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Generalized Edema in Anti-NXP2 Antibody-Positive Dermatomyositis. 抗nxp2抗体阳性皮肌炎的全身性水肿。
IF 2.4 4区 医学 Q2 RHEUMATOLOGY Pub Date : 2025-06-01 Epub Date: 2025-01-31 DOI: 10.1097/RHU.0000000000002207
Yuki Koshida, Takashi Yamane
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引用次数: 0
Prevalence and Associated Factors of Sarcopenia in Thai Rheumatoid Arthritis Patients: A Cross-Sectional Study. 泰国类风湿性关节炎患者肌肉减少症的患病率及相关因素:一项横断面研究。
IF 2.4 4区 医学 Q2 RHEUMATOLOGY Pub Date : 2025-06-01 Epub Date: 2025-01-06 DOI: 10.1097/RHU.0000000000002194
Wanitcha Gumtorntip, Phichayut Phinyo, Nuntana Kasitanon, Worawit Louthrenoo

Background/objectives: Sarcopenia associates with poor health-related quality of life (HRQoL). This study aimed to determine prevalence and factors associated with sarcopenia in rheumatoid arthritis (RA) patients. The HRQoL between RA patients with and without sarcopenia also was compared.

Methods: This cross-sectional study recruited RA patients, aged ≥20 years, at Chiang Mai University Hospital between May and November 2023. Their skeletal muscle mass (bioelectrical impedance analysis), grip strength (hand dynamometer), and physical performance (gait speed test) were evaluated. Sarcopenia was defined according to the Asian Working Group for Sarcopenia. Patient characteristics, disease activity, physical disability (HAQ-DI), HRQoL (SF-36), nutrition (mini nutrition assessment), and medications were recorded.

Results: Of 299 patients (89.0% female; age 61.3 ± 11.6 years; disease duration 13.9 ± 8.6 years), 37.5% had sarcopenia (27.4% severe sarcopenia). In multivariable analysis, body mass index (odds ratio [95% confidence interval] 1.46/1 unit decrease [1.27, 1.68], p < 0.001), disease duration (1.89/10 years increase [1.27, 2.83], p = 0.002), deformity of hands/feet (3.80 [1.50, 9.61], p = 0.005), mini nutritional assessment score (1.21/1 score decrease [1.04, 1.40], p = 0.012), and high C-reactive protein (CRP) (1.94 [1.02, 3.69], p = 0.044) were independent factors associated with sarcopenia. There was no relationship between sarcopenia and disease activity (DAS-28-ESR or CRP) or medication use. RA patients with sarcopenia had higher HAQ score than those without, but potentially lower scores in physical function domain of SF-36 with adjusted mean difference of 0.2 (95% confidence interval 0.03, 0.32, p = 0.022) and -5.9 (-12.24, 0.38, p = 0.066), respectively.

Conclusions: About one third of the Thai RA patients in this center had sarcopenia. Low body mass index, long disease duration, joint deformity, malnutrition, and high CRP were independent factors associated with sarcopenia. Sarcopenia affects physical capabilities and the physical function domain of the HRQoL.

背景/目的:肌肉减少症与较差的健康相关生活质量(HRQoL)相关。本研究旨在确定类风湿关节炎(RA)患者肌肉减少症的患病率和相关因素。并比较了有无肌少症的RA患者的HRQoL。方法:这项横断面研究招募了2023年5月至11月在清迈大学医院就诊的年龄≥20岁的RA患者。评估他们的骨骼肌质量(生物电阻抗分析)、握力(手测力计)和身体表现(步速测试)。肌少症是根据亚洲肌少症工作组定义的。记录患者特征、疾病活动度、身体残疾(HAQ-DI)、HRQoL (SF-36)、营养(迷你营养评估)和用药情况。结果:299例患者中,女性占89.0%;年龄61.3±11.6岁;病程13.9±8.6年),37.5%出现肌肉减少症(重度肌肉减少症占27.4%)。在多变量分析中,体重指数(优势比[95%置信区间]1.46/1单位减少[1.27,1.68],p < 0.001)、病程(1.89/10年增加[1.27,2.83],p = 0.002)、手/脚畸形(3.80 [1.50,9.61],p = 0.005)、迷你营养评估评分(1.21/1评分减少[1.04,1.40],p = 0.012)、高c反应蛋白(CRP) (1.94 [1.02, 3.69], p = 0.044)是与肌肉减少症相关的独立因素。肌少症与疾病活动度(DAS-28-ESR或CRP)或药物使用没有关系。RA肌少症患者的HAQ评分高于无肌少症患者,但SF-36生理功能域评分可能较低,调整后平均差异分别为0.2(95%可信区间0.03,0.32,p = 0.022)和-5.9(95%可信区间-12.24,0.38,p = 0.066)。结论:该中心约三分之一的泰国类风湿性关节炎患者患有肌肉减少症。体重指数低、病程长、关节畸形、营养不良、高CRP是与肌少症相关的独立因素。肌肉减少症影响身体机能和HRQoL的物理功能域。
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引用次数: 0
Childhood Familial Mediterranean Fever in the United States: Spectrum of Clinical Phenotypes and MEFV Genotypes in a Cohort From Southeast Michigan. 美国儿童家族性地中海热:来自密歇根州东南部队列的临床表型和MEFV基因型谱
IF 2.4 4区 医学 Q2 RHEUMATOLOGY Pub Date : 2025-04-01 Epub Date: 2024-12-03 DOI: 10.1097/RHU.0000000000002183
Basil M Fathalla, Ronald Thomas

