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Response to Chen and Liu regarding correspondence to “Development of a prediction model for progression of rheumatoid arthritis–associated interstitial lung disease using serologic and clinical factors: The prospective KORAIL cohort” 对Chen和Liu关于“基于血清学和临床因素的类风湿关节炎相关间质性肺病进展预测模型的建立:前瞻性KORAIL队列”的回应。
IF 4.4 2区 医学 Q1 RHEUMATOLOGY Pub Date : 2026-04-01 Epub Date: 2026-01-03 DOI: 10.1016/j.semarthrit.2026.152912
Sung Hae Chang , Misti L. Paudel , Eun Young Lee , Jeffrey A. Sparks
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引用次数: 0
Reply to letter to the editor 给编辑回信。
IF 4.4 2区 医学 Q1 RHEUMATOLOGY Pub Date : 2026-04-01 Epub Date: 2026-01-11 DOI: 10.1016/j.semarthrit.2026.152918
Yasser El Miedany
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引用次数: 0
Fronto-cerebellar features associate with cognitive dysfunction in childhood-onset systemic lupus erythematosus 儿童期全身性红斑狼疮的额小脑特征与认知功能障碍相关。
IF 4.4 2区 医学 Q1 RHEUMATOLOGY Pub Date : 2026-04-01 Epub Date: 2026-01-11 DOI: 10.1016/j.semarthrit.2026.152916
Hanne Van der Heijden , Gabrielle Alonzi , Amanda Cao , Raquel van Gool , Merve Koç Yekedüz , Lise Vrolix , Itamar Ronen , Vanessa Rameh , Kyle McBrearty , Aditi Deokar , Robert P. Sundel , Eyal Muscal , Joseph Gonzalez-Heydrich , Andrea Knight , Joyce C. Chang , Jaymin Upadhyay

Objective

Cognitive dysfunction (CD) is a prevalent symptom in childhood-onset systemic lupus erythematosus (cSLE). This study aimed to investigate the neurobehavioral basis of CD in cSLE.

Methods

Patients with cSLE (N=20) and age- and sex-matched healthy controls (HCs, N=20) completed questionnaires and multiple neurocognitive tests. The Systemic Lupus Erythematosus Disease Activity Index 2000 and laboratory markers were used to monitor patients’ clinical status. Neuroimaging assessments included functional near-infrared spectroscopy (fNIRS), functional magnetic resonance imaging (fMRI), and structural MRI.

Results

cSLE patients demonstrated moderate disease activity with high inflammation and immune dysregulation, alongside low medication adherence. Relative to HCs, cSLE patients showed worse cognitive functioning, higher emotional distress and more physical symptoms. fNIRS revealed higher prefrontal cortex activity in cSLE vs. HCs during the color-word Stroop task, suggesting impaired cognitive flexibility. fMRI performed during the N-back working memory task revealed altered frontal cortex and cerebellum activity, while modulations in resting-state fronto-cerebellar connectivity in the cSLE cohort were observed. Patients with cSLE were characterized by reduced gray matter morphological properties in frontal cortex and cerebellar subdivisions (e.g., crus II) alongside altered white matter structural connectivity among these cognitive processing hubs. K-means clustering analysis delineated three subgroups within the cSLE cohort based on neuroimaging profiles, where subgroups varied based on cognitive and emotional health.

