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Autoantibodies to dsDNA in the diagnosis, classification and follow-up of patients with systemic lupus erythematosus dsDNA自身抗体在系统性红斑狼疮患者诊断、分型及随访中的应用
IF 3.9 Q2 IMMUNOLOGY Pub Date : 2023-01-01 DOI: 10.1016/j.jtauto.2023.100191
Jan Damoiseaux, Joyce van Beers

Autoantibodies, in particular anti-dsDNA antibodies, are increasingly used for diagnosis, classification and follow-up of patients with SLE. Since standardization of autoantibody assays is a major challenge, more attention should be paid to harmonization initiatives and better definition of required test characteristics in classification criteria. For diagnosis and follow-up separate multi-center studies are required to establish test characteristics of distinct immuno-assays for both purposes. Finally, such studies should consider not to evaluate SLE as a single disease, but as a disease with distinct subtypes.

自身抗体,特别是抗dsDNA抗体,越来越多地用于SLE患者的诊断、分类和随访。由于自身抗体检测的标准化是一个重大挑战,因此应更多地关注统一举措和在分类标准中更好地定义所需的检测特征。为了诊断和随访,需要进行单独的多中心研究,以确定用于这两个目的的不同免疫测定的测试特征。最后,此类研究不应考虑将SLE评估为单一疾病,而应考虑将其评估为具有不同亚型的疾病。
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引用次数: 2
Preventive plasmapheresis for rituximab related flare in cryoglobulinemic vasculitis 预防性血浆置换治疗利妥昔单抗相关的冷球蛋白性血管炎
IF 3.9 Q2 IMMUNOLOGY Pub Date : 2023-01-01 DOI: 10.1016/j.jtauto.2023.100194
Léa Fornero , Tarik Kanouni , Jean-Jacques Tudesq , Camille Pochard , Pauline Verot , Wendy Renier , Ludovic Gabellier , Guillaume Cartron , Philippe Guilpain , Charles Herbaux

Introduction

Rituximab monotherapy represents the main therapeutic option for cryoglobulinemic vasculitis (CV) with severe organ involvement. However, initial worsening of the CV, known as rituximab-associated CV flare (=CV flare), has been described and are associated with high mortality rates. The aim of the present study is to evaluate the outcomes of plasmapheresis initiated before or during rituximab treatment, as prevention of CV flare.

Methods

We conducted a retrospecttive study in our tertiary referral center from 2001 to 2020. We have included all patients with CV receiving rituximab and divided them in two groups whether they had flare prevention by plasmapheresis or not. We evaluated rituximab-related CV flare incidence in both groups. CV flare was defined as the onset of a new organ involvement or worsening of the initial manifestations within 4 weeks following rituximab.

Results

Among the 71 patients included, 44 received rituximab without plasmapheresis (control = CT cohort) and 27 received plasmapheresis before or during rituximab treatment (preventive plasmapheresis = PP cohort). PP was given to patients thought to have a high risk of CV flare, with significantly more severe diseases than patients in the CT cohort. Despite this, no CV flare was observed in the PP group. In the other hand, 5 flares occurred in the CT cohort.

Conclusion

Our results show that plasmapheresis is efficient and well tolerated to prevent rituximab-associated CV flare. We believe that our data support the use of plasmapheresis in this indication, especially in patients with high risk of CV flare.

利妥昔单抗是严重器官受累的冷球蛋白性血管炎(CV)的主要治疗选择。然而,CV的初始恶化,被称为利妥昔单抗相关的CV耀斑(=CV耀斑),已被描述并与高死亡率相关。本研究的目的是评估血浆置换在利妥昔单抗治疗前或治疗期间预防心血管耀斑的效果。方法对2001年至2020年我院三级转诊中心进行回顾性研究。我们纳入了所有接受利妥昔单抗治疗的CV患者,并将他们分为两组,无论他们是否通过血浆置换预防耀斑。我们评估了两组患者与利妥昔单抗相关的CV耀斑发生率。CV耀斑定义为利妥昔单抗治疗后4周内出现新的器官受累或初始表现恶化。结果纳入的71例患者中,44例接受了利妥昔单抗治疗但未进行血浆置换(对照组= CT队列),27例在利妥昔单抗治疗前或治疗中进行了血浆置换(预防性血浆置换= PP队列)。PP被给予被认为有CV耀斑高风险的患者,其疾病明显比CT组患者严重。尽管如此,PP组未观察到CV耀斑。另一方面,CT组中发生了5次耀斑。结论血浆置换对预防利妥昔单抗相关的CV耀斑有效且耐受性良好。我们认为,我们的数据支持血浆置换在这一适应症中的应用,特别是在心血管耀斑高风险的患者中。
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引用次数: 1
Belimumab treatment in autoimmune hepatitis and primary biliary cholangitis – a case series 贝利单抗治疗自身免疫性肝炎和原发性胆管炎-一个病例系列
IF 3.9 Q2 IMMUNOLOGY Pub Date : 2023-01-01 DOI: 10.1016/j.jtauto.2023.100189
Mirjam Kolev , Adela-Cristina Sarbu , Burkhard Möller , Britta Maurer , Florian Kollert , Nasser Semmo

