用次数: 0
Patient and public involvement in rheumatic and musculoskeletal research: an idea whose time has firmly come. 病人和公众参与风湿病和肌肉骨骼研究:一个时机已经成熟的想法。
IF 2.2 Q3 RHEUMATOLOGY Pub Date : 2023-05-31 DOI: 10.1186/s41927-023-00340-z
Angie Botto-van Bemden, Adewale O Adebajo, Ciarán Martin Fitzpatrick

Patient and public involvement is an idea whose time has firmly come. It is the views of these Guest Editors that it is the right thing to do morally and improves research quality and applicability.

耐心和公众参与是一个时机已经成熟的想法。这些特邀编辑认为,从道德上讲,这样做是正确的,可以提高研究的质量和适用性。
{"title":"Patient and public involvement in rheumatic and musculoskeletal research: an idea whose time has firmly come.","authors":"Angie Botto-van Bemden,&nbsp;Adewale O Adebajo,&nbsp;Ciarán Martin Fitzpatrick","doi":"10.1186/s41927-023-00340-z","DOIUrl":"https://doi.org/10.1186/s41927-023-00340-z","url":null,"abstract":"<p><p>Patient and public involvement is an idea whose time has firmly come. It is the views of these Guest Editors that it is the right thing to do morally and improves research quality and applicability.</p>","PeriodicalId":9150,"journal":{"name":"BMC Rheumatology","volume":"7 1","pages":"12"},"PeriodicalIF":2.2,"publicationDate":"2023-05-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10229221/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9553319","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
Predictors of severe lupus flare: a prospective follow-up study. 严重狼疮发作的预测因素:一项前瞻性随访研究。
IF 2.2 Q3 RHEUMATOLOGY Pub Date : 2023-05-24 DOI: 10.1186/s41927-023-00333-y
Alimohammad Fatemi, Elaheh Keivani-Boroujeni, Abbas Smiley

Background: Flare-up of systemic lupus erythematosus (SLE) is a common characteristic that could have deleterious effects on patients' outcome and survival. The aim of this study was to identify the predictors of severe lupus flare.

Methods: 120 patients with SLE were enrolled and followed-up for 23 months. Demographic, clinical manifestations, laboratory parameters and disease activity were recorded at each visit. In addition, presence of severe lupus flare at each visit was evaluated by using the Safety of Estrogens in Lupus Erythematosus National Assessment (SELENA)-SLE disease activity index (SLEDAI) flare composite index. Predictors of severe lupus flare were obtained by backward logistic regression analyses. Predictors of SLEDAI were obtained by backward linear regression analyses.

Results: During the follow-up period, 47 patients had at least one episode of severe lupus flare. Mean (SD) age of patients with severe flare versus no flare was 31.7 (7.89) and 38.3 (8.24) years, respectively (P = 0.001). Ten (62.5%) out of 16 males and 37 (35.5%) out of 104 females had severe flare (P = 0.04). History of lupus nephritis (LN) was recorded in 76.5% and 44% of patients with severe flare and no severe flare, respectively (P = 0.001). Thirty-five (29.2%) patients with high anti-double-stranded DNA (anti-ds-DNA antibody) and 12 (10%) with negative anti-ds-DNA antibody had severe lupus flare (P = 0.02). By multivariable logistic regression analysis, younger age (OR = 0.87, 95% CI 0.80-0.94, P = 0.0001), history of LN (OR = 4.66, 95% CI 1.55-14.002, P = 0.006) and high SLEDAI at the first visit (OR = 1.19, 95% CI 1.026-1.38) were the main predictors of flare. When severe lupus flare after the first visit was used as the outcome variable, similar findings were observed but, SLEDAI, although left among the final predictors in the model, was not significant. SLEDAIs in future visits were mainly predicted by Anti-ds-DNA antibody, 24-h urine protein and arthritis at the first visit.

Conclusion: SLE patients with younger age, history of previous LN or high baseline SLEDAI, may need closer monitoring and follow up.

背景:系统性红斑狼疮(SLE)的突然发作是一种常见的特征,可能对患者的预后和生存产生有害影响。本研究的目的是确定严重狼疮发作的预测因素。方法:选取120例SLE患者,随访23个月。每次访问时记录人口统计学、临床表现、实验室参数和疾病活动。此外,通过使用雌激素在红斑狼疮国家评估(SELENA)-SLE疾病活动性指数(SLEDAI)耀斑复合指数来评估每次就诊时是否存在严重狼疮耀斑。通过逆向logistic回归分析获得严重狼疮发作的预测因子。通过反向线性回归分析获得SLEDAI的预测因子。结果:在随访期间,47例患者至少有一次严重狼疮发作。严重耀斑患者与无耀斑患者的平均(SD)年龄分别为31.7(7.89)岁和38.3(8.24)岁(P = 0.001)。16例男性患者中有10例(62.5%),104例女性患者中有37例(35.5%)发生严重耀斑(P = 0.04)。重度发作和无重度发作患者中分别有76.5%和44%有狼疮性肾炎(LN)病史(P = 0.001)。高抗双链DNA(抗ds-DNA抗体)患者35例(29.2%),抗ds-DNA抗体阴性患者12例(10%)发生严重狼疮发作(P = 0.02)。多变量logistic回归分析显示,年龄较轻(OR = 0.87, 95% CI 0.80-0.94, P = 0.0001)、LN病史(OR = 4.66, 95% CI 1.55-14.002, P = 0.006)和首次就诊时SLEDAI较高(OR = 1.19, 95% CI 1.026-1.38)是光照度发生的主要预测因素。当首次就诊后严重狼疮发作用作结果变量时,观察到类似的结果,但是SLEDAI虽然留在模型的最终预测因子中,但并不显著。未来访视SLEDAIs主要通过抗ds- dna抗体、24小时尿蛋白和首次访视时的关节炎来预测。结论:年龄较小、既往有LN病史或SLEDAI基线较高的SLE患者可能需要更密切的监测和随访。
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引用次数: 0
Hands and feet radiologic involvements in systemic sclerosis. 系统性硬化症的手脚影像学表现。
IF 2.2 Q3 RHEUMATOLOGY Pub Date : 2023-05-20 DOI: 10.1186/s41927-023-00336-9
Fatemeh Badiee, Alireza Fatemi, Reza Zahedpasha, Mohammad Hadi Gharib, Mohammadhassan Jokar, Somayeh Livani, Mehrdad Aghaie, Nafiseh Abdolahi