Conclusion

This study provides evidence of fronto-cerebellar abnormalities and their associations with CD in cSLE. This investigation underscores the need for multidisciplinary research efforts to further elucidate the neurobiological underpinnings of CD in cSLE.
目的:认知功能障碍(CD)是儿童期系统性红斑狼疮(cSLE)的常见症状。本研究旨在探讨cSLE中CD的神经行为基础。方法:cSLE患者(N=20)和年龄、性别匹配的健康对照(hc, N=20)完成问卷调查和多项神经认知测试。采用系统性红斑狼疮疾病活动指数2000和实验室标志物监测患者的临床状况。神经影像学评估包括功能近红外光谱(fNIRS)、功能磁共振成像(fMRI)和结构MRI。结果:cSLE患者表现出中度疾病活动性,伴有高炎症和免疫失调,同时药物依从性低。与hc相比,cSLE患者表现出更差的认知功能、更高的情绪困扰和更多的身体症状。fNIRS显示,在颜色单词Stroop任务中,cSLE与HCs的前额叶皮层活动更高,表明认知灵活性受损。在N-back工作记忆任务期间进行的fMRI显示额叶皮层和小脑活动发生了变化,而在cSLE队列中,静息状态下额小脑连通性发生了调节。cSLE患者的特征是额叶皮层和小脑分支(如小腿II)的灰质形态特征减少,同时这些认知加工中枢之间的白质结构连通性改变。k -均值聚类分析根据神经影像学概况在cSLE队列中划分了三个亚组,其中亚组根据认知和情绪健康而变化。结论:本研究为cSLE患者的额小脑异常及其与CD的关系提供了证据。这项研究强调了多学科研究的必要性,以进一步阐明cSLE中CD的神经生物学基础。
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引用次数: 0
From Aortitis to Sweet’s: The Immune Spectrum of G-CSF Adverse Events 从大动脉炎到鼻窦炎:G-CSF不良事件的免疫谱。
IF 4.4 2区 医学 Q1 RHEUMATOLOGY Pub Date : 2026-04-01 Epub Date: 2026-02-02 DOI: 10.1016/j.semarthrit.2026.152939
Jozélio Freire de Carvalho , Cezar Augusto Muniz Caldas
<div><h3>Background</h3><div>Granulocyte colony-stimulating factor (G-CSF) and its PEGylated formulation (pegfilgrastim) are indispensable for preventing chemotherapy-induced neutropenia and for stem-cell mobilization. Beyond hematopoiesis, G-CSF modulates innate/adaptive immunity and may precipitate immune-mediated events in susceptible hosts. Clinical signals span large-vessel vasculitis/aortitis and non-vascular neutrophil-dominant phenotypes, yet timing, spectrum, and management remain variably characterized.</div></div><div><h3>Objective</h3><div>To identify and synthesize primary clinical reports of autoimmune/rheumatic disease triggered or exacerbated by exogenous G-CSF (filgrastim or pegfilgrastim), describing phenotypes, latency, management, outcomes, and implications for practice.</div></div><div><h3>Methods</h3><div>We performed a narrative review of primary clinical studies, including observational cohorts and case reports or short case series, identified through targeted searches of PubMed/MEDLINE, Scopus, and Embase, complemented by investigator-curated references. Eligible reports described autoimmune, inflammatory, or rheumatic manifestations occurring after exposure to exogenous G-CSF. Predefined phenotypes included large-vessel vasculitis/aortitis; neutrophilic dermatoses (e.g., Sweet’s syndrome, neutrophilic dermatosis of the dorsal hands); cutaneous small-vessel vasculitis; inflammatory or crystal arthritis (e.g., calcium pyrophosphate deposition disease); pulmonary inflammatory injury (e.g., diffuse alveolar hemorrhage); and clear flares of established autoimmune disease. Narrative or mechanistic reviews and trials of GM-CSF pathway blockade were excluded from qualitative synthesis.</div></div><div><h3>Results</h3><div>Twenty-four studies met inclusion criteria: two observational cohorts and twenty-two single-patient reports/short series. Cohorts quantified a low but reproducible burden of pegfilgrastim-associated aortitis, with stereotyped involvement of the aortic arch/proximal branches and typical latency of 7–15 days. Case-level data confirmed marked elevation of acute phase reactants, usually negative autoantibodies, favorable outcomes after G-CSF withdrawal with or without short glucocorticoid courses, and recurrence/migration on re-exposure. Non-vascular events clustered earlier (2–7 days) and included biopsy-proven Sweet’s/NDDH, leukocytoclastic vasculitis, CPPD flares, and rare diffuse alveolar hemorrhage; granulomatous dermatitis and perioperative pyoderma gangrenosum were also observed. <em>Re</em>-exposure information suggested phenotype-specific risk: recurrent CPPD with pegfilgrastim was mitigated by switching to short-acting filgrastim; selected limited aortitis resolved without steroids; refractory aortitis responded to IL-6 blockade, enabling uninterrupted chemotherapy.</div></div><div><h3>Conclusions</h3><div>Exogenous G-CSF can precipitate a coherent spectrum of immune-mediated toxicity with distinct, clini
背景:粒细胞集落刺激因子(G-CSF)及其聚乙二醇化制剂(pegfilgrastim)对于预防化疗诱导的中性粒细胞减少症和干细胞动员是必不可少的。除了造血功能,G-CSF调节先天/适应性免疫,并可能在易感宿主中沉淀免疫介导的事件。临床信号跨越大血管血管炎/主动脉炎和非血管中性粒细胞显性表型,但时间、频谱和管理仍然具有不同的特征。目的:识别和综合外源性G-CSF(非格昔汀或聚非格昔汀)引发或加重的自身免疫性/风湿性疾病的主要临床报告,描述表型、潜伏期、管理、结果和实践意义。方法:我们对主要临床研究进行了叙述性回顾,包括观察性队列和病例报告或短病例系列,通过PubMed/MEDLINE、Scopus和Embase的目标搜索确定,并辅以研究者策划的参考文献。符合条件的报告描述了暴露于外源性G-CSF后发生的自身免疫、炎症或风湿病表现。预定义的表型包括大血管血管炎/主动脉炎;中性粒细胞性皮肤病(如:Sweet’s综合征、手背中性粒细胞性皮肤病);皮肤小血管炎;炎性或结晶性关节炎(如焦磷酸钙沉积病);肺部炎性损伤(如弥漫性肺泡出血);还有明显的自身免疫性疾病症状定性综合排除了叙述性或机械性的综述和GM-CSF通路阻断的试验。结果:24项研究符合纳入标准:2个观察性队列和22个单患者报告/短系列。队列量化了pegfilgrastim相关性主炎的低但可重复的负担,具有主动脉弓/近端分支的刻板累及,典型潜伏期为7-15天。病例水平数据证实急性期反应物明显升高,通常为阴性自身抗体,G-CSF停药后有或没有短期糖皮质激素疗程的良好结果,以及再次暴露后复发/迁移。非血管事件聚集较早(2-7天),包括活检证实的Sweet /NDDH、白细胞破裂性血管炎、CPPD发作和罕见的弥漫性肺泡出血;同时还观察到肉芽肿性皮炎和坏疽性脓皮病。再暴露信息提示表型特异性风险:用聚非格昔汀治疗CPPD复发可通过改用短效非格昔汀减轻;选择性局限性主动脉炎不使用类固醇治疗;难治性大动脉炎对IL-6阻断有反应,使得不间断化疗成为可能。结论:外源性G-CSF可以沉淀免疫介导毒性的连贯谱,具有明确的临床可操作的时间窗:血管事件在聚非格昔汀后7-15天达到峰值,而皮肤、关节和肺部症状在2-7天内出现。识别取决于时间联系,成像/组织学,并排除模仿。管理是表型意识-药物停药±短糖皮质激素,CPPD的配方转换,以及选择性难治性主动脉的靶向IL-6阻断-允许保留血液学益处,同时最小化免疫毒性。前瞻性监测和结构化再暴露研究(包括配方调整)是从信号检测转向预防的重点。
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引用次数: 0
Risk factors for herpes zoster in patients with systemic lupus erythematosus 系统性红斑狼疮患者带状疱疹的危险因素
IF 4.4 2区 医学 Q1 RHEUMATOLOGY Pub Date : 2026-04-01 Epub Date: 2026-01-22 DOI: 10.1016/j.semarthrit.2026.152934
Arthur Mageau , Valdrin Shala , Clémence David , Tiphaine Goulenok , Thomas Papo , Pascale Nicaise-Roland , Karim Sacre