Background

The majority of patients with autoimmune hepatitis (AIH) achieve complete remission with established treatment regiments. In patients with intolerance or insufficient response to these drugs, the remaining options are limited and novel treatment approaches necessary. In primary biliary cholangitis (PBC), ursodeoxycholic acid (UDCA) and fibrates have improved prognosis dramatically, but there remains a proportion of patients with refractory disease.

In patients with refractory AIH and/or PBC, we used a novel treatment strategy with the anti-B cell activating factor, belimumab. The first three patients had concomitant Sjögren's disease. The connecting element between all three diseases is B cell activation, including elevated levels of the B cell activating factor (BAFF). Furthermore, belimumab has been shown to be beneficial in Sjögren's disease.

Aims and methods

To retrospectively investigate treatment response in six patients with AIH or PBC with or without concomitant Sjögren's disease treated with the anti-BAFF therapy belimumab at the University Hospital in Bern, Switzerland.

Results

In all three patients with AIH, belimumab improved disease control and helped by-pass or reduce problematic side effects from corticosteroids and calcineurin inhibitors. In PBC patients (n = 3), there was no clear improvement of liver function tests, despite reduction or normalization of IgM. All patients with concomitant Sjögren's disease (n = 3) had an improvement of sicca symptoms and two out of three patients experienced an initially marked reduction in fatigue, which lessened over time.

Conclusions

Belimumab may be a promising treatment option for patients with AIH and further investigations are needed. In PBC however, response was not convincing. The effects on sicca symptoms and fatigue were encouraging.

背景:大多数自身免疫性肝炎(AIH)患者通过既定的治疗方案获得完全缓解。对于不耐受或对这些药物反应不足的患者,剩余的选择有限,需要新的治疗方法。在原发性胆道胆管炎(PBC)中,熊去氧胆酸(UDCA)和贝特类药物可显著改善预后,但仍有一部分患者难治性疾病。在难治性AIH和/或PBC患者中,我们使用了一种新的治疗策略,即抗b细胞活化因子贝利单抗。前三名患者同时患有Sjögren病。这三种疾病之间的联系因素是B细胞活化,包括B细胞活化因子(BAFF)水平升高。此外,belimumab已被证明对Sjögren的疾病有益。目的和方法回顾性研究在瑞士伯尔尼大学医院接受抗baff治疗的6例伴有或不伴有Sjögren病的AIH或PBC患者的治疗反应。结果在所有3例AIH患者中,贝利单抗改善了疾病控制,并有助于绕过或减少皮质类固醇和钙调磷酸酶抑制剂的问题副作用。在PBC患者(n = 3)中,尽管IgM降低或正常化,但肝功能检查没有明显改善。所有伴有Sjögren's疾病的患者(n = 3)都有sicca症状的改善,三分之二的患者最初的疲劳明显减轻,随着时间的推移逐渐减轻。结论belimumab可能是AIH患者的一种有希望的治疗选择,需要进一步的研究。然而,中国人民银行的回应并不令人信服。对干燥症状和疲劳的效果令人鼓舞。
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引用次数: 1
Risk factors of cardiovascular involvement in patients with Behcet's disease 白塞病患者心血管疾病的危险因素
IF 3.9 Q2 IMMUNOLOGY Pub Date : 2023-01-01 DOI: 10.1016/j.jtauto.2023.100195
Yuqian Wang, Sheng Li, Shunli Tang, Xiaoxuan Cai, Juan Bai, Qingmiao Sun, Jianjun Qiao, Hong Fang

Objectives

Behcet's disease (BD) is a multi-systemic inflammatory vasculitis which may be life-threatening if combined with cardiovascular problems. The aim of the study was to identify potential risk factors associated with cardiovascular involvement in BD.

Methods

We reviewed the medical databases of a single center. All BD patients identified as fulfilling the 1990 International Study Group criteria or the International Criteria for Behcet's Disease criteria. Cardiovascular involvement, clinical manifestations, laboratory features, and treatments were recorded. The relationship between parameters and cardiovascular involvement was analyzed.

