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Hepatic Manifestations in Systemic Juvenile Idiopathic Arthritis and Macrophage Activation Syndrome. 系统性幼年特发性关节炎和巨噬细胞激活综合征的肝脏表现。
Pub Date : 2025-12-15 DOI: 10.3899/jrheum.2025-0699
Esraa Eloseily,Ivanna Romankevych,Taskin Sabit,Lara Berklite,Sarangarajan Ranganathan,Paul Rosen,Michael Henrickson,Jennifer Huggins,Grant Schulert,Alexei Grom
OBJECTIVESystemic juvenile idiopathic arthritis (SJIA) is a chronic inflammatory disease characterized by systemic features and arthritis. Macrophage activation syndrome (MAS) is a severe complication of SJIA often involving the liver. MAS confined predominantly to the liver, causing severe hepatitis, has been increasingly recognized. When liver MAS is the primary manifestation, significant hepatic injury can occur, requiring differentiation from other forms of SJIA-related liver involvement, which may warrant distinct treatment approaches. This study examined liver pathology in SJIA-MAS patients and explored potential mechanisms.METHODSThis retrospective case series analyzed data from four SJIA-MAS patients who presented with liver dysfunction and underwent core liver biopsies at Cincinnati Children's Hospital Medical Center (2019-2024).RESULTSFour patients (age range 4 -15 years) had elevated transaminases, with one meeting MAS criteria and three diagnosed with subclinical MAS. Liver biopsies showed portal and sinusoidal inflammatory infiltrates of CD3+ CD8+ T cells and CD163+ macrophages, with extensive hepatocellular damage, including centrilobular parenchymal collapse, multifocal necrosis, and lymphocyte-mediated bile duct injury. One case revealed features of veno-occlusive disease (VOD), a novel finding. Elevated serum CXCL9 and rapid response to emapalumab (anti-IFNγ) in all patients suggested IFNγ-driven liver pathology.CONCLUSIONThis study underscores the critical roles of CD8+ T cells, macrophages, and IFNγ in SJIA-MAS hepatitis. Future research should explore whether serum biomarkers of IFNγ activity can differentiate SJIA-MAS from other liver pathologies, such as drug-induced liver injury (methotrexate or anakinra-induced) and hepatic steatosis, to guide tailored therapies.
目的全身性青少年特发性关节炎(SJIA)是一种以全身特征和关节炎为特征的慢性炎症性疾病。巨噬细胞活化综合征(MAS)是SJIA的严重并发症,常累及肝脏。MAS主要局限于肝脏,引起严重肝炎,已被越来越多地认识到。当肝脏MAS为主要表现时,可发生明显的肝损伤,需要与其他形式的sjia相关的肝脏受损伤区分开来,这可能需要不同的治疗方法。本研究检查了SJIA-MAS患者的肝脏病理,并探讨了可能的机制。方法:本回顾性病例系列分析了2019-2024年在辛辛那提儿童医院医学中心(Cincinnati Children Hospital Medical Center)接受核心肝活检的4例SJIA-MAS患者的数据。结果4例患者(年龄4 ~ 15岁)转氨酶升高,1例符合MAS标准,3例诊断为亚临床MAS。肝活检显示门静脉和窦静脉内CD3+ CD8+ T细胞和CD163+巨噬细胞炎症浸润,伴广泛肝细胞损伤,包括小叶中心实质塌陷、多灶性坏死和淋巴细胞介导的胆管损伤。1例显示静脉闭塞性疾病(VOD)的特征,这是一个新的发现。所有患者血清CXCL9升高和对emapalumab(抗ifn γ)的快速反应提示ifn γ驱动的肝脏病理。结论本研究强调CD8+ T细胞、巨噬细胞和IFNγ在SJIA-MAS肝炎中的关键作用。未来的研究应该探索IFNγ活性的血清生物标志物是否可以区分SJIA-MAS与其他肝脏病变,如药物性肝损伤(甲氨蝶呤或阿那金诱导)和肝脂肪变性,以指导定制治疗。
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引用次数: 0
The Impact of Everyday Discrimination on Systemic Lupus Erythematous Disease Activity and Mental Health Outcomes. 日常歧视对系统性红斑狼疮疾病活动和心理健康结果的影响。