Introduction

International guidelines state that all immunocompromised adults should be vaccinated with the recombinant varicella zoster virus (VZV) vaccine. We aimed to assess the risk factors for herpes zoster (HZ) in patients with systemic lupus erythematosus (SLE).

Methods

Electronic medical records of all SLE patients registered at our referral centre were analysed at the time of clinical visit between July and December 2024. Demographic, medical history, laboratory and treatment data were extracted using a standardised data collection form. Herpes zoster incidence and associated factors were identified.

Results

A total of 224 SLE patients (female 93.8 %, median age 48 [38;56] years) were included. Of these, 30 (n = 30/224, 13.4 %) had HZ. The incidence was 0.81 for 100 patient-years. Univariable analysis showed that gamma globulin level (OR 0.81, 95 %CI 0.70–0.92), duration of steroid treatment (OR 1.07, 95 % CI 1.03–1.11), immunosuppressive (IS) drugs (OR 2.41, 95 % CI 1.06–6.05), duration of IS treatment (OR 1.05, 95 % CI 1.00–1.09) and biologics (OR 3.50, 95 % CI 1.53–7.93) were significantly associated with HZ. In a multivariable logistic regression analysis, only gamma globulin level (OR 0.86, 95 % CI 0.74–0.98) remained independently associated with HZ. The ROC curve for gamma globulin levels showed significant associations with HZ (AUC = 0.71 (0.59–0.82)), with a threshold of 10.3 g/L providing the best discrimination between SLE patients with and without HZ.