Objectives: The aim of this study was to report the spectrum of Familial Mediterranean Fever (FMF) in children living in Southeast Michigan.

Methods: We reviewed prerecorded data in medical records of FMF patients. Statistical analysis of the data included Fisher exact test, Pearson χ 2 procedure, parametric independent samples t test, and parametric analysis of variance using SPSS Version 29.0, IBM Inc.

Results: The study included 29 males and 21 females. The mean age at presentation was 4.63 ± 3.66 years, and the mean time to diagnosis was 2.1 ± 2.18 years. A slight majority presented in the first 3 years of age (54%). Family history of FMF was reported in only 58% of patients. Clinical manifestations included fever (84%), gastrointestinal (84%), musculoskeletal (64%; including chronic arthritis, sacroiliitis, and nonbacterial osteomyelitis), chest (28%), cutaneous (14%), and other manifestations (16%). Fever without other manifestations was reported only in patients presenting at ≤3 years of age ( p = 0.016), whereas older patients reported more gastrointestinal manifestations ( p = 0.04). Reported MEFV variants included p.M694V (n = 26), p.V726A (n = 23), p.M694I (n = 13), and others (n = 10). Homozygote and compound heterozygote patients had more gastrointestinal manifestations ( p < 0.001), whereas fever was more common in the heterozygote patients ( p = 0.04). The mean follow-up period was 5.34 ± 4.13 years with no renal disease.

Conclusions: We report the largest childhood FMF cohort in the United States. A negative family history should not preclude consideration of FMF as a cause of periodic fever. Recurrent fever can be the only manifestation, particularly in young patients with FMF. The absence of fever and chronic progressive musculoskeletal manifestations can uncommonly occur.

目的:本研究的目的是报告居住在密歇根州东南部的儿童家族性地中海热(FMF)的频谱。方法:我们回顾了FMF患者病历中预先记录的数据。数据的统计分析采用Fisher精确检验、Pearson χ2检验、参数独立样本t检验和参数方差分析,采用IBM公司的SPSS 29.0版本。结果:研究纳入男性29人,女性21人。平均发病年龄为4.63±3.66岁,平均诊断时间为2.1±2.18岁。大多数出现在前3岁(54%)。仅58%的患者报告有FMF家族史。临床表现包括发热(84%)、胃肠道(84%)、肌肉骨骼(64%);包括慢性关节炎、骶髂炎和非细菌性骨髓炎)、胸部(28%)、皮肤(14%)和其他表现(16%)。无其他表现的发热仅在≤3岁的患者中报告(p = 0.016),而老年患者报告更多的胃肠道表现(p = 0.04)。已报道的MEFV变异包括p.M694V (n = 26)、p.V726A (n = 23)、p.M694I (n = 13)和其他(n = 10)。纯合子和复合杂合子患者胃肠道表现较多(p < 0.001),而杂合子患者发热较多(p = 0.04)。平均随访时间5.34±4.13年,无肾脏疾病。结论:我们报告了美国最大的儿童FMF队列。阴性家族史不应排除FMF是周期性发热的原因。反复发热是唯一的表现,特别是在年轻的FMF患者中。没有发烧和慢性进行性肌肉骨骼表现可以罕见地发生。
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引用次数: 0
Crowned Dens Syndrome. 冠齿综合症。
IF 2.4 4区 医学 Q2 RHEUMATOLOGY Pub Date : 2025-04-01 Epub Date: 2025-01-31 DOI: 10.1097/RHU.0000000000002208
Yunqin Wu, Qinfeng Wu
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引用次数: 0
Age-Related Differences Between Juvenile and Adult Autoimmune Inflammatory Myopathies. 青少年和成年自身免疫性炎症性肌病之间与年龄有关的差异
IF 2.4 4区 医学 Q2 RHEUMATOLOGY Pub Date : 2025-04-01 Epub Date: 2024-12-12 DOI: 10.1097/RHU.0000000000002180
Melike Mehveş Kaplan, Zahide Ekici Tekin, Elif Çelikel, Vildan Güngörer, Cüneyt Karagöl, Nimet Öner, Merve Cansu Polat, Didem Öztürk, Emine Özçelik, Mehveş Işıklar Ekici, Pınar Akyüz Dağlı, Şükran Erten, Banu Çelikel Acar

