Journal of Translational Autoimmunity最新文献

{"title":"Hyperimmunoglobulin syndromes: A review of HIGM, HIES, and HIDS","authors":"Wushu Chen ,&nbsp;Xingpei Li ,&nbsp;Junye Mai ,&nbsp;Kailang Tang ,&nbsp;Yingqiao Wang ,&nbsp;Yan-yan Lu ,&nbsp;Jie Liang ,&nbsp;Ni-jiao Li ,&nbsp;Xiu-Yu Qin ,&nbsp;Yu Li ,&nbsp;Lunkai Yao ,&nbsp;Ye Qiu","doi":"10.1016/j.jtauto.2025.100297","DOIUrl":"10.1016/j.jtauto.2025.100297","url":null,"abstract":"<div><div>At present, there is a lack of detailed understanding and research on the pathogenesis and treatment of Hyperimmunoglobulin M syndrome (HIGM), Hyperimmunoglobulin E syndrome (HIES), and hyperimmunoglobulin D syndrome (HIDS), and few studies have been conducted to correlate the pathogenesis and treatment of the three disorders. The existing studies are rarely related to the three diseases. We searched PubMed for a large number of relevant literature and analyzed and summarized the contents. We analyzed and introduced the three diseases and their Categorization, Epidemiology, Clinical manifestations, Laboratory diagnosis, and Treatment by means of tables and Figures. It is hoped that this analysis and summary can play a certain role in the research and treatment of related diseases and promote the understanding and prevention of related diseases.</div></div>","PeriodicalId":36425,"journal":{"name":"Journal of Translational Autoimmunity","volume":"11 ","pages":"Article 100297"},"PeriodicalIF":4.7,"publicationDate":"2025-06-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144556826","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Autoimmune manifestations in children with inborn errors of immunity in Morocco: A study from the national registry","authors":"Ahamada Elamine ,&nbsp;Ibtihal Benhsaien ,&nbsp;Fatima Ailal ,&nbsp;Abderrahmane Errami ,&nbsp;Zakaria Kasmi ,&nbsp;Zahra Aadam ,&nbsp;Asmaa Drissi Bourhanbour ,&nbsp;Ahmed Aziz Bousfiha ,&nbsp;Jalila El Bakkouri","doi":"10.1016/j.jtauto.2025.100299","DOIUrl":"10.1016/j.jtauto.2025.100299","url":null,"abstract":"<div><div>Inborn Errors of Immunity (IEI) are a heterogeneous group of genetic disorders characterized by increased susceptibility to infections and immune dysregulation, including autoimmunity and autoinflammation. Despite their clinical significance, data on autoimmune manifestations in Moroccan pediatric patients with IEI remain limited.</div><div>This study aims to investigate the prevalence, spectrum, and clinical patterns of autoimmune manifestations in pediatric patients with IEI in Morocco.</div><div>We conducted a retrospective analysis of pediatric patients registered in the Moroccan IEI registry from January 2007 to December 2023. Demographic, clinical, and laboratory data were extracted, with a particular focus on autoimmune manifestations.</div><div>Among 769 patients registered in the Moroccan IEI registry, 108 (14 %) exhibited at least one autoimmune manifestation. Consanguinity was observed in 59 (55 %) of cases, and the male-to-female ratio was 1.14. The median age at the onset of clinical symptoms was 10 months (2–33 months), and the median age at IEI diagnosis was 30 months (10.5–84 months). A total of 191 autoimmune manifestations were recorded among these patients, with a notable predominance of autoimmune cytopenia (72 %), followed by cutaneous (10 %) and gastrointestinal (9 %) manifestations. Poly-autoimmunity was present in 47.3 % of affected patients. The most frequently associated IEI subtype with these autoimmune manifestations was common variable immunodeficiency (16,7 %).</div><div>Autoimmune manifestations are a frequent complication in Moroccan children with IEI, with autoimmune cytopenias predominating. A high index of suspicion for IEI should be maintained in patients initially presenting with autoimmunity, particularly autoimmune cytopenia. These patients require personalized management due to their higher risk of mortality.