Pub Date : 2025-12-15 DOI: 10.3899/jrheum.2025-0631
Kathryn R K Benson,Alissa Becerril,Joonsuk Park,Laura Plantinga,Jinoos Yazdany,Maria Dall'Era,Patricia P Katz
OBJECTIVESystemic Lupus Erythematosus (SLE) disproportionately affects racial and ethnic minority populations in the US. Though discrimination is linked to worse SLE outcomes, the mechanisms of this association remain underexplored. Among a multiracial, multiethnic cohort with SLE, we explored relationships between discrimination and patient-reported outcomes (PROs) and if mental health affects these relationships.METHODSWe analyzed data from the California Lupus Epidemiology Study, comprised of Asian (31%), Black (10%), Hispanic (24%), and White (34%) participants (N=245). Participants completed the Everyday Discrimination Scale and the following PROs: Systemic Lupus Activity Questionnaire (SLAQ), PROMIS Pain Interference and Fatigue, PHQ8 (depression), and GAD7 (anxiety). Multivariable linear regressions modeled associations between discrimination and PROs. Mediation analyses evaluated if depression or anxiety mediated associations between discrimination and SLAQ, pain, or fatigue.RESULTSEveryday discrimination scores were highest among Black and Asian participants. Among multivariable models, higher discrimination was associated with worse SLAQ (β=0.25, P < 0.001), PHQ8 (β=0.21, P < 0.001), GAD7 (β=0.27, P < 0.001), pain (β=0.24, P=0.01), and fatigue (β=0.3, P=0.007). By racial/ethnic subgroups, discrimination was significantly associated with higher SLAQ among Black and Asian participants. Discrimination was no longer significantly associated with SLAQ, pain, or fatigue after adjusting for PHQ8 or GAD7.CONCLUSIONIn this diverse SLE cohort, discrimination was associated with greater patient-reported disease activity, pain, fatigue, depression, and anxiety. Mediation analyses suggest that mental health mediates the relationship between discrimination and PROs. These results highlight the impact of discrimination as a psychosocial stressor on disease outcome variables.
在美国,系统性红斑狼疮(SLE)不成比例地影响着种族和少数民族人群。尽管歧视与更糟糕的SLE结果有关,但这种关联的机制仍未得到充分探讨。在一个多种族、多民族的SLE患者队列中,我们探讨了歧视与患者报告结果(PROs)之间的关系,以及心理健康是否影响这些关系。方法我们分析来自加州狼疮流行病学研究的数据,包括亚洲(31%)、黑人(10%)、西班牙裔(24%)和白人(34%)参与者(N=245)。参与者完成了日常歧视量表和以下PROs:系统性狼疮活动问卷(SLAQ), PROMIS疼痛干扰和疲劳,PHQ8(抑郁)和GAD7(焦虑)。多变量线性回归模拟了歧视与PROs之间的关系。中介分析评估了抑郁或焦虑是否介导了歧视与SLAQ、疼痛或疲劳之间的关联。结果黑人和亚裔参与者的日常歧视得分最高。在多变量模型中,高分辨力与较差的SLAQ (β=0.25, P < 0.001)、PHQ8 (β=0.21, P < 0.001)、GAD7 (β=0.27, P < 0.001)、疼痛(β=0.24, P=0.01)和疲劳(β=0.3, P=0.007)相关。在种族/民族亚组中,黑人和亚洲参与者的歧视与较高的SLAQ显著相关。在调整PHQ8或GAD7后,歧视不再与SLAQ、疼痛或疲劳显著相关。结论:在这个多样化的SLE队列中,歧视与更大的患者报告的疾病活动、疼痛、疲劳、抑郁和焦虑相关。中介分析表明,心理健康在歧视与职业倾向之间起中介作用。这些结果突出了歧视作为一种社会心理压力源对疾病结局变量的影响。
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引用次数: 0
Changes in Modern Care for Juvenile Idiopathic Arthritis: How Much Does This Affect Health-Related Quality of Life? 青少年特发性关节炎现代护理的变化:这对健康相关生活质量有多大影响?