Results

111 BD patients were included: 21 (18.9%) had documented cardiovascular involvement (CV BD group) and 99 (81.1%) had no cardiovascular involvement (non-CV BD group). Compared with non-CV BD, the proportion of males and smokers were significantly increased in CV BD (p = 0.024 and p < 0.001, respectively). Levels of activated partial thromboplastin time (APTT), cardiac troponin I and C-reactive protein were significantly higher (p = 0.001, p = 0.031, and p = 0.034, respectively) in the CV BD group. Cardiovascular involvement was associated with smoking state, the presence of papulopustular lesions, and higher APTT in multivariate analyzed (p = 0.029, p = 0.021, and p = 0.006, respectively). The ROC curve showed that APTT predicts the risk of cardiovascular involvement (p < 0.01) at a cut-off value of 33.15 s with a sensitivity of 57.1% and specificity of 82.2%.

Conclusion

Cardiovascular involvement in BD patients was associated with gender, smoking state, the presence of papulopustular lesions, and higher APTT. All patients newly diagnosed with BD should be systematically screened for cardiovascular involvement.

目的白塞氏病(BD)是一种多系统炎症性血管炎,合并心血管疾病可危及生命。本研究的目的是确定与心血管疾病相关的潜在危险因素。方法我们回顾了单一中心的医学数据库。所有双相障碍患者均符合1990年国际研究组标准或白塞病国际标准。记录心血管受累情况、临床表现、实验室特征和治疗情况。分析了参数与心血管受累的关系。结果纳入111例BD患者:21例(18.9%)有心血管受累(CV BD组),99例(81.1%)无心血管受累(非CV BD组)。与非CV型BD患者相比,CV型BD患者中男性和吸烟者的比例显著增加(p = 0.024和p <分别为0.001)。活性部分凝血活素时间(APTT)、心肌肌钙蛋白I和c反应蛋白水平在CV BD组显著升高(p = 0.001、p = 0.031和p = 0.034)。在多变量分析中,心血管疾病与吸烟状态、丘疹病变的存在和较高的APTT相关(p = 0.029, p = 0.021和p = 0.006)。ROC曲线显示APTT可预测心血管受累风险(p <0.01),临界值为33.15 s,敏感性为57.1%,特异性为82.2%。结论BD患者心血管疾病累及与性别、吸烟状况、丘疹病变及APTT升高有关。所有新诊断为双相障碍的患者都应系统地筛查心血管疾病。
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引用次数: 1
Adding low dose cyclophosphamide to rituximab for remission-induction may prolong relapse-free survival in patients with ANCA vasculitis: A retrospective study 在利妥昔单抗中加入低剂量环磷酰胺进行缓解诱导可能延长ANCA血管炎患者的无复发生存期:一项回顾性研究
IF 3.9 Q2 IMMUNOLOGY Pub Date : 2023-01-01 DOI: 10.1016/j.jtauto.2022.100178
Renée Ysermans , Matthias H. Busch , Joop P. Aendekerk , Jan G.M.C. Damoiseaux , Pieter van Paassen

Objective

Rituximab (RTX) and cyclophosphamide (CYC) are effective remission-induction therapies in anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV). However, combining these therapies may favor prognosis in patients with a major disease presentation. We conducted a retrospective study to compare patients treated with a combination of RTX and low dose CYC (RTX-CYC) or with RTX only, both followed by tailored maintenance with RTX, with regard to long-term outcomes.

Methods

Patients treated in the Maastricht University Medical Center between March 2007 and January 2019, were screened for eligibility. The primary outcome variable was major relapse rate after two and five years. Secondary outcome variables were clinical data and laboratory parameters.

Results

Of the 246 screened patients, 34 received RTX-CYC and 28 RTX only for remission-induction. All patients were followed for at least two years, with a median follow-up of 48 months (IQR 24–60). At baseline, renal involvement was more prevalent in the RTX-CYC patients (85% vs. 61%, P = 0.028). Major relapse rates within two years, but not after five years, were significantly lower in the RTX-CYC group (3% vs. 24%, P = 0.032). The rate of infections, hypogammaglobulinemia, end-stage renal disease, malignancies, and mortality did not differ after two and five years.

Conclusion

Adding low dose CYC to RTX is safe and may prevent major relapses in patients with severe AAV in the first two years after remission-induction. Randomized controlled trials that compare the efficacy and safety between RTX and a combination of RTX with CYC are needed.