Conclusion

A reduced level gammaglobulin - with a cut-off of 10.3 g/L - is associated with HZ in SLE and may identify SLE patients who should be prioritized for VZV vaccination.
国际指南指出,所有免疫功能低下的成年人都应接种重组水痘带状疱疹病毒(VZV)疫苗。我们旨在评估系统性红斑狼疮(SLE)患者发生带状疱疹(HZ)的危险因素。方法对2024年7月至12月在我院转诊中心登记的所有SLE患者的电子病历进行分析。使用标准化数据收集表提取人口统计、病史、实验室和治疗数据。确定带状疱疹发病率及相关因素。结果共纳入224例SLE患者,女性占93.8%,中位年龄48[38;56]岁。其中30例(n = 30/224, 13.4%)患有HZ。100例患者年的发病率为0.81。单变量分析显示,γ球蛋白水平(OR 0.81, 95% CI 0.70-0.92)、类固醇治疗持续时间(OR 1.07, 95% CI 1.03-1.11)、免疫抑制(IS)药物(OR 2.41, 95% CI 1.06-6.05)、IS治疗持续时间(OR 1.05, 95% CI 1.00-1.09)和生物制剂(OR 3.50, 95% CI 1.53-7.93)与HZ显著相关。在多变量logistic回归分析中,只有丙种球蛋白水平(OR 0.86, 95% CI 0.74-0.98)仍然与HZ独立相关。γ -球蛋白水平的ROC曲线显示与HZ有显著相关性(AUC = 0.71 (0.59-0.82)), 10.3 g/L的阈值是区分合并和不合并HZ的SLE患者的最佳阈值。结论:降低的丙种球蛋白水平(临界值为10.3 g/L)与SLE患者的HZ相关,可以确定哪些SLE患者应该优先接种VZV疫苗。
{"title":"Risk factors for herpes zoster in patients with systemic lupus erythematosus","authors":"Arthur Mageau ,&nbsp;Valdrin Shala ,&nbsp;Clémence David ,&nbsp;Tiphaine Goulenok ,&nbsp;Thomas Papo ,&nbsp;Pascale Nicaise-Roland ,&nbsp;Karim Sacre","doi":"10.1016/j.semarthrit.2026.152934","DOIUrl":"10.1016/j.semarthrit.2026.152934","url":null,"abstract":"<div><h3>Introduction</h3><div>International guidelines state that all immunocompromised adults should be vaccinated with the recombinant varicella zoster virus (VZV) vaccine. We aimed to assess the risk factors for herpes zoster (HZ) in patients with systemic lupus erythematosus (SLE).</div></div><div><h3>Methods</h3><div>Electronic medical records of all SLE patients registered at our referral centre were analysed at the time of clinical visit between July and December 2024. Demographic, medical history, laboratory and treatment data were extracted using a standardised data collection form. Herpes zoster incidence and associated factors were identified.</div></div><div><h3>Results</h3><div>A total of 224 SLE patients (female 93.8 %, median age 48 [38;56] years) were included. Of these, 30 (<em>n</em> = 30/224, 13.4 %) had HZ. The incidence was 0.81 for 100 patient-years. Univariable analysis showed that gamma globulin level (OR 0.81, 95 %CI 0.70–0.92), duration of steroid treatment (OR 1.07, 95 % CI 1.03–1.11), immunosuppressive (IS) drugs (OR 2.41, 95 % CI 1.06–6.05), duration of IS treatment (OR 1.05, 95 % CI 1.00–1.09) and biologics (OR 3.50, 95 % CI 1.53–7.93) were significantly associated with HZ. In a multivariable logistic regression analysis, only gamma globulin level (OR 0.86, 95 % CI 0.74–0.98) remained independently associated with HZ. The ROC curve for gamma globulin levels showed significant associations with HZ (AUC = 0.71 (0.59–0.82)), with a threshold of 10.3 g/L providing the best discrimination between SLE patients with and without HZ.</div></div><div><h3>Conclusion</h3><div>A reduced level gammaglobulin - with a cut-off of 10.3 g/L - is associated with HZ in SLE and may identify SLE patients who should be prioritized for VZV vaccination.</div></div>","PeriodicalId":21715,"journal":{"name":"Seminars in arthritis and rheumatism","volume":"77 ","pages":"Article 152934"},"PeriodicalIF":4.4,"publicationDate":"2026-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146078669","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"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
Vaccine-associated lupus and related organ involvements: A pharmacovigilance analysis of VAERS database 1990–2025 疫苗相关狼疮和相关器官受损伤:VAERS数据库1990-2025的药物警戒分析
IF 4.4 2区 医学 Q1 RHEUMATOLOGY Pub Date : 2026-04-01 Epub Date: 2026-01-23 DOI: 10.1016/j.semarthrit.2026.152933
Ruiyan Xie , Lai Yee Cheong , Danting Zhang , Kevin C.H. Wu , Shirley C.W. Chan , Ivan Au , Kathy S.M. Leung , Joseph T.K. Wu , Tak Mao Chan , Desmond Y.H. Yap

Objectives

The relationships between vaccines and the development of systemic lupus erythematosus (SLE) and related organ involvements remain unclear.

Methods

We analyzed data from the Vaccine Adverse Event Reporting System (VAERS) during the period of 1990 to 2025 to determine the incidence of vaccine-associated SLE over time. The relationships between vaccines and the development of SLE and organ involvements were assessed by disproportionality and case-control analyses.

Results

1976 (0.017%) cases of vaccine-associated lupus were identified amongst 11,301,263 adverse events (AEs) reported in VAERS (1990-2025). Median time from vaccination to symptom onset was 8 (0-8,070) days. The most common manifestations are musculoskeletal and connective tissue disorders (82.94%) followed by skin and subcutaneous tissue lesions (10.83%), while kidney and nervous system involvements are rare. 702 (40.48%) patients had serious outcomes, and 22 (3.13%) died. Human papilloma virus (HPV) vaccine is associated with lupus AEs, especially musculoskeletal/connective tissue disorders, kidney and nervous system involvements. Hepatitis B virus (HBV) vaccine also shows correlations with lupus AEs, particularly musculoskeletal/connective tissue disorders. COVID-19, influenza and zoster vaccines show no relationship with lupus AEs and specific organ involvements. Recombinant protein vaccines show significant links with lupus AEs.