</div></div>","PeriodicalId":36425,"journal":{"name":"Journal of Translational Autoimmunity","volume":"11 ","pages":"Article 100299"},"PeriodicalIF":4.7,"publicationDate":"2025-06-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144490062","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"An international multicenter retrospective analysis of repeated anti-ENA testing in ANA-associated rheumatic diseases, a data-driven proposal to increase testing efficacy","authors":"Wim H.M. Vroemen ,&nbsp;Maria Infantino ,&nbsp;Mariangela Manfredi ,&nbsp;Joyce J.B.C. van Beers ,&nbsp;Carolien Bonroy ,&nbsp;Jan G.M.C. Damoiseaux","doi":"10.1016/j.jtauto.2025.100298","DOIUrl":"10.1016/j.jtauto.2025.100298","url":null,"abstract":"<div><h3>Background</h3><div>Autoantibodies detection in ANA-associated rheumatic diseases (AARD) is not only used for diagnostic and classification purposes, but also for monitoring. In case of AARD it is questioned if repeated anti-ENA testing is of any substantial value. In this international multicenter retrospective study, repeated anti-ENA testing according to local AARD testing algorithms was investigated.</div></div><div><h3>Methods</h3><div>Anti-ENA results (anti-SSA60, -Ro52, -SSB, -Scl-70, -CENP-B, -RNP, -Sm) over a 6 to 10-year period were extracted from the laboratory information systems of three participating centers. Time between repeated testing was determined and concordance analysis was performed.</div></div><div><h3>Results</h3><div>The study included 28557 anti-ENA requests from 19388 patients (72 % female). In 15227 patients (78.5 %) only one anti-ENA test was performed (79.9 % negative), while 4161 patients (21.5 %) underwent multiple (median [interquartile range (IQR)]; 2 [2–4]) tests with a maximum of 31 tests. The median [IQR] time interval between anti-ENA testing for the total cohort was 364 [195–539] days. Concordance analysis demonstrated that repeated anti-ENA test results did not show any change in 3583 patients (86.1 %). Additional autoantibodies were observed in 243 patients (5.8 %). In 76 (1.8 %) patients a positive anti-ENA test was obtained after an initial negative anti-ENA test result, while in 167 (4.0 %) patients additional autoantibodies were detected after an initial positive anti-ENA result.</div></div><div><h3>Conclusions</h3><div>Repeated anti-ENA testing with a median time interval of about one year is common independently of local laboratory testing algorithms, but showed a limited added value since only 1.8 % of the patients have demonstrated a positive anti-ENA test after an initial negative anti-ENA test. These data at least suggest that repeated anti-ENA tests should be discouraged and only be instigated by a change in clinical manifestations.</div></div>","PeriodicalId":36425,"journal":{"name":"Journal of Translational Autoimmunity","volume":"11 ","pages":"Article 100298"},"PeriodicalIF":4.7,"publicationDate":"2025-06-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144481543","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Dysregulation of innate and adaptive lymphoid immunity may have implications for symptom attribution and predict responses to targeted therapies in neuropsychiatric systemic lupus erythematosus","authors":"Julius Lindblom ,&nbsp;Guillermo Barturen ,&nbsp;Lorenzo Beretta ,&nbsp;Daniel Toro-Domínguez ,&nbsp;Elena Carnero-Montoro ,&nbsp;Maria Orietta Borghi ,&nbsp;Jessica Castillo ,&nbsp;Ellen Iacobaeus ,&nbsp;Yvonne Enman","doi":"10.1016/j.jtauto.2025.100296","DOIUrl":"10.1016/j.jtauto.2025.100296","url":null,"abstract":"<div><h3>Objectives</h3><div>To gain insights into the pathogenesis of neuropsychiatric systemic lupus erythematosus (NPSLE) and identify potential drug targets through investigation of whole-blood human transcriptome.</div></div><div><h3>Methods</h3><div>We analysed differentially expressed genes in peripheral blood from active central nervous system (CNS) lupus (n = 26) and active non-neuropsychiatric SLE (n = 38) patients versus healthy controls (n = 497) from the European PRECISESADS project (NTC02890121). We further explored dysregulated gene modules in active CNS lupus and their correlation with serological markers. Lastly, we performed regulatory network and druggability analysis.