目的:利妥昔单抗(RTX)和环磷酰胺(CYC)是抗中性粒细胞胞浆抗体(ANCA)相关血管炎(AAV)的有效缓解诱导疗法。然而,联合这些疗法可能有利于有重大疾病表现的患者的预后。我们进行了一项回顾性研究,比较RTX和低剂量CYC (RTX-CYC)联合治疗或仅RTX治疗的患者,两种治疗均采用RTX进行量身定制的维持,以观察长期结果。方法对2007年3月至2019年1月期间在马斯特里赫特大学医学中心接受治疗的患者进行资格筛选。主要结局变量为2年和5年后的主要复发率。次要结局变量为临床数据和实验室参数。结果在246例筛选的患者中,34例接受RTX- cyc治疗,28例仅用于缓解诱导。所有患者随访至少2年,中位随访48个月(IQR 24-60)。在基线时,肾受累在RTX-CYC患者中更为普遍(85%比61%,P = 0.028)。RTX-CYC组两年内的主要复发率显著低于RTX-CYC组(3% vs. 24%, P = 0.032)。感染率、低丙种球蛋白血症、终末期肾病、恶性肿瘤和死亡率在2年和5年后没有差异。结论在RTX中加入低剂量CYC是安全的,可以预防严重AAV患者在缓解诱导后的头2年内的严重复发。需要进行随机对照试验,比较RTX与RTX联合CYC的疗效和安全性。
{"title":"Adding low dose cyclophosphamide to rituximab for remission-induction may prolong relapse-free survival in patients with ANCA vasculitis: A retrospective study","authors":"Renée Ysermans ,&nbsp;Matthias H. Busch ,&nbsp;Joop P. Aendekerk ,&nbsp;Jan G.M.C. Damoiseaux ,&nbsp;Pieter van Paassen","doi":"10.1016/j.jtauto.2022.100178","DOIUrl":"10.1016/j.jtauto.2022.100178","url":null,"abstract":"<div><h3>Objective</h3><p>Rituximab (RTX) and cyclophosphamide (CYC) are effective remission-induction therapies in anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV). However, combining these therapies may favor prognosis in patients with a major disease presentation. We conducted a retrospective study to compare patients treated with a combination of RTX and low dose CYC (RTX-CYC) or with RTX only, both followed by tailored maintenance with RTX, with regard to long-term outcomes.</p></div><div><h3>Methods</h3><p>Patients treated in the Maastricht University Medical Center between March 2007 and January 2019, were screened for eligibility. The primary outcome variable was major relapse rate after two and five years. Secondary outcome variables were clinical data and laboratory parameters.</p></div><div><h3>Results</h3><p>Of the 246 screened patients, 34 received RTX-CYC and 28 RTX only for remission-induction. All patients were followed for at least two years, with a median follow-up of 48 months (IQR 24–60). At baseline, renal involvement was more prevalent in the RTX-CYC patients (85% vs. 61%, <em>P</em> = 0.028). Major relapse rates within two years, but not after five years, were significantly lower in the RTX-CYC group (3% vs. 24%, <em>P</em> = 0.032). The rate of infections, hypogammaglobulinemia, end-stage renal disease, malignancies, and mortality did not differ after two and five years.</p></div><div><h3>Conclusion</h3><p>Adding low dose CYC to RTX is safe and may prevent major relapses in patients with severe AAV in the first two years after remission-induction. Randomized controlled trials that compare the efficacy and safety between RTX and a combination of RTX with CYC are needed.</p></div>","PeriodicalId":36425,"journal":{"name":"Journal of Translational Autoimmunity","volume":"6 ","pages":"Article 100178"},"PeriodicalIF":3.9,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9800337/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10471603","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
Antiphospholipid antibodies induce proinflammatory and procoagulant pathways in endothelial cells 抗磷脂抗体诱导内皮细胞的促炎和促凝途径
IF 3.9 Q2 IMMUNOLOGY Pub Date : 2023-01-01 DOI: 10.1016/j.jtauto.2023.100202
Markos Patsouras , Eirini Alexopoulou , Spyros Foutadakis , Eirini Tsiki , Panagiota Karagianni , Marios Agelopoulos , Panayiotis G. Vlachoyiannopoulos

Antiphospholipid syndrome (APS) is an autoimmune thrombophilia characterized by recurrent thrombotic events and/or pregnancy morbidity in the presence of antiphospholipid antibodies detected either as anti-cardiolipin, anti-β2 Glycoprotein I (anti-β2GPI) or Lupus anticoagulant (LA). Endothelial deregulation characterizes the syndrome. To address gene expression changes accompanying the development of autoimmune phenotype in endothelial cells in the context of APS, we performed transcriptomics analysis in Human Umbilical Vein Endothelial Cells (HUVECs) stimulated with IgG from APS patients and β2GPI, followed by intersection of RNA-seq data with published microarray and ChIP-seq results (Chromatin Immunoprecipitation). Our strategy revealed that during HUVEC activation diverse signaling pathways such as TNF-α, TGF-β, MAPK38, and Hippo are triggered as indicated by Gene Ontology (GO) classification and pathway analysis. Finally, cell biology approaches performed side-by-side in naïve and stimulated cultured HUVECs, as well as, in placenta specimens derived from Healthy donors (HDs) and APS-patients verified the evolution of an APS-characteristic gene expression program in endothelial cells during the initial stages of the disease's development.