Conclusion

Vaccine-associated lupus is uncommon and manifestations are generally mild. HPV and HBV vaccines show strong associations with lupus AEs with distinct organ involvements.
目的:疫苗与系统性红斑狼疮(SLE)发展及相关脏器受累的关系尚不清楚。方法分析1990年至2025年期间疫苗不良事件报告系统(VAERS)的数据,以确定疫苗相关SLE随时间的发病率。疫苗与SLE发展和器官受累之间的关系通过歧化和病例对照分析进行评估。结果1990-2025年,在VAERS报告的11,301,263例不良事件(ae)中,鉴定出1976例(0.017%)疫苗相关狼疮。从接种疫苗到出现症状的中位时间为8(0- 8070)天。最常见的表现是肌肉骨骼和结缔组织病变(82.94%),其次是皮肤和皮下组织病变(10.83%),而肾脏和神经系统的累及是罕见的。702例(40.48%)患者预后严重,死亡22例(3.13%)。人乳头瘤病毒(HPV)疫苗与狼疮ae有关,特别是肌肉骨骼/结缔组织疾病、肾脏和神经系统受累。乙型肝炎病毒(HBV)疫苗也显示与狼疮ae,特别是肌肉骨骼/结缔组织疾病相关。COVID-19、流感和带状疱疹疫苗与狼疮ae和特定器官受累无关。重组蛋白疫苗与狼疮ae有显著联系。结论疫苗相关性狼疮少见,临床表现一般较轻。HPV和HBV疫苗与不同器官受累的狼疮ae有很强的相关性。
{"title":"Vaccine-associated lupus and related organ involvements: A pharmacovigilance analysis of VAERS database 1990–2025","authors":"Ruiyan Xie ,&nbsp;Lai Yee Cheong ,&nbsp;Danting Zhang ,&nbsp;Kevin C.H. Wu ,&nbsp;Shirley C.W. Chan ,&nbsp;Ivan Au ,&nbsp;Kathy S.M. Leung ,&nbsp;Joseph T.K. Wu ,&nbsp;Tak Mao Chan ,&nbsp;Desmond Y.H. Yap","doi":"10.1016/j.semarthrit.2026.152933","DOIUrl":"10.1016/j.semarthrit.2026.152933","url":null,"abstract":"<div><h3>Objectives</h3><div>The relationships between vaccines and the development of systemic lupus erythematosus (SLE) and related organ involvements remain unclear.</div></div><div><h3>Methods</h3><div>We analyzed data from the Vaccine Adverse Event Reporting System (VAERS) during the period of 1990 to 2025 to determine the incidence of vaccine-associated SLE over time. The relationships between vaccines and the development of SLE and organ involvements were assessed by disproportionality and case-control analyses.</div></div><div><h3>Results</h3><div>1976 (0.017%) cases of vaccine-associated lupus were identified amongst 11,301,263 adverse events (AEs) reported in VAERS (1990-2025). Median time from vaccination to symptom onset was 8 (0-8,070) days. The most common manifestations are musculoskeletal and connective tissue disorders (82.94%) followed by skin and subcutaneous tissue lesions (10.83%), while kidney and nervous system involvements are rare. 702 (40.48%) patients had serious outcomes, and 22 (3.13%) died. Human papilloma virus (HPV) vaccine is associated with lupus AEs, especially musculoskeletal/connective tissue disorders, kidney and nervous system involvements. Hepatitis B virus (HBV) vaccine also shows correlations with lupus AEs, particularly musculoskeletal/connective tissue disorders. COVID-19, influenza and zoster vaccines show no relationship with lupus AEs and specific organ involvements. Recombinant protein vaccines show significant links with lupus AEs.</div></div><div><h3>Conclusion</h3><div>Vaccine-associated lupus is uncommon and manifestations are generally mild. HPV and HBV vaccines show strong associations with lupus AEs with distinct organ involvements.</div></div>","PeriodicalId":21715,"journal":{"name":"Seminars in arthritis and rheumatism","volume":"77 ","pages":"Article 152933"},"PeriodicalIF":4.4,"publicationDate":"2026-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146078670","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"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
Response to letter to the editor regarding "Nailfold Capillaroscopy as a predictor of cardiovascular events and mortality in systemic sclerosis" 关于“甲襞毛细血管镜检查作为系统性硬化症心血管事件和死亡率的预测指标”致编辑的信的回复
IF 4.4 2区 医学 Q1 RHEUMATOLOGY Pub Date : 2026-04-01 Epub Date: 2026-01-01 DOI: 10.1016/j.semarthrit.2025.152909
Carlos Valera-Ribera, Juan José Alegre-Sancho, Joaquín Lacasa-Molina, Montserrat Robustillo-Villarino, Javier Narváez
{"title":"Response to letter to the editor regarding \"Nailfold Capillaroscopy as a predictor of cardiovascular events and mortality in systemic sclerosis\"","authors":"Carlos Valera-Ribera,&nbsp;Juan José Alegre-Sancho,&nbsp;Joaquín Lacasa-Molina,&nbsp;Montserrat Robustillo-Villarino,&nbsp;Javier Narváez","doi":"10.1016/j.semarthrit.2025.152909","DOIUrl":"10.1016/j.semarthrit.2025.152909","url":null,"abstract":"","PeriodicalId":21715,"journal":{"name":"Seminars in arthritis and rheumatism","volume":"77 ","pages":"Article 152909"},"PeriodicalIF":4.4,"publicationDate":"2026-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145928551","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"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
Almost half of the patients with axial spondyloarthritis reporting an acceptable symptom state have high disease activity; data from two standard-of-care cohorts 几乎一半报告可接受症状状态的轴型脊柱炎患者有高疾病活动性;数据来自两个标准治疗队列
IF 4.4 2区 医学 Q1 RHEUMATOLOGY Pub Date : 2026-04-01 Epub Date: 2025-12-29 DOI: 10.1016/j.semarthrit.2025.152903
Marlies Carbo , Anne Kampman , Davy Paap , Freke Wink , Casper Webers , Harald Vonkeman , Astrid van Tubergen , Suzanne Arends , Anneke Spoorenberg

Objectives

To assess the proportion of axial spondylarthritis (axSpA) patients reporting PASS, explore variables associated with PASS and determine the relation with axSpA Disease Activity Score (ASDAS) to predict PASS in two Dutch standard-of-care cohorts.