</div></div><div><h3>Results</h3><div>Unsupervised weighted gene co-expression network analysis (WGCNA) revealed 23 dysregulated gene modules and two subgroups of active CNS lupus. The interferon gene module was prominently upregulated in subgroup 1, while the B cell, T cell, and cytotoxic/natural killer (NK) cell modules were downregulated. Subgroup 2 showed less marked dysregulation patterns. Subgroup 1 had lower estimated proportions of lymphoid cell subsets and proportionally more patients positive for anti-dsDNA antibodies compared to subgroup 2, pointing to molecularly distinct subgroups or misclassification of subgroup 2. <em>In silico</em> prediction algorithms demonstrated a greater anticipated response to anifrolumab, C3 inhibitors, and calcineurin inhibitors for patients in CNS lupus subgroup 1 compared with subgroup 2.</div></div><div><h3>Conclusions</h3><div>Gene dysregulation patterns related to innate and adaptive lymphoid immunity separated active CNS lupus patients into two distinct subgroups with differential anticipated response to type I interferon, C3, and calcineurin inhibition. Our study provides a conceptual framework for precision medicine in NPSLE and implications for overcoming the major clinical challenge of attributing neuropsychiatric features to SLE versus other causes.</div></div>","PeriodicalId":36425,"journal":{"name":"Journal of Translational Autoimmunity","volume":"11 ","pages":"Article 100296"},"PeriodicalIF":4.7,"publicationDate":"2025-06-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144502082","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"The ‘autoimmunome’ of centenarians","authors":"Pedro Carrera-Bastos ,&nbsp;Abel Plaza-Florido ,&nbsp;Alejandro Santos-Lozano ,&nbsp;Vânia Borba ,&nbsp;Gabriel Rodríguez-Romo ,&nbsp;Celia García-Chico ,&nbsp;Simone Lista ,&nbsp;Gonzalo Saco-Ledo ,&nbsp;Enzo Emanuele ,&nbsp;Yehuda Shoenfeld ,&nbsp;Alejandro Lucia","doi":"10.1016/j.jtauto.2025.100295","DOIUrl":"10.1016/j.jtauto.2025.100295","url":null,"abstract":"<div><h3>Objective</h3><div>To identify signature proteins potentially linked to resistance to autoimmunity in the blood of centenarians.</div></div><div><h3>Methods</h3><div>We conducted <em>in silico</em> data mining of previously published proteomic results using the Search Tool for the Retrieval of Interacting Genes/Proteins (STRING) and PHENOPEDIA databases.</div></div><div><h3>Results</h3><div>Sixteen autoimmune disease-related proteins were identified within the proteomic signatures of centenarians. Albumin was the most connected hub protein, notably elevated in centenarians compared to younger controls, suggesting a protective role. Eight of the identified autoimmunity-related proteins—ADIPOQ, C1S, C5, C7, C9, CFD, MASP1, and SERPING1—were associated with the complement system.</div></div><div><h3>Conclusion</h3><div>Elevated albumin levels and a prominent complement system presence in centenarians' blood proteome may contribute to resistance to autoimmunity, highlighting potential protective mechanisms against autoimmune diseases in extreme longevity.</div></div>","PeriodicalId":36425,"journal":{"name":"Journal of Translational Autoimmunity","volume":"11 ","pages":"Article 100295"},"PeriodicalIF":4.7,"publicationDate":"2025-06-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144255113","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"A comparative study of different assays for autoantibodies detection in patients with autoimmune gastritis","authors":"Małgorzata Osmola ,&nbsp;Caroline Hémont ,&nbsp;Marcin Romańczyk ,&nbsp;Amaury Druet ,&nbsp;Nicolas Chapelle ,&nbsp;Tamara Matysiak-Budnik ,&nbsp;Marco Vincenzo Lenti ,&nbsp;Jérôme C. Martin","doi":"10.1016/j.jtauto.2025.100294","DOIUrl":"10.1016/j.jtauto.2025.100294","url":null,"abstract":"<div><h3>Objective</h3><div>Autoimmune gastritis (AIG) is an important health problem and a risk factor for gastric neoplasms. This study assessed the diagnostic performance of different assays for anti-parietal cell antibodies (APCA) and anti-intrinsic factor antibodies (AIFA) in patients with histologically confirmed AIG.