Background: Clinical features and prognosis of autoimmune inflammatory myopathies (AIMs) can vary depending on the age of disease onset. The aim of this study was to compare the demographic characteristics, clinical features, laboratory findings, and long-term prognosis of juvenile and adult AIMs.

Methods: Patients diagnosed with AIM between 2009 and 2023 in the pediatric rheumatology and rheumatology departments of our hospital were included in this medical records review study. Demographic characteristics, clinical features, laboratory findings, treatments, and prognosis of juvenile and adult AIM patients were compared with statistical methods.

Results: Of the 94 patients diagnosed with AIM, 34 (36.2%) patients were juvenile and 60 (63.8%) patients were adult. At the time of diagnosis, while Gottron papules, dysphonia, and subcutaneous edema were more common in juvenile patients, fever was more common in adult patients ( p = 0.003, p = 0.05, p = 0.005 p = 0.05, respectively). During follow-up, while calcinosis was more common in juvenile patients, lung involvement and malignancy were more common in adult patients ( p = 0.022, p = 0.009, p = 0.006, respectively). The methylprednisolone pulse therapy requirement was significantly higher in juvenile patients ( p = 0.0001). Clinically inactive disease was more common in juvenile patients ( p = 0.01).

Conclusions: AIM with different onset ages is associated with distinct clinical manifestations and outcomes. The present study reported that in AIM patients, lung involvement and malignancy increase with age while clinically inactive disease decreases.

背景:自身免疫性炎症性肌病(AIMs)的临床特征和预后会因发病年龄而异。本研究旨在比较幼年和成年自身免疫性炎症性肌病的人口统计学特征、临床特征、实验室检查结果和长期预后:方法:将本院儿童风湿病科和风湿病科 2009 年至 2023 年期间确诊的 AIM 患者纳入病历回顾研究。用统计学方法比较了幼年和成年 AIM 患者的人口统计学特征、临床特征、实验室检查结果、治疗方法和预后:在 94 名确诊为 AIM 的患者中,34 名(36.2%)为青少年患者,60 名(63.8%)为成人患者。确诊时,幼年患者多见戈特龙丘疹、发音障碍和皮下水肿,而成年患者多见发热(分别为 p = 0.003、p = 0.05、p = 0.005、p = 0.05)。在随访期间,青少年患者更常见钙化,而成年患者更常见肺部受累和恶性肿瘤(分别为 p = 0.022、p = 0.009、p = 0.006)。青少年患者的甲基强的松龙脉冲疗法需求量明显更高(p = 0.0001)。临床上不活跃的疾病在青少年患者中更为常见(p = 0.01):结论:不同发病年龄的 AIM 具有不同的临床表现和预后。本研究报告显示,AIM 患者的肺部受累和恶性肿瘤随年龄增长而增加,而临床非活动性疾病则随年龄增长而减少。
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引用次数: 0
Telescoping Fingers in Arthritis Mutilans. 伸缩式手指在关节炎致残中的应用。
IF 2.4 4区 医学 Q2 RHEUMATOLOGY Pub Date : 2025-04-01 Epub Date: 2025-01-22 DOI: 10.1097/RHU.0000000000002189
Eaman Alhassan, Francisco P Quismorio
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引用次数: 0
Risk of Cancers According to the Use of Biological Agents in Patients With Radiographic Axial Spondyloarthritis: A Nationwide Population-Based Cohort Study. 放射治疗的中轴性脊柱炎患者使用生物制剂的癌症风险:一项全国性人群队列研究
IF 2.4 4区 医学 Q2 RHEUMATOLOGY Pub Date : 2025-04-01 Epub Date: 2025-01-27 DOI: 10.1097/RHU.0000000000002188
Oh Chan Kwon, Hye Sun Lee, Juyeon Yang, Min-Chan Park

Objective: As the duration of use of biological disease-modifying antirheumatic drugs (bDMARDs) in patients with radiographic axial spondyloarthritis (r-axSpA) accumulates over time, long-term real-world safety data on cancer risk are needed. This study assessed the association between tumor necrosis factor inhibitors (TNFis) and interleukin 17 inhibitors (IL-17is) exposures and cancer risk in patients with r-axSpA.