Pub Date : 2025-12-15 DOI: 10.3899/jrheum.2025-1181
Ruby J Mckenna,Stephanie J W Shoop-Worrall
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引用次数: 0
Evaluating the Applicability of the EULAR/ACR-2019, SLICC-2012, and ACR-1997 Classification Criteria for Systemic Lupus Erythematosus in Children: a multicenter study. 评估EULAR/ACR-2019、SLICC-2012和ACR-1997对儿童系统性红斑狼疮分类标准的适用性:一项多中心研究
Pub Date : 2025-12-15 DOI: 10.3899/jrheum.2025-0559
Jingyi Qiao,Yanan Ma,Xinyue Zhang,Guohao Zhu,Yaoyao Shangguan,Yangqi Yin,Hong Chang,Jiakai Wang,Gang Luo,Sana Qureshi,Dahai Wang,Wenjie Zheng,Xiaoxue Ma
OBJECTIVESystemic lupus erythematosus (SLE) is a chronic autoimmune disease that affects approximately 15%-20% of patients during childhood at initial onset. For childhood-onset SLE (cSLE), clinical manifestations are not typical at early stages, thus cSLE classification criteria are lacking, which makes diagnoses difficult. To evaluate suitable classification criteria for these children, we conducted a multicenter cohort study to compare the practicability of EULAR/ACR-2019, SLICC-2012, and ACR-1997 Classification Criteria for SLE.METHODSPatients from three different regions participated, including children with cSLE (n=348), a children's control group (n=59), adults with SLE (n=80), and an adult control group (n=76). Sensitivity, specificity, and Area Under the Curve (AUC) values for EULAR/ACR-2019, SLICC-2012, and ACR-1997 Classification Criteria were calculated. Serial and parallel tests were conducted, and data when adjusting for EULAR/ACR-2019 classification criteria score thresholds, were compared.RESULTSIn our study, 348 cases with a firmly established clinical cSLE diagnosis (83.62% female) were collected. For children with SLE, ACR-1997 criteria showed the highest specificity (98.31%, 95% confidence interval (CI): 90.9%-100.0%), while SLICC-2012 criteria had the highest sensitivity (94.54%, 95% CI: 91.6%-96.7%). In comprehensive comparisons, EULAR/ACR-2019 criteria had the best AUC (0.944) and Youden Index (0.889) values. Parallel tests using SLICC-2012 or EULAR/ACR-2019 criteria for cSLE achieved the highest AUC (0.962) value and increased sensitivity to 99.14%. Finally, a EULAR/ACR-2019 score = 10 (cutoff for cSLE) generated the highest AUC value (0.953, 95% CI: 0.928-0.971).CONCLUSIONEULAR/ACR-2019 criteria were the most appropriate criteria for patients with cSLE. Moreover, in parallel tests using SLICC-2012 or EULAR/ACR-2019 criteria, diagnostic sensitivity was enhanced. Also, a total EULAR/ACR-2019 score ≥ 10 was appropriate for classifying cSLE. Our findings provide a basis for determining the most appropriate diagnostic strategy for children with SLE.
系统性红斑狼疮(SLE)是一种慢性自身免疫性疾病,约15%-20%的患者在儿童期发病。儿童期SLE (cSLE)早期临床表现不典型,缺乏分类标准,给诊断带来困难。为了评估适合这些儿童的分类标准,我们进行了一项多中心队列研究,比较EULAR/ACR-2019、SLICC-2012和ACR-1997 SLE分类标准的实用性。方法来自3个不同地区的患者,包括cSLE患儿(n=348)、儿童对照组(n=59)、成人SLE患者(n=80)和成人对照组(n=76)。计算EULAR/ACR-2019、SLICC-2012和ACR-1997分类标准的敏感性、特异性和曲线下面积(AUC)值。进行了串行和并行检验,并比较了调整EULAR/ACR-2019分类标准评分阈值后的数据。结果本组共收集临床诊断明确的cSLE病例348例,其中女性占83.62%。对于SLE患儿,ACR-1997标准的特异性最高(98.31%,95%可信区间(CI): 90.9% ~ 100.0%), SLICC-2012标准的敏感性最高(94.54%,95% CI: 91.6% ~ 96.7%)。综合比较,EULAR/ACR-2019标准的AUC(0.944)和约登指数(0.889)最佳。采用SLICC-2012或EULAR/ACR-2019标准对cSLE进行平行试验,获得了最高的AUC(0.962)值,灵敏度提高到99.14%。最后,EULAR/ACR-2019评分= 10 (cSLE截止值)产生最高的AUC值(0.953,95% CI: 0.928-0.971)。结论ular /ACR-2019标准是最适合cSLE患者的标准。此外,在采用SLICC-2012或EULAR/ACR-2019标准的平行试验中,诊断敏感性提高。此外,EULAR/ACR-2019总分≥10分适合用于cSLE的分类。我们的研究结果为确定儿童SLE最合适的诊断策略提供了基础。
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引用次数: 0
Hydroxychloroquine Ocular Toxicity - A Comprehensive Review. 羟基氯喹眼毒性综述。
Pub Date : 2025-12-15 DOI: 10.3899/jrheum.2025-0127
Marisa Sit,Ryan H Mason,Alexander J Kaplan
Hydroxychloroquine is a pharmacological agent in the management of various rheumatological disorders, including systemic lupus erythematosus and rheumatoid arthritis. Its therapeutic effects are attributed to its immunomodulatory and antimicrobial properties, and it is widely used due to its efficacy, relatively low cost, and generally favorable safety profile. Despite its well-established clinical utility, chronic use of hydroxychloroquine is associated with serious ocular side effects. This review covers hydroxychloroquine's history, pharmacokinetics and ocular safety profile, with a focus on the risk, diagnosis and screening for ocular toxicity. Retinopathy, the most significant adverse ocular side effect, may lead to irreversible vision loss if not promptly detected.