</div></div><div><h3>Methods</h3><div>This prospective, multicenter study included 50 AIG patients and 93 controls. The diagnostic performance of fluorescent enzyme immunoassay (FEIA) and immunoblot was evaluated for the detection of both APCA and AIFA, while indirect immunofluorescence (IIF) was assessed for APCA only.</div></div><div><h3>Results</h3><div>Overall, AIFA detection using FEIA demonstrated slightly better performance (specificity [Sp] 100 %, positive predictive value [PPV] 100 %, negative predictive value [NPV] 75 %) compared to immunoblot (Sp 98.9 %, PPV 94.1 %, NPV 73 %). However, both methods showed low sensitivity (Se): 38 % for FEIA and 32 % for immunoblot. When the FEIA cut-off was adjusted using ROC curve analysis, Se increased to 50 %, while maintaining high Sp (98.9 %). For APCA detection, Se was similar across all methods (∼80 %), but Sp varied: immunoblot showed lower Sp (89.3 %) compared to IIF (98.8 %) and FEIA (95.7 %). PPV was highest for IIF (97.5 %), followed by FEIA (89.9 %) and immunoblot (89.3 %). NPV was lowest for immunoblot (80 %), while IIF and FEIA showed comparable values (89.5 % and 90.9 %, respectively). Adjusting the FEIA cut-off for APCA increased Sp to 98.9 % without reducing Se (76 %). Combining AIFA and APCA testing improved diagnostic performance, yielding a sensitivity of 90 % and specificity of 95.7 %.</div></div><div><h3>Conclusions</h3><div>FEIA offers superior diagnostic accuracy for APCA and AIFA testing in AIG. The highest diagnostic yield for AIG is observed when both APCA and AIFA are assessed. This approach could be clinically applicable in the screening for AIG and diagnostic process of AIG.</div></div>","PeriodicalId":36425,"journal":{"name":"Journal of Translational Autoimmunity","volume":"10 ","pages":"Article 100294"},"PeriodicalIF":4.7,"publicationDate":"2025-05-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144167145","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Epigenetic regulation of thrombo-inflammation in Behçet and antiphospholipid syndrome","authors":"Alessandra Bettiol ,&nbsp;Giacomo Bagni ,&nbsp;Francesca Di Patti ,&nbsp;Elena Lastraioli ,&nbsp;Alice Barinotti ,&nbsp;Massimo Radin ,&nbsp;Savino Sciascia ,&nbsp;Domenico Prisco ,&nbsp;Annarosa Arcangeli ,&nbsp;Giacomo Emmi","doi":"10.1016/j.jtauto.2025.100293","DOIUrl":"10.1016/j.jtauto.2025.100293","url":null,"abstract":"<div><h3>Background</h3><div>An epigenetic regulation of thrombo-inflammation has been reported in Behçet syndrome (BS), likely driven by a unique profile of three plasmatic circulating microRNAs (ci-miRNAs) (miR-206, miR-224-5p, and miR-653-5p). We compared this ci-miRNAs expression in BS and antiphospholipid syndrome (APS), the prototype of acquired pro-thrombotic autoimmune disease. To further corroborate the hypothesis that shared mechanisms drive the thrombotic process in BS and APS, we further assessed their thrombin generation assay (TGA) profile.</div></div><div><h3>Methods</h3><div>The three ci-miRNA expression was evaluated in 39 patients with BS, 33 with APS and 30 healthy controls (HCs). Single marker and combined ROC curve analyses were performed. TGA was conducted on pre-collected platelet poor plasma samples from 35 patients with BS and 77 with APS.</div></div><div><h3>Results</h3><div>The three ci-miRNAs, taken individually or combined, lacked acceptable discriminating power between groups [AUC from combiROC 0.64 (95 % CI: 0.51–0.78)]. Conversely, in the subgroups of BS and APS patients with vascular involvement (n = 22 each), the combined signature well discriminated between the two syndromes [AUC 0.83 (0.71–0.96), specificity 0.91, sensitivity 0.77], as well as between thrombotic APS and HCs [AUC 0.79 (0.64–0.91)]. Also, distinct trends in thrombograms emerged between BS and APS, with BS TGA displaying lower tLag and tPeak, higher Peak and similar AUC as compared to APS.