Pub Date : 2023-01-01DOI: 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.
{"title":"Autoantibodies to dsDNA in the diagnosis, classification and follow-up of patients with systemic lupus erythematosus","authors":"Jan Damoiseaux, Joyce van Beers","doi":"10.1016/j.jtauto.2023.100191","DOIUrl":"10.1016/j.jtauto.2023.100191","url":null,"abstract":"<div><p>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.</p></div>","PeriodicalId":36425,"journal":{"name":"Journal of Translational Autoimmunity","volume":"6 ","pages":"Article 100191"},"PeriodicalIF":3.9,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9883238/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10593352","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}
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.
{"title":"Preventive plasmapheresis for rituximab related flare in cryoglobulinemic vasculitis","authors":"Léa Fornero , Tarik Kanouni , Jean-Jacques Tudesq , Camille Pochard , Pauline Verot , Wendy Renier , Ludovic Gabellier , Guillaume Cartron , Philippe Guilpain , Charles Herbaux","doi":"10.1016/j.jtauto.2023.100194","DOIUrl":"10.1016/j.jtauto.2023.100194","url":null,"abstract":"<div><h3>Introduction</h3><p>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.</p></div><div><h3>Methods</h3><p>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.</p></div><div><h3>Results</h3><p>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.</p></div><div><h3>Conclusion</h3><p>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.</p></div>","PeriodicalId":36425,"journal":{"name":"Journal of Translational Autoimmunity","volume":"6 ","pages":"Article 100194"},"PeriodicalIF":3.9,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9975310/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10845050","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}
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.
{"title":"Belimumab treatment in autoimmune hepatitis and primary biliary cholangitis – a case series","authors":"Mirjam Kolev , Adela-Cristina Sarbu , Burkhard Möller , Britta Maurer , Florian Kollert , Nasser Semmo","doi":"10.1016/j.jtauto.2023.100189","DOIUrl":"10.1016/j.jtauto.2023.100189","url":null,"abstract":"<div><h3>Background</h3><p>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.</p><p>In patients with refractory AIH and/or PBC, we used a novel treatment strategy with the <em>anti</em>-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.</p></div><div><h3>Aims and methods</h3><p>To retrospectively investigate treatment response in six patients with AIH or PBC with or without concomitant Sjögren's disease treated with the <em>anti</em>-BAFF therapy belimumab at the University Hospital in Bern, Switzerland.</p></div><div><h3>Results</h3><p>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.</p></div><div><h3>Conclusions</h3><p>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.</p></div>","PeriodicalId":36425,"journal":{"name":"Journal of Translational Autoimmunity","volume":"6 ","pages":"Article 100189"},"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/8d/8d/main.PMC9883290.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10590392","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}
Pub Date : 2023-01-01DOI: 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.
{"title":"Risk factors of cardiovascular involvement in patients with Behcet's disease","authors":"Yuqian Wang, Sheng Li, Shunli Tang, Xiaoxuan Cai, Juan Bai, Qingmiao Sun, Jianjun Qiao, Hong Fang","doi":"10.1016/j.jtauto.2023.100195","DOIUrl":"10.1016/j.jtauto.2023.100195","url":null,"abstract":"<div><h3>Objectives</h3><p>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.</p></div><div><h3>Methods</h3><p>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.</p></div><div><h3>Results</h3><p>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%.</p></div><div><h3>Conclusion</h3><p>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.</p></div>","PeriodicalId":36425,"journal":{"name":"Journal of Translational Autoimmunity","volume":"6 ","pages":"Article 100195"},"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/e3/8b/main.PMC9982677.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10845049","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}
Pub Date : 2023-01-01DOI: 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 , Matthias H. Busch , Joop P. Aendekerk , Jan G.M.C. Damoiseaux , 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}
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.
{"title":"Antiphospholipid antibodies induce proinflammatory and procoagulant pathways in endothelial cells","authors":"Markos Patsouras , Eirini Alexopoulou , Spyros Foutadakis , Eirini Tsiki , Panagiota Karagianni , Marios Agelopoulos , Panayiotis G. Vlachoyiannopoulos","doi":"10.1016/j.jtauto.2023.100202","DOIUrl":"10.1016/j.jtauto.2023.100202","url":null,"abstract":"<div><p>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 <em>transcriptomics</em> 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.</p></div>","PeriodicalId":36425,"journal":{"name":"Journal of Translational Autoimmunity","volume":"6 ","pages":"Article 100202"},"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/ac/b2/main.PMC10197110.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9509645","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}
Pub Date : 2023-01-01DOI: 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。
{"title":"Clinical characteristics and outcome of elderly onset adult-onset Still's disease: A 10-year retrospective study","authors":"Sheng Li , Shuni Ying , Juan Bai , Yuqian Wang, Changyi Yang, Qingmiao Sun, Hong Fang, Jianjun Qiao","doi":"10.1016/j.jtauto.2023.100196","DOIUrl":"10.1016/j.jtauto.2023.100196","url":null,"abstract":"<div><h3>Objective</h3><p>Our objective was to retrospectively analyze the clinical characteristics and outcome of adult-onset Still's disease (AOSD) patients with elderly onset.</p></div><div><h3>Methods</h3><p>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.</p></div><div><h3>Results</h3><p>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 (<em>p</em> = 0.047), sore throat (<em>p</em> = 0.001), myalgia (<em>p</em> = 0.001), splenomegaly (<em>p</em> = 0.039), hepatosplenomegaly (<em>p</em> = 0.002) and the Pouchot's score (<em>p</em> = 0.002) were significantly lower in the elderly onset group. The death rate (<em>p</em> = 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, <em>p</em> = 0.044), disseminated intravascular coagulation (HR: 391.576, <em>p</em> = 0.001) and pleuritis (HR: 23.162, <em>p</em> = 0.033). The probability of relapse was significantly increased in the patients with macrophage activation syndrome (MAS) compared with the patients without MAS (<em>p</em> < 0.001), though the different age groups of AOSD patients with MAS showed no difference in the probability of relapse (<em>p</em> = 0.737).</p></div><div><h3>Conclusion</h3><p>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.</p></div>","PeriodicalId":36425,"journal":{"name":"Journal of Translational Autoimmunity","volume":"6 ","pages":"Article 100196"},"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/ee/24/main.PMC10009280.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9129360","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}
Pub Date : 2023-01-01DOI: 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.
{"title":"Immunological and translational key challenges in systemic lupus erythematosus: A symposium update","authors":"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","doi":"10.1016/j.jtauto.2023.100199","DOIUrl":"10.1016/j.jtauto.2023.100199","url":null,"abstract":"<div><p>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.</p></div>","PeriodicalId":36425,"journal":{"name":"Journal of Translational Autoimmunity","volume":"6 ","pages":"Article 100199"},"PeriodicalIF":3.9,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10090709/pdf/main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9317793","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}
Pub Date : 2023-01-01DOI: 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.
{"title":"Development and validation of the AF score for diagnosis of adult-onset Still's disease in fever of unknown origin","authors":"Shuni Ying , Duo Lv , Dingxian Zhu , Sheng Li , Yuwei Ding , Chuanyin Sun , Yu Shi , Hong Fang , 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> < 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}
Pub Date : 2023-01-01DOI: 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.
{"title":"Polyautoimmunity in systemic lupus erythematosus patients: New insights from a cross-sectional study","authors":"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","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}