Aim: Systemic sclerosis (SSc) is a rare autoimmune disorder characterized by vascular and fibrosing involvement of the skin and internal organs. In this study, we determined the prevalence and characteristics of radiological hands and feet involvements in Iranian SSc patients to identify the associations between clinical features and radiologic findings.

Methods: 43 SSc patients (41 women and 2 men), with a median age of 44.8 years (ranges 26-70 years) and a mean disease duration of 11.8 years (ranges 2-28 years) were studied in this cross-sectional study.

Results: 42 patients had radiological changes both in their hands and feet. Only one patient had alteration just in hand. The most frequent changes that we found in hand were Juxta-articular Osteoporosis (93%), Acro-osteolysis (58.2%), and Joint Space Narrowing (55.8%). The prevalence of joint space narrowing or acro-osteolysis was higher in subjects with active skin involvement [modified Rodnan skin score (mRSS) > 14] [16/21 vs. 4/16 for patients with inactive skin involvement (mRSS < 14); p = 0.002]. The most frequent changes that we found in the foot were Juxta-articular Osteoporosis (93%), Acro-osteolysis (46.5%), Joint Space Narrowing (58.1%), and subluxation (44.2%). The presence of anti-ccp antibody was detected in 4 (9.3%), while positive rheumatoid factor was found in 13 (30.2%) of SSc patients.

Conclusion: This study corroborates that arthropathy is common in SSc patients. The introduction of the specific radiological involvements of SSc needs to be confirmed by further studies, in order to define the appropriate prognosis and treatment of patients.

目的:系统性硬化症(SSc)是一种罕见的自身免疫性疾病,其特征是血管和纤维化累及皮肤和内脏器官。在这项研究中,我们确定了伊朗SSc患者放射学上手脚受累的患病率和特征,以确定临床特征与放射学表现之间的关系。方法:43例SSc患者(女41例,男2例),中位年龄44.8岁(26 ~ 70岁),平均病程11.8年(2 ~ 28年)。结果:42例患者手脚均有影像学改变。只有一个病人做了修改。我们发现手部最常见的变化是关节旁骨质疏松症(93%)、肢端骨溶解(58.2%)和关节间隙狭窄(55.8%)。活动性皮肤受累患者关节间隙狭窄或肢端骨溶解的发生率更高[改良罗德曼皮肤评分(mRSS) > 14][16/21对4/16]非活动性皮肤受累患者(mRSS)。结论:本研究证实SSc患者普遍存在关节病变。引入SSc的具体放射学影响需要进一步的研究来证实,以便确定患者的适当预后和治疗。
{"title":"Hands and feet radiologic involvements in systemic sclerosis.","authors":"Fatemeh Badiee,&nbsp;Alireza Fatemi,&nbsp;Reza Zahedpasha,&nbsp;Mohammad Hadi Gharib,&nbsp;Mohammadhassan Jokar,&nbsp;Somayeh Livani,&nbsp;Mehrdad Aghaie,&nbsp;Nafiseh Abdolahi","doi":"10.1186/s41927-023-00336-9","DOIUrl":"https://doi.org/10.1186/s41927-023-00336-9","url":null,"abstract":"<p><strong>Aim: </strong>Systemic sclerosis (SSc) is a rare autoimmune disorder characterized by vascular and fibrosing involvement of the skin and internal organs. In this study, we determined the prevalence and characteristics of radiological hands and feet involvements in Iranian SSc patients to identify the associations between clinical features and radiologic findings.</p><p><strong>Methods: </strong>43 SSc patients (41 women and 2 men), with a median age of 44.8 years (ranges 26-70 years) and a mean disease duration of 11.8 years (ranges 2-28 years) were studied in this cross-sectional study.</p><p><strong>Results: </strong>42 patients had radiological changes both in their hands and feet. Only one patient had alteration just in hand. The most frequent changes that we found in hand were Juxta-articular Osteoporosis (93%), Acro-osteolysis (58.2%), and Joint Space Narrowing (55.8%). The prevalence of joint space narrowing or acro-osteolysis was higher in subjects with active skin involvement [modified Rodnan skin score (mRSS) > 14] [16/21 vs. 4/16 for patients with inactive skin involvement (mRSS < 14); p = 0.002]. The most frequent changes that we found in the foot were Juxta-articular Osteoporosis (93%), Acro-osteolysis (46.5%), Joint Space Narrowing (58.1%), and subluxation (44.2%). The presence of anti-ccp antibody was detected in 4 (9.3%), while positive rheumatoid factor was found in 13 (30.2%) of SSc patients.</p><p><strong>Conclusion: </strong>This study corroborates that arthropathy is common in SSc patients. The introduction of the specific radiological involvements of SSc needs to be confirmed by further studies, in order to define the appropriate prognosis and treatment of patients.</p>","PeriodicalId":9150,"journal":{"name":"BMC Rheumatology","volume":"7 1","pages":"9"},"PeriodicalIF":2.2,"publicationDate":"2023-05-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10199501/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9551605","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
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