羟氯喹是一种治疗各种风湿性疾病的药物,包括系统性红斑狼疮和类风湿关节炎。其治疗效果归因于其免疫调节和抗菌特性,并且由于其疗效,相对较低的成本和普遍有利的安全性而被广泛使用。尽管羟基氯喹具有良好的临床疗效,但长期使用羟基氯喹会导致严重的眼部副作用。本文综述了羟氯喹的历史、药代动力学和眼部安全性,重点介绍了羟氯喹眼毒性的风险、诊断和筛查。视网膜病变是眼部最严重的不良副作用,如果不及时发现,可能导致不可逆的视力丧失。
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引用次数: 0
Rituximab Versus Mycophenolate Mofetil for Infection Risk in Systemic Sclerosis. 利妥昔单抗与霉酚酸酯对系统性硬化症感染风险的影响。
Pub Date : 2025-12-15 DOI: 10.3899/jrheum.2025-1042
Arjun Mahajan,Maureen Whittelsey,Nikki Zangenah,Maria Vazquez-Machado,Jeffrey S Smith,Jeffrey A Sparks,Avery H LaChance
{"title":"Rituximab Versus Mycophenolate Mofetil for Infection Risk in Systemic Sclerosis.","authors":"Arjun Mahajan,Maureen Whittelsey,Nikki Zangenah,Maria Vazquez-Machado,Jeffrey S Smith,Jeffrey A Sparks,Avery H LaChance","doi":"10.3899/jrheum.2025-1042","DOIUrl":"https://doi.org/10.3899/jrheum.2025-1042","url":null,"abstract":"","PeriodicalId":501812,"journal":{"name":"The Journal of Rheumatology","volume":"156 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2025-12-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145760028","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
Developing and Implementing an Entrustable Professional Activity for Musculoskeletal Ultrasound in an Adult Rheumatology Residency: A Pilot Study. 在成人风湿病住院医师中发展和实施可信赖的肌肉骨骼超声专业活动:一项试点研究。
Pub Date : 2025-12-15 DOI: 10.3899/jrheum.2025-0693
Samar Aboulenain,Sahil Koppikar,Heather McDonald-Blumer,Maria F Powell,Pooneh Akhavan,Alan Liang Zhou,Ahmed Omar,Shirley Lake
{"title":"Developing and Implementing an Entrustable Professional Activity for Musculoskeletal Ultrasound in an Adult Rheumatology Residency: A Pilot Study.","authors":"Samar Aboulenain,Sahil Koppikar,Heather McDonald-Blumer,Maria F Powell,Pooneh Akhavan,Alan Liang Zhou,Ahmed Omar,Shirley Lake","doi":"10.3899/jrheum.2025-0693","DOIUrl":"https://doi.org/10.3899/jrheum.2025-0693","url":null,"abstract":"","PeriodicalId":501812,"journal":{"name":"The Journal of Rheumatology","volume":"35 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2025-12-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145760027","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
Metabolomic Signatures of Dietary Patterns and Incident Radiographic Knee Osteoarthritis: A Case-Cohort Study from Osteoarthritis Initiative. 饮食模式的代谢组学特征和膝关节骨性关节炎的放射照相:来自骨关节炎倡议的病例队列研究。
Pub Date : 2025-12-15 DOI: 10.3899/jrheum.2025-0830
Shike Xu,Jeffrey B Driban,Priya S Prakash,Timothy E McAlindon,Charles B Eaton,Bing Lu
OBJECTIVEDietary factors related to inflammation, obesity, and metabolism may contribute to OA development. This study examines the relationship between metabolomic signatures of dietary patterns and radiographic knee OA incidence.METHODSThis case-cohort study included 603 participants from the Osteoarthritis Initiative, comprising 237 incident radiographic knee OA cases and 366 non-cases during a 6-year followup. Plasma metabolomes were analyzed using liquid chromatography-tandem mass spectrometry. We averaged metabolite levels at baseline and year 1 as the exposure and identified incident cases with those who had a Kellgren and Lawrence grade ≥2 in follow-ups. We selected 46 metabolites significantly associated with major food groups based on previous studies. Principal component analysis identified four metabolomic signatures related to specific food groups. Weighted logistic regression was used to examine associations between metabolomic signature scores and knee OA incidence.RESULTSAmong the 603 participants, after adjusting for age, sex and other covariates the highest quartiles of two metabolomic signatures associated with healthy food groups were linked to a reduced OA risk compared to the lowest quartile [Odds ratio (95% CI): 0.69 (0.44, 1.08) and 0.61 (0.40, 0.92)]. However, after further adjustment for BMI, these associations weakened. The proportions of the associations mediated through BMI for these two signatures were 43.7% and 81.4%, respectively. The other signatures were null.CONCLUSIONMetabolomic signatures of healthy dietary patterns may be linked to a lower risk of radiographic knee OA, with BMI potentially playing an intermediary role. Further studies are needed to explore causality.