Methods: From the Korean nationwide database, we assembled 41,889 patients without prior history of cancer who were diagnosed with r-axSpA from 2010 onwards. Patients were followed up through 2021. Multivariable time-varying Cox models were performed to estimate the adjusted hazards ratios (aHRs) and 95% confidence intervals (CIs) of (1) overall cancers and (2) cancer subtypes according to TNFis exposure versus bDMARDs nonexposure, IL-17is exposure versus bDMARDs nonexposure, and IL-17is exposure versus TNFis exposure.

Results: The incident rates of overall cancers during bDMARDs nonexposure, TNFis exposure, and IL-17is exposure were 53.8, 37.6, and 67.3 per 10,000 person-years, respectively. TNFis exposure versus bDMARDs nonexposure was not associated with an increased risk of overall cancers (aHR = 0.9, 95% CI = 0.8-1.1). IL-17is exposure was not associated with an increased risk of overall cancers compared with bDMARDs nonexposure (aHR = 1.2, 95% CI = 0.5-3.0) or TNFis exposure (aHR = 1.3, 95% CI = 0.6-3.3). Similarly, no significant associations were observed between bDMARDs exposures and the risk of cancer subtypes.

Conclusions: In patients with r-axSpA, there was no evidence of increased cancer risk with TNFis and IL-17is exposures compared with bDMARDs nonexposure, suggesting that the use of bDMARDs is safe with respect to cancer risk in patients with r-axSpA.