抗磷脂综合征(APS)是一种自身免疫性血栓形成倾向性疾病,其特征是在存在抗磷脂抗体(抗心磷脂、抗β2糖蛋白I(抗β2GPI)或狼疮抗凝剂(LA))的情况下反复发生血栓事件和/或妊娠发病率。内皮失调是该综合征的特征。为了解决APS背景下内皮细胞中伴随自身免疫表型发展的基因表达变化,我们对用APS患者IgG和β2GPI刺激的人脐静脉内皮细胞(HUVECs)进行了转录组学分析,然后将RNA-seq数据与已发表的微阵列和ChIP-seq结果进行交叉(染色质免疫沉淀)。我们的策略显示,在HUVEC激活过程中,不同的信号通路,如TNF-α、TGF-β、MAPK38和Hippo,如基因本体论(GO)分类和通路分析所示,会被触发。最后,在幼稚和刺激培养的HUVECs中,以及在来自健康供体(HD)和APS患者的胎盘标本中并行进行的细胞生物学方法验证了在疾病发展的初始阶段内皮细胞中APS特征基因表达程序的进化。
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引用次数: 0
Clinical characteristics and outcome of elderly onset adult-onset Still's disease: A 10-year retrospective study 老年发病成人发病斯蒂尔氏病的临床特点和预后:一项10年回顾性研究
IF 3.9 Q2 IMMUNOLOGY Pub Date : 2023-01-01 DOI: 10.1016/j.jtauto.2023.100196
Sheng Li , Shuni Ying , Juan Bai , Yuqian Wang, Changyi Yang, Qingmiao Sun, Hong Fang, Jianjun Qiao

Objective

Our objective was to retrospectively analyze the clinical characteristics and outcome of adult-onset Still's disease (AOSD) patients with elderly onset.

Methods

Retrospective data of patients diagnosed with AOSD in our institute during 2013–2021 were analyzed. The diagnoses were based on the Yamaguchi criteria for AOSD. All long-term follow-up data were collected from medical records and phone calls.

Results

In total, 281 AOSD patients were enrolled in this study, with the median follow-up interval of 47 months. Thirty-two (11.4%, ≥65 years) AOSD patients were classified into the elderly onset groups. Compared to the younger onset group, the percentage of patients with skin rash (p = 0.047), sore throat (p = 0.001), myalgia (p = 0.001), splenomegaly (p = 0.039), hepatosplenomegaly (p = 0.002) and the Pouchot's score (p = 0.002) were significantly lower in the elderly onset group. The death rate (p = 0.014) of elderly onset group is higher than younger onset group, and the independent risk factors of mortality in all AOSD patients were age at onset (HR: 1.115, p = 0.044), disseminated intravascular coagulation (HR: 391.576, p = 0.001) and pleuritis (HR: 23.162, p = 0.033). The probability of relapse was significantly increased in the patients with macrophage activation syndrome (MAS) compared with the patients without MAS (p < 0.001), though the different age groups of AOSD patients with MAS showed no difference in the probability of relapse (p = 0.737).

Conclusion

Elderly onset AOSD patients were distinguished by several distinct clinical features compared to younger onset AOSD patients. The frequency of relapse and complications were similar to that of AOSD patients with elderly or younger onset. A higher mortality rate was observed in elderly onset AOSD patients, and the mortality of AOSD patients was related to age at onset, DIC and pleuritis.

目的回顾性分析老年起病成人起病斯蒂尔氏病(AOSD)患者的临床特点和转归。方法回顾性分析我院2013-2021年诊断为AOSD的患者资料。诊断依据AOSD的Yamaguchi标准。所有的长期随访数据都是从医疗记录和电话中收集的。结果共纳入281例AOSD患者,中位随访时间为47个月。32例(11.4%,≥65岁)AOSD患者分为老年起病组。与年轻起病组相比,老年起病组出现皮疹(p = 0.047)、喉咙痛(p = 0.001)、肌痛(p = 0.001)、脾肿大(p = 0.039)、肝脾肿大(p = 0.002)和Pouchot评分(p = 0.002)的比例显著降低。老年发病组的死亡率(p = 0.014)高于年轻发病组,且所有AOSD患者死亡的独立危险因素为发病年龄(HR: 1.115, p = 0.044)、弥散性血管内凝血(HR: 391.576, p = 0.001)和胸膜炎(HR: 23.162, p = 0.033)。巨噬细胞活化综合征(MAS)患者的复发概率明显高于无MAS患者(p <0.001),但不同年龄组的AOSD合并MAS患者的复发概率没有差异(p = 0.737)。结论老年AOSD与年轻AOSD有明显的临床特征。复发及并发症发生率与老年或低龄AOSD患者相似。高龄AOSD患者病死率较高,且病死率与发病年龄、DIC、胸膜炎有关。
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引用次数: 1
Immunological and translational key challenges in systemic lupus erythematosus: A symposium update 系统性红斑狼疮的免疫学和翻译的关键挑战:研讨会更新
IF 3.9 Q2 IMMUNOLOGY Pub Date : 2023-01-01 DOI: 10.1016/j.jtauto.2023.100199
Yves Renaudineau , Sylviane Muller , Christian M. Hedrich , Dominique Chauveau , Julie Bellière , Sébastien De Almeida , Jan Damoiseaux , Marc Scherlinger , Jean Charles Guery , Laurent Sailler , Chloé Bost