与炎症、肥胖和代谢相关的饮食因素可能有助于OA的发展。本研究探讨了饮食模式的代谢组学特征与膝关节炎发病率之间的关系。方法:本病例队列研究包括来自骨关节炎倡议的603名参与者,其中包括237例放射学膝关节OA病例和366例非病例,随访6年。采用液相色谱-串联质谱法分析血浆代谢组。我们在基线和第1年平均代谢物水平作为暴露,并在随访中确定Kellgren和Lawrence分级≥2的事件病例。根据以往的研究,我们选择了46种与主要食物组显著相关的代谢物。主成分分析确定了与特定食物组相关的四个代谢组学特征。使用加权逻辑回归来检验代谢组学特征评分与膝关节OA发病率之间的关系。结果在603名参与者中,在调整了年龄、性别和其他协变量后,与健康食品组相关的两个代谢组特征的最高四分位数与OA风险降低相关,而与最低四分位数相比[比值比(95% CI): 0.69(0.44, 1.08)和0.61(0.40,0.92)]。然而,在进一步调整BMI后,这些关联减弱了。通过BMI介导的这两个特征的关联比例分别为43.7%和81.4%。其他签名都是空的。结论健康饮食模式的代谢组学特征可能与较低的膝关节骨性关节炎风险有关,BMI可能在其中起中介作用。需要进一步的研究来探索因果关系。
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引用次数: 0
Interdependent Cardiometabolic and Psoriatic Disease: An Intriguing Process That Calls for Far More Integrative Intervention. 相互依存的心脏代谢和银屑病:一个有趣的过程,需要更多的综合干预。
Pub Date : 2025-12-15 DOI: 10.3899/jrheum.2025-1216
Patrick H Dessein,Mpoti Seboka,Ahmed Solomon
{"title":"Interdependent Cardiometabolic and Psoriatic Disease: An Intriguing Process That Calls for Far More Integrative Intervention.","authors":"Patrick H Dessein,Mpoti Seboka,Ahmed Solomon","doi":"10.3899/jrheum.2025-1216","DOIUrl":"https://doi.org/10.3899/jrheum.2025-1216","url":null,"abstract":"","PeriodicalId":501812,"journal":{"name":"The Journal of Rheumatology","volume":"23 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2025-12-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145760033","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
Calcium Pyrophosphate Deposition Disease-Induced Acute Dactylitis in the Elderly: A Clinical Ultrasound Mimic. 焦磷酸钙沉积病引起的老年人急性趾炎:临床超声模拟。
Pub Date : 2025-11-15 DOI: 10.3899/jrheum.2025-0929
Nicolò Girolimetto,Niccolò Possemato,Raffaele Scarpa,Francesco Caso
{"title":"Calcium Pyrophosphate Deposition Disease-Induced Acute Dactylitis in the Elderly: A Clinical Ultrasound Mimic.","authors":"Nicolò Girolimetto,Niccolò Possemato,Raffaele Scarpa,Francesco Caso","doi":"10.3899/jrheum.2025-0929","DOIUrl":"https://doi.org/10.3899/jrheum.2025-0929","url":null,"abstract":"","PeriodicalId":501812,"journal":{"name":"The Journal of Rheumatology","volume":"61 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2025-11-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145525151","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
期刊
The Journal of Rheumatology
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