目的:随着放射性轴性脊柱关节炎(r-axSpA)患者使用生物疾病修饰抗风湿药(bDMARDs)的时间不断延长,我们需要有关癌症风险的长期真实世界安全数据。本研究评估了肿瘤坏死因子抑制剂(TNFis)和白细胞介素17抑制剂(IL-17is)暴露与r-axSpA患者癌症风险之间的关系:我们从韩国全国数据库中收集了41889名2010年以后确诊为r-axSpA的无癌症病史患者。患者随访至 2021 年。我们采用多变量时变Cox模型估算了(1)总体癌症和(2)根据TNFis暴露与bDMARDs未暴露、IL-17is暴露与bDMARDs未暴露、IL-17is暴露与TNFis暴露的癌症亚型的调整危险比(aHRs)和95%置信区间(CIs):结果:在未暴露于bDMARDs、暴露于TNFis和暴露于IL-17is期间,总体癌症发病率分别为每万人年53.8例、37.6例和67.3例。暴露于 TNFis 与未暴露于 bDMARDs 相比,总体癌症风险的增加与 TNFis 暴露无关(aHR = 0.9,95% CI = 0.8-1.1)。与未接触bDMARDs(aHR = 1.2,95% CI = 0.5-3.0)或接触TNFis(aHR = 1.3,95% CI = 0.6-3.3)相比,接触IL-17is与总体癌症风险增加无关。同样,在bDMARDs暴露与癌症亚型风险之间也没有观察到明显的关联:在r-axSpA患者中,与未暴露于bDMARDs相比,没有证据表明暴露于TNFis和IL-17is会增加癌症风险,这表明在r-axSpA患者中使用bDMARDs对癌症风险是安全的。
{"title":"Risk of Cancers According to the Use of Biological Agents in Patients With Radiographic Axial Spondyloarthritis: A Nationwide Population-Based Cohort Study.","authors":"Oh Chan Kwon, Hye Sun Lee, Juyeon Yang, Min-Chan Park","doi":"10.1097/RHU.0000000000002188","DOIUrl":"10.1097/RHU.0000000000002188","url":null,"abstract":"<p><strong>Objective: </strong>As the duration of use of biological disease-modifying antirheumatic drugs (bDMARDs) in patients with radiographic axial spondyloarthritis (r-axSpA) accumulates over time, long-term real-world safety data on cancer risk are needed. This study assessed the association between tumor necrosis factor inhibitors (TNFis) and interleukin 17 inhibitors (IL-17is) exposures and cancer risk in patients with r-axSpA.</p><p><strong>Methods: </strong>From the Korean nationwide database, we assembled 41,889 patients without prior history of cancer who were diagnosed with r-axSpA from 2010 onwards. Patients were followed up through 2021. Multivariable time-varying Cox models were performed to estimate the adjusted hazards ratios (aHRs) and 95% confidence intervals (CIs) of (1) overall cancers and (2) cancer subtypes according to TNFis exposure versus bDMARDs nonexposure, IL-17is exposure versus bDMARDs nonexposure, and IL-17is exposure versus TNFis exposure.</p><p><strong>Results: </strong>The incident rates of overall cancers during bDMARDs nonexposure, TNFis exposure, and IL-17is exposure were 53.8, 37.6, and 67.3 per 10,000 person-years, respectively. TNFis exposure versus bDMARDs nonexposure was not associated with an increased risk of overall cancers (aHR = 0.9, 95% CI = 0.8-1.1). IL-17is exposure was not associated with an increased risk of overall cancers compared with bDMARDs nonexposure (aHR = 1.2, 95% CI = 0.5-3.0) or TNFis exposure (aHR = 1.3, 95% CI = 0.6-3.3). Similarly, no significant associations were observed between bDMARDs exposures and the risk of cancer subtypes.</p><p><strong>Conclusions: </strong>In patients with r-axSpA, there was no evidence of increased cancer risk with TNFis and IL-17is exposures compared with bDMARDs nonexposure, suggesting that the use of bDMARDs is safe with respect to cancer risk in patients with r-axSpA.</p>","PeriodicalId":14745,"journal":{"name":"JCR: Journal of Clinical Rheumatology","volume":" ","pages":"e13-e21"},"PeriodicalIF":2.4,"publicationDate":"2025-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143052651","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
ANCA-Negative Pauci-Immune Crescentic Glomerulonephritis and Buerger Disease. anca阴性Pauci-Immune新月型肾小球肾炎和伯格病。
IF 2.4 4区 医学 Q2 RHEUMATOLOGY Pub Date : 2025-04-01 Epub Date: 2025-01-31 DOI: 10.1097/RHU.0000000000002206
Yujia Wang, Yanfang Xu
{"title":"ANCA-Negative Pauci-Immune Crescentic Glomerulonephritis and Buerger Disease.","authors":"Yujia Wang, Yanfang Xu","doi":"10.1097/RHU.0000000000002206","DOIUrl":"10.1097/RHU.0000000000002206","url":null,"abstract":"","PeriodicalId":14745,"journal":{"name":"JCR: Journal of Clinical Rheumatology","volume":" ","pages":"e26"},"PeriodicalIF":2.4,"publicationDate":"2025-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143074421","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
A Unique Documented Case of Undifferentiated Connective Tissue Disease Presenting With Isolated Right Knee Monarthritis. 一例独特的未分化结缔组织疾病,表现为孤立的右膝单关节炎。
IF 2.4 4区 医学 Q2 RHEUMATOLOGY Pub Date : 2025-04-01 Epub Date: 2025-01-06 DOI: 10.1097/RHU.0000000000002195
Angelo Nigro
{"title":"A Unique Documented Case of Undifferentiated Connective Tissue Disease Presenting With Isolated Right Knee Monarthritis.","authors":"Angelo Nigro","doi":"10.1097/RHU.0000000000002195","DOIUrl":"10.1097/RHU.0000000000002195","url":null,"abstract":"","PeriodicalId":14745,"journal":{"name":"JCR: Journal of Clinical Rheumatology","volume":" ","pages":"e24"},"PeriodicalIF":2.4,"publicationDate":"2025-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142949214","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
DECT Imaging Unmasks MSU Crystals Despite Negative Aspiration. 尽管抽吸结果为阴性,但 DECT 成像仍能揭示 MSU 晶体。
IF 2.4 4区 医学 Q2 RHEUMATOLOGY Pub Date : 2025-04-01 Epub Date: 2024-12-16 DOI: 10.1097/RHU.0000000000002197
Fatima K Alduraibi
{"title":"DECT Imaging Unmasks MSU Crystals Despite Negative Aspiration.","authors":"Fatima K Alduraibi","doi":"10.1097/RHU.0000000000002197","DOIUrl":"10.1097/RHU.0000000000002197","url":null,"abstract":"","PeriodicalId":14745,"journal":{"name":"JCR: Journal of Clinical Rheumatology","volume":" ","pages":"e25"},"PeriodicalIF":2.4,"publicationDate":"2025-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142818020","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
期刊
JCR: Journal of Clinical Rheumatology
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