The first LBMR-Tim (Toulouse Referral Medical Laboratory of Immunology) symposium convened on December 16, 2022 in Toulouse, France to address challenging questions in systemic lupus erythematosus (SLE). Special focus was put on (i) the role played by genes, sex, TLR7, and platelets on SLE pathophysiology; (ii) autoantibodies, urinary proteins, and thrombocytopenia contribution at the time of diagnosis and during follow-up; (iii) neuropsychiatric involvement, vaccine response in the COVID-19 era, and lupus nephritis management at the clinical frontline; and (iv) therapeutic perspectives in patients with lupus nephritis and the unexpected adventure of the Lupuzor/P140 peptide. The multidisciplinary panel of experts further supports the concept that a global approach including basic sciences, translational research, clinical expertise, and therapeutic development have to be prioritized in order to better understand and then improve the management of this complex syndrome.

首届LBMR-Tim(图卢兹转诊免疫医学实验室)研讨会于2022年12月16日在法国图卢兹召开,旨在解决系统性红斑狼疮(SLE)的挑战性问题。特别关注(i)基因、性别、TLR7和血小板在SLE病理生理中的作用;(ii)自身抗体、尿蛋白和血小板减少症在诊断和随访期间的贡献;(iii) 2019冠状病毒病(COVID-19)时代的神经精神病学参与、疫苗反应和狼疮性肾炎的临床一线管理;(iv)狼疮性肾炎患者的治疗前景和Lupuzor/P140肽的意外冒险。多学科专家小组进一步支持这一概念,即必须优先考虑包括基础科学、转化研究、临床专业知识和治疗开发在内的全球方法,以便更好地了解并改进这一复杂综合征的管理。
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引用次数: 3
Development and validation of the AF score for diagnosis of adult-onset Still's disease in fever of unknown origin 发展和验证AF评分诊断成人起病不明原因发热斯蒂尔氏病
IF 3.9 Q2 IMMUNOLOGY Pub Date : 2023-01-01 DOI: 10.1016/j.jtauto.2022.100184
Shuni Ying , Duo Lv , Dingxian Zhu , Sheng Li , Yuwei Ding , Chuanyin Sun , Yu Shi , Hong Fang , Jianjun Qiao

Objective

To develop and validate a diagnostic score to identify adult-onset Still's disease (AOSD) in fever of unknown origin (FUO).

Methods

A single center, retrospective case-control study of inpatients with FUO from January 2018 to December 2021. Using clinical and laboratory data from 178 cases with AOSD and 486 cases with FUO, we developed an AOSD/FUO (AF) score with a Bayesian Model Averaging approach. AF score and Yamaguchi's criteria were evaluated by sensitivity, specificity, accuracy, and positive/negative predictive value for diagnosis of AOSD in developmental and validation samples.

Results

Persistent pruritic eruptions (PPEs) in patients with rashes was higher in AOSD group than FUO group (52.3% vs 7.4%; P < 0.01). PPEs yielded a specificity of 97.5% and a sensitivity of 44.9%. AF score = PPEs × 3.795+Evanescent rash × 2.774+Serum ferritin × 1.678+Myalgia × 0.958+Neutrophil count × 0.185+Platelet count × 0.004. A cut-off value ≥ 5.245 revealed the maximizing sensitivity of 88.7% and specificity of 95.8% in discriminating AOSD from FUO in the validation group. And AF score improved the accuracy from 82.6% to 93.3% compared with Yamaguchi's criteria.

Conclusions

We developed and validated a new score which can identify AOSD in FUO with higher classification accuracy than Yamaguchi's criteria. Future multi-centric prospective studies need to be designed to confirm the diagnosis value of AF score.