Methods

Patients from the GLAS cohort were included in this cross-sectional analysis. External validation was performed in the SpA-Net cohort. Univariable and multivariable logistic regression were performed to identify determinants of PASS. The predictive accuracy and threshold of the ASDAS for predicting PASS were determined using AUC and highest Youden’s index.

Results

Of 673 included GLAS patients, 63 % were male, mean age was 48 (±14) years, and mean ASDAS 2.3 (±0.9). In total, 77 % perceived their symptom state as acceptable. Of these patients, 44 % had an ASDAS ≥2.1. In multivariable regression, lower ASDAS, absence of tender entheses and older age were independently associated with reported PASS (R² = 0.39). The ASDAS showed good accuracy in predicting PASS, with AUC of 0.84 (95 % CI 0.80–0.85) and optimal cut-off value of 2.6 (sensitivity 78 %, specificity 77 %). Similar results were found in the 159 patients from SpA-Net.

Conclusion

In daily clinical practice, 3 out of 4 axSpA patients report an acceptable symptom state, although almost half of them have high disease activity. In line, the ASDAS threshold for predicting PASS is 2.6. Our results show that the PASS question provides additional information to disease activity, which can contribute to better shared decision making.
目的评估报告PASS的轴型脊柱炎(axSpA)患者的比例,探索与PASS相关的变量,并确定与axSpA疾病活动评分(ASDAS)的关系,以预测两个荷兰标准治疗队列中的PASS。方法来自GLAS队列的患者纳入横断面分析。在SpA-Net队列中进行外部验证。采用单变量和多变量逻辑回归来确定PASS的决定因素。采用AUC和最高约登指数确定ASDAS预测PASS的预测精度和阈值。结果673例GLAS患者中,63%为男性,平均年龄48(±14)岁,平均ASDAS 2.3(±0.9)岁。总的来说,77%的人认为他们的症状状态是可以接受的。在这些患者中,44%的患者ASDAS≥2.1。在多变量回归中,较低的ASDAS、没有压痛性鼻窦和年龄较大与报告的PASS独立相关(R²= 0.39)。ASDAS预测PASS具有良好的准确性,AUC为0.84 (95% CI 0.80-0.85),最佳临界值为2.6(敏感性78%,特异性77%)。在SpA-Net的159名患者中也发现了类似的结果。结论在日常临床实践中,4例axSpA患者中有3例症状状态可接受,但近一半患者有较高的疾病活动性。因此,预测PASS的ASDAS阈值是2.6。我们的研究结果表明,PASS问题为疾病活动提供了额外的信息,这有助于更好地共同决策。
{"title":"Almost half of the patients with axial spondyloarthritis reporting an acceptable symptom state have high disease activity; data from two standard-of-care cohorts","authors":"Marlies Carbo ,&nbsp;Anne Kampman ,&nbsp;Davy Paap ,&nbsp;Freke Wink ,&nbsp;Casper Webers ,&nbsp;Harald Vonkeman ,&nbsp;Astrid van Tubergen ,&nbsp;Suzanne Arends ,&nbsp;Anneke Spoorenberg","doi":"10.1016/j.semarthrit.2025.152903","DOIUrl":"10.1016/j.semarthrit.2025.152903","url":null,"abstract":"<div><h3>Objectives</h3><div>To assess the proportion of axial spondylarthritis (axSpA) patients reporting PASS, explore variables associated with PASS and determine the relation with axSpA Disease Activity Score (ASDAS) to predict PASS in two Dutch standard-of-care cohorts.</div></div><div><h3>Methods</h3><div>Patients from the GLAS cohort were included in this cross-sectional analysis. External validation was performed in the SpA-Net cohort. Univariable and multivariable logistic regression were performed to identify determinants of PASS. The predictive accuracy and threshold of the ASDAS for predicting PASS were determined using AUC and highest Youden’s index.</div></div><div><h3>Results</h3><div>Of 673 included GLAS patients, 63 % were male, mean age was 48 (±14) years, and mean ASDAS 2.3 (±0.9). In total, 77 % perceived their symptom state as acceptable. Of these patients, 44 % had an ASDAS ≥2.1. In multivariable regression, lower ASDAS, absence of tender entheses and older age were independently associated with reported PASS (R² = 0.39). The ASDAS showed good accuracy in predicting PASS, with AUC of 0.84 (95 % CI 0.80–0.85) and optimal cut-off value of 2.6 (sensitivity 78 %, specificity 77 %). Similar results were found in the 159 patients from SpA-Net.</div></div><div><h3>Conclusion</h3><div>In daily clinical practice, 3 out of 4 axSpA patients report an acceptable symptom state, although almost half of them have high disease activity. In line, the ASDAS threshold for predicting PASS is 2.6. Our results show that the PASS question provides additional information to disease activity, which can contribute to better shared decision making.</div></div>","PeriodicalId":21715,"journal":{"name":"Seminars in arthritis and rheumatism","volume":"77 ","pages":"Article 152903"},"PeriodicalIF":4.4,"publicationDate":"2026-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145886337","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"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
Characteristics and long-term outcomes of patients with rheumatoid arthritis and concurrent calcium pyrophosphate deposition disease 类风湿关节炎并发焦磷酸钙沉积病患者的特点和长期预后
IF 4.4 2区 医学 Q1 RHEUMATOLOGY Pub Date : 2026-04-01 Epub Date: 2025-12-29 DOI: 10.1016/j.semarthrit.2025.152902
Natalie Schanzer , Bryant R. England , Katherine D. Wysham , Thomas R. Riley IV , Brian Sauer , Grant W. Cannon , Ted R. Mikuls , Joshua F. Baker

Purpose

To compare the characteristics, treatment patterns, and long-term outcomes of patients with concurrent calcium pyrophosphate deposition disease (CPPD) and rheumatoid arthritis (RA) to patients with RA without CPPD.