目的建立并验证不明原因发热(FUO)成人发病斯蒂尔氏病(AOSD)的诊断评分。方法对2018年1月至2021年12月住院的FUO患者进行单中心、回顾性病例对照研究。利用178例AOSD和486例FUO的临床和实验室数据,我们采用贝叶斯模型平均方法建立了AOSD/FUO (AF)评分。在发展样本和验证样本中,评估AF评分和Yamaguchi标准诊断AOSD的敏感性、特异性、准确性和阳性/阴性预测值。结果AOSD组皮疹患者持续瘙痒性发疹(PPEs)发生率高于FUO组(52.3% vs 7.4%;P & lt;0.01)。PPEs的特异性为97.5%,敏感性为44.9%。AF评分= PPEs × 3.795+消逝性皮疹× 2.774+血清铁蛋白× 1.678+肌痛× 0.958+中性粒细胞计数× 0.185+血小板计数× 0.004。截断值≥5.245时,验证组区分AOSD和FUO的最大灵敏度为88.7%,特异度为95.8%。与山口标准相比,AF评分将准确率从82.6%提高到93.3%。结论建立并验证了一种新的诊断FUO中AOSD的评分方法,其分类准确率高于Yamaguchi标准。未来需要设计多中心前瞻性研究来证实房颤评分的诊断价值。
{"title":"Development and validation of the AF score for diagnosis of adult-onset Still's disease in fever of unknown origin","authors":"Shuni Ying ,&nbsp;Duo Lv ,&nbsp;Dingxian Zhu ,&nbsp;Sheng Li ,&nbsp;Yuwei Ding ,&nbsp;Chuanyin Sun ,&nbsp;Yu Shi ,&nbsp;Hong Fang ,&nbsp;Jianjun Qiao","doi":"10.1016/j.jtauto.2022.100184","DOIUrl":"10.1016/j.jtauto.2022.100184","url":null,"abstract":"<div><h3>Objective</h3><p>To develop and validate a diagnostic score to identify adult-onset Still's disease (AOSD) in fever of unknown origin (FUO).</p></div><div><h3>Methods</h3><p>A single center, retrospective case-control study of inpatients with FUO from January 2018 to December 2021. Using clinical and laboratory data from 178 cases with AOSD and 486 cases with FUO, we developed an AOSD/FUO (AF) score with a Bayesian Model Averaging approach. AF score and Yamaguchi's criteria were evaluated by sensitivity, specificity, accuracy, and positive/negative predictive value for diagnosis of AOSD in developmental and validation samples.</p></div><div><h3>Results</h3><p>Persistent pruritic eruptions (PPEs) in patients with rashes was higher in AOSD group than FUO group (52.3% vs 7.4%; <em>P</em> &lt; 0.01). PPEs yielded a specificity of 97.5% and a sensitivity of 44.9%. AF score = PPEs × 3.795+Evanescent rash × 2.774+Serum ferritin × 1.678+Myalgia × 0.958+Neutrophil count × 0.185+Platelet count × 0.004. A cut-off value ≥ 5.245 revealed the maximizing sensitivity of 88.7% and specificity of 95.8% in discriminating AOSD from FUO in the validation group. And AF score improved the accuracy from 82.6% to 93.3% compared with Yamaguchi's criteria.</p></div><div><h3>Conclusions</h3><p>We developed and validated a new score which can identify AOSD in FUO with higher classification accuracy than Yamaguchi's criteria. Future multi-centric prospective studies need to be designed to confirm the diagnosis value of AF score.</p></div>","PeriodicalId":36425,"journal":{"name":"Journal of Translational Autoimmunity","volume":"6 ","pages":"Article 100184"},"PeriodicalIF":3.9,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9826851/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10522716","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
Polyautoimmunity in systemic lupus erythematosus patients: New insights from a cross-sectional study 系统性红斑狼疮患者的多重自身免疫:来自横断面研究的新见解
IF 3.9 Q2 IMMUNOLOGY Pub Date : 2023-01-01 DOI: 10.1016/j.jtauto.2022.100187
Pedro Santos-Moreno , Julián Arias-Aponte , Gabriel-Santiago Rodríguez-Vargas , Paula Daniela Nieto-Zambrano , Laura Villarreal , Linda Ibatá , Susan Martinez , Jaime-Andrés Rubio-Rubio , Pedro Rodríguez , Adriana Rojas-Villarraga

Objective

To assess the frequency of polyautoimmunity (PolyA) in a cohort of Colombian patients with systemic lupus erythematosus (SLE) and to identify associated factors.

Methods

This is an analytical cross-sectional study in a specialized center., a comprehensive review of the medical records of SLE patients was performed from 2015 to 2020 in order to obtain demographic, clinical data, laboratory, and treatment information. Associations between PolyA, demographic, and characteristics of the disease were explored.

Results

A total of 463 patients were included in the analysis. The average age was 47.3 ± 15 years. Most of this population were female (87.4%), whom were diagnosed with SLE in a long-term SLE (10.6 ± 10.1 years). Out of the total patients, 34.7% were diagnosed with PolyA. Among the most frequent clinical criteria for SLICC, arthritis (65%), kidney impairment (39.5%), and alopecia (34.8%) were found. The most frequent SLE-associated PolyA were antiphospholipid syndrome (APS) and Sjögren's syndrome (SS) (16.63% and 10.58%, respectively). PolyA-associated factors were age, xerophthalmia, central nervous system occlusion, and deep vein thrombosis (DVT). In contrast, renal impairment was significantly less frequent in PolyA patients after multivariate analysis.

Conclusion

The results have showed associated factors with PolyA like age, xerophthalmia, central nervous system occlusion, and deep vein thrombosis in this cohort. On the other hand, lupus nephritis was less frequent in patients with PolyA. This study provides a spotlight of a specific SLE population as real-life evidence for a better characterization of PolyA in the future.

目的评估哥伦比亚系统性红斑狼疮(SLE)患者多重自身免疫(PolyA)的发生率,并确定相关因素。方法:本研究是在某专业中心进行的分析性横断面研究。,对2015年至2020年SLE患者的医疗记录进行了全面回顾,以获得人口统计学、临床数据、实验室和治疗信息。探讨了PolyA、人口统计学和疾病特征之间的关系。结果共纳入463例患者。平均年龄47.3±15岁。其中女性居多(87.4%),诊断为长期SLE(10.6±10.1年)。在所有患者中,34.7%被诊断为PolyA。SLICC最常见的临床诊断标准是关节炎(65%)、肾脏损害(39.5%)和脱发(34.8%)。最常见的与slea相关的PolyA是抗磷脂综合征(APS)和Sjögren综合征(SS)(分别为16.63%和10.58%)。与polya相关的因素有年龄、干眼症、中枢神经系统阻塞和深静脉血栓形成。相比之下,多因素分析后,PolyA患者肾脏损害的发生率明显较低。结论年龄、干眼症、中枢神经系统闭塞、深静脉血栓形成等因素与PolyA的发生有关。另一方面,狼疮肾炎在PolyA患者中较少发生。这项研究提供了一个特定SLE人群的焦点,作为未来更好地表征PolyA的现实证据。
{"title":"Polyautoimmunity in systemic lupus erythematosus patients: New insights from a cross-sectional study","authors":"Pedro Santos-Moreno ,&nbsp;Julián Arias-Aponte ,&nbsp;Gabriel-Santiago Rodríguez-Vargas ,&nbsp;Paula Daniela Nieto-Zambrano ,&nbsp;Laura Villarreal ,&nbsp;Linda Ibatá ,&nbsp;Susan Martinez ,&nbsp;Jaime-Andrés Rubio-Rubio ,&nbsp;Pedro Rodríguez ,&nbsp;Adriana Rojas-Villarraga","doi":"10.1016/j.jtauto.2022.100187","DOIUrl":"10.1016/j.jtauto.2022.100187","url":null,"abstract":"<div><h3>Objective</h3><p>To assess the frequency of polyautoimmunity (PolyA) in a cohort of Colombian patients with systemic lupus erythematosus (SLE) and to identify associated factors.</p></div><div><h3>Methods</h3><p>This is an analytical cross-sectional study in a specialized center., a comprehensive review of the medical records of SLE patients was performed from 2015 to 2020 in order to obtain demographic, clinical data, laboratory, and treatment information. Associations between PolyA, demographic, and characteristics of the disease were explored.</p></div><div><h3>Results</h3><p>A total of 463 patients were included in the analysis. The average age was 47.3 ± 15 years. Most of this population were female (87.4%), whom were diagnosed with SLE in a long-term SLE (10.6 ± 10.1 years). Out of the total patients, 34.7% were diagnosed with PolyA. Among the most frequent clinical criteria for SLICC, arthritis (65%), kidney impairment (39.5%), and alopecia (34.8%) were found. The most frequent SLE-associated PolyA were antiphospholipid syndrome (APS) and Sjögren's syndrome (SS) (16.63% and 10.58%, respectively). PolyA-associated factors were age, xerophthalmia, central nervous system occlusion, and deep vein thrombosis (DVT). In contrast, renal impairment was significantly less frequent in PolyA patients after multivariate analysis.</p></div><div><h3>Conclusion</h3><p>The results have showed associated factors with PolyA like age, xerophthalmia, central nervous system occlusion, and deep vein thrombosis in this cohort. On the other hand, lupus nephritis was less frequent in patients with PolyA. This study provides a spotlight of a specific SLE population as real-life evidence for a better characterization of PolyA in the future.</p></div>","PeriodicalId":36425,"journal":{"name":"Journal of Translational Autoimmunity","volume":"6 ","pages":"Article 100187"},"PeriodicalIF":3.9,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/39/48/main.PMC9841268.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9100305","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: 1
期刊
Journal of Translational Autoimmunity
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