Methods

We studied patients with RA from the Veteran’s Affairs RA (VARA) registry and identified patients with CPPD using administrative codes. We compared characteristics of patients with concurrent CPPD and RA to those with RA alone at enrollment. We used parsimonious multivariable logistic regression to study the probability of achieving a low disease activity as well as receiving prednisone and new biologic or targeted synthetic disease modifying antirheumatic drugs (b/tsDMARDs) over follow-up, adjusting for pre-hypothesized confounders and stratifying by autoantibody status.

Results

Among 2771 U.S. veterans with RA, we identified 64 (2.3 %) patients with CPPD at enrollment. Patients with CPPD were older [68.5 (9.0) v. 64.2 (11.0), p < 0.001], had higher rates of comorbidities, including osteoarthritis (93.8 % v. 80.1 %, p = 0.007), spine disease, and diabetes, and were less likely to have ACPA (63.5 % v. 77.8 %, p = 0.01). While those with CPPD were numerically less likely to achieve low disease activity over time, this was not statistically significant. Among seronegative patients, CPPD patients exhibited more prednisone use (OR 2.44 [95 % CI 1.15–5.22]) and more frequent initiation of b/tsDMARDs (OR 2.79 [95 % CI 1.46–5.32]) as well as higher rates of joint replacement and death during follow-up.

Conclusions

Seronegative RA patients with CPPD changed therapies more frequently, used more prednisone, and had worse long-term outcomes. These findings suggest some seronegative RA may represent CPPD and require alternative diagnostic and treatment approaches.
目的比较焦磷酸钙沉积病(CPPD)合并类风湿关节炎(RA)患者与无CPPD合并类风湿关节炎(RA)患者的特点、治疗模式和长期预后。方法研究退伍军人事务RA (VARA)登记的RA患者,并使用行政代码识别CPPD患者。在入组时,我们比较了伴有CPPD和RA的患者与仅伴有RA的患者的特征。我们使用简约的多变量逻辑回归来研究在随访期间实现低疾病活动性以及接受强的松和新的生物或靶向合成疾病修饰抗风湿药物(b/tsDMARDs)的概率,调整预先假设的混杂因素并根据自身抗体状态分层。结果在2771名患有RA的美国退伍军人中,我们在入组时确定了64名(2.3%)CPPD患者。CPPD患者年龄较大[68.5 (9.0)vs . 64.2 (11.0), p < 0.001],合并症发生率较高,包括骨关节炎(93.8% vs . 80.1%, p = 0.007)、脊柱疾病和糖尿病,ACPA发生率较低(63.5% vs . 77.8%, p = 0.01)。虽然CPPD患者在数字上不太可能随着时间的推移达到低疾病活动性,但这在统计学上并不显著。在血清阴性患者中,CPPD患者在随访期间表现出更多的强的松使用(OR 2.44 [95% CI 1.15-5.22])和更频繁的b/ tsdmard启动(OR 2.79 [95% CI 1.46-5.32])以及更高的关节置换率和死亡率。结论血清阴性RA合并CPPD患者更频繁地改变治疗方法,使用更多的强的松,长期预后更差。这些发现提示一些血清阴性的RA可能代表CPPD,需要其他的诊断和治疗方法。
{"title":"Characteristics and long-term outcomes of patients with rheumatoid arthritis and concurrent calcium pyrophosphate deposition disease","authors":"Natalie Schanzer ,&nbsp;Bryant R. England ,&nbsp;Katherine D. Wysham ,&nbsp;Thomas R. Riley IV ,&nbsp;Brian Sauer ,&nbsp;Grant W. Cannon ,&nbsp;Ted R. Mikuls ,&nbsp;Joshua F. Baker","doi":"10.1016/j.semarthrit.2025.152902","DOIUrl":"10.1016/j.semarthrit.2025.152902","url":null,"abstract":"<div><h3>Purpose</h3><div>To compare the characteristics, treatment patterns, and long-term outcomes of patients with concurrent calcium pyrophosphate deposition disease (CPPD) and rheumatoid arthritis (RA) to patients with RA without CPPD.</div></div><div><h3>Methods</h3><div>We studied patients with RA from the Veteran’s Affairs RA (VARA) registry and identified patients with CPPD using administrative codes. We compared characteristics of patients with concurrent CPPD and RA to those with RA alone at enrollment. We used parsimonious multivariable logistic regression to study the probability of achieving a low disease activity as well as receiving prednisone and new biologic or targeted synthetic disease modifying antirheumatic drugs (b/tsDMARDs) over follow-up, adjusting for pre-hypothesized confounders and stratifying by autoantibody status.</div></div><div><h3>Results</h3><div>Among 2771 U.S. veterans with RA, we identified 64 (2.3 %) patients with CPPD at enrollment. Patients with CPPD were older [68.5 (9.0) v. 64.2 (11.0), <em>p</em> &lt; 0.001], had higher rates of comorbidities, including osteoarthritis (93.8 % v. 80.1 %, <em>p</em> = 0.007), spine disease, and diabetes, and were less likely to have ACPA (63.5 % v. 77.8 %, <em>p</em> = 0.01). While those with CPPD were numerically less likely to achieve low disease activity over time, this was not statistically significant. Among seronegative patients, CPPD patients exhibited more prednisone use (OR 2.44 [95 % CI 1.15–5.22]) and more frequent initiation of b/tsDMARDs (OR 2.79 [95 % CI 1.46–5.32]) as well as higher rates of joint replacement and death during follow-up.</div></div><div><h3>Conclusions</h3><div>Seronegative RA patients with CPPD changed therapies more frequently, used more prednisone, and had worse long-term outcomes. These findings suggest some seronegative RA may represent CPPD and require alternative diagnostic and treatment approaches.</div></div>","PeriodicalId":21715,"journal":{"name":"Seminars in arthritis and rheumatism","volume":"77 ","pages":"Article 152902"},"PeriodicalIF":4.4,"publicationDate":"2026-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145886336","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"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
Gastric disease in systemic sclerosis: Spectrum, challenges, and insights from a systematic literature review 系统性硬化症中的胃疾病:光谱、挑战和来自系统文献综述的见解
IF 4.4 2区 医学 Q1 RHEUMATOLOGY Pub Date : 2026-04-01 Epub Date: 2026-02-09 DOI: 10.1016/j.semarthrit.2026.152944
Robert Mack Anderton , Austin Zhu , Ali Ayla , Montserrat Chavez , Amanda Mayer , Kelsey Koym , Alfredo Guillen-del-Castillo , Luis G Alcala-Gonzalez , Michael Hughes , Zsuzsanna H. McMahan

Objective

Gastric complications in systemic sclerosis (SSc) are common, clinically diverse, and often overlooked, despite their profound impact on patient quality of life. This review synthesizes current evidence on prevalence, clinical relevance, and diagnostic strategies, while identifying key gaps and proposing priorities to advance care and research.

Methods

A systematic review was conducted per PRISMA guidelines and registered with PROSPERO. MEDLINE, EMBASE, and Web of Science were systematically searched (September 2024). Included studies addressed gastric manifestations in SSc, such as dysmotility, gastric antral vascular ectasia (GAVE), mucosal abnormalities, and/or Helicobacter pylori, and reported on prevalence, clinical impact, or diagnostic approaches.

Results

Of 123 full-text articles, 37 met our specified inclusion criteria. Gastric dysmotility was frequent, with delayed emptying in 18–64 % and bradygastria in 12–70 %. GAVE prevalence ranged from 0 to 22.3 % and was negatively associated with anti-topoisomerase-I and variably associated with anti-RNA polymerase III antibodies. Mucosal abnormalities (e.g., gastritis, ulcers, cancer) were common but inconsistently defined. Helicobacter pylori prevalence varied widely (10–78 %), with lower detection via biopsy or breath testing than serology. Associations between objective findings and patient-reported outcomes (PROs) were inconsistent and underexplored; data on gastric-specific mortality were limited.

Conclusion

Gastric disease in SSc is underrecognized, yet clinically significant. Progress necessitates standardized diagnostics (scintigraphy, breath tests, electrogastrography), validated patient-reported outcomes (PROs), and longitudinal studies to better define disease burden, optimize screening, and guide targeted therapies that improve outcomes and quality of life in SSc.
目的:系统性硬化症(SSc)的胃并发症是常见的,临床多样的,但往往被忽视,尽管它们对患者的生活质量有深远的影响。本综述综合了有关患病率、临床相关性和诊断策略的现有证据,同时确定了关键差距并提出了推进护理和研究的优先事项。方法根据PRISMA指南进行系统评价,并在PROSPERO注册。系统检索MEDLINE、EMBASE和Web of Science(2024年9月)。纳入的研究涉及SSc的胃表现,如运动障碍、胃胃窦血管扩张(GAVE)、粘膜异常和/或幽门螺杆菌,并报告了患病率、临床影响或诊断方法。结果123篇全文文章中,37篇符合纳入标准。胃运动障碍频繁发生,排空延迟占18 - 64%,胃蠕动缓慢占12 - 70%。give的患病率从0到22.3%不等,与抗拓扑异构酶i呈负相关,与抗rna聚合酶III抗体呈可变相关。粘膜异常(如胃炎、溃疡、癌症)很常见,但定义不一致。幽门螺杆菌的患病率差异很大(10 - 78%),活检或呼吸检测的检出率低于血清学。客观结果与患者报告结果(PROs)之间的关联不一致且未得到充分探讨;关于胃特异性死亡率的数据有限。结论SSc的胃疾病未被充分认识,但具有临床意义。进展需要标准化的诊断(扫描、呼吸测试、胃电图)、经过验证的患者报告结果(PROs)和纵向研究,以更好地定义疾病负担,优化筛查,并指导靶向治疗,改善SSc的结果和生活质量。
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期刊
Seminars in arthritis and rheumatism
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