</div></div><div><h3>Conclusions</h3><div>Despite shared elements in the ci-miRNA regulation of their pro-thrombotic tendency, distinct epigenetic factors seem to contribute to the pathogenesis of vascular events in BS and APS, possibly accounting also for the different global clotting assay observed in these diseases.</div></div>","PeriodicalId":36425,"journal":{"name":"Journal of Translational Autoimmunity","volume":"11 ","pages":"Article 100293"},"PeriodicalIF":4.7,"publicationDate":"2025-05-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144212458","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Characteristics of polyclonal anti-interferon-gamma autoantibodies and novel diagnostic strategies: A prospective cohort study of new biomarkers","authors":"Xidong Wang ,&nbsp;Feng Ye ,&nbsp;Hongling Liu ,&nbsp;Shaoqiang Li ,&nbsp;Jinglu Yang ,&nbsp;Xue Yu ,&nbsp;Yilei Hui ,&nbsp;Yongming Li ,&nbsp;Yangqing Zhan ,&nbsp;Yan Wang ,&nbsp;Jing Liu ,&nbsp;Zhengtu Li","doi":"10.1016/j.jtauto.2025.100292","DOIUrl":"10.1016/j.jtauto.2025.100292","url":null,"abstract":"<div><h3>Background</h3><div><em>Anti</em>-γ interferon autoantibody (AIGA) syndrome is a widespread and grossly underestimated immunodeficiency disorder characterized by high mortality rates and a lack of standardized diagnostic methods. A highly accurate AIGA biomarker that meets the requirements of absolute quantification is urgently needed to enable the early diagnosis and treatment monitoring of the disease. In our study, we aimed to identify the primary components of AIGAs, determine their function, and develop a novel diagnostic method.</div></div><div><h3>Methods</h3><div>Immune repertoire sequencing and a retrospective antibody subtype index analysis were performed for typical patients. Affinity chromatography was used to isolate and purify IgGs from AIGAs in the plasma of AIGA(+) patients. The clinical application value of chromatography for testing AIGAs was evaluated in a prospective clinical cohort.</div></div><div><h3>Results</h3><div>A total of 114 eligible subjects were enrolled. Immune repertoire sequencing revealed that 74 % of the AIGA(+) patients had IgG clone types, with the somatic hypermutation (SHM) analysis being the most informative. We isolated AIGAs from the blood and interpreted their affinity and major components completely. Based on the results of this prospective cohort study, AIGAs, an absolute quantitative biomarker, were significantly better than the ELISA method (Delong test, P = 0.0018).</div></div><div><h3>Conclusions</h3><div>Patients with AIGA syndrome have abnormally elevated IgG levels, with IgG3 subtypes dominating. The disorder is characterized by the rapid acquisition of polyclonal AIGAs. The obtained AIGAs had a good neutralization capacity and potential as absolute quantitative biomarkers.</div></div>","PeriodicalId":36425,"journal":{"name":"Journal of Translational Autoimmunity","volume":"10 ","pages":"Article 100292"},"PeriodicalIF":4.7,"publicationDate":"2025-05-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144071497","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Autoantibodies as prognostic markers in rheumatoid arthritis","authors":"Carl Turesson ,&nbsp;Johan Rönnelid ,&nbsp;Alf Kastbom","doi":"10.1016/j.jtauto.2025.100291","DOIUrl":"10.1016/j.jtauto.2025.100291","url":null,"abstract":"<div><h3>Background and purpose</h3><div>Rheumatoid arthritis (RA) is a chronic inflammatory disorder characterized by progressively destructive polyarthritis. Key autoimmune features include the presence of autoantibodies. The purpose of this review is to discuss the diagnostic and prognostic properties of rheumatoid factor (RF) and anti-citrullinated protein/peptide antibodies (ACPA), based on current use in Sweden. Furthermore, we discuss their relation to disease outcomes and their importance for management of patients with RA.</div></div><div><h3>Principal findings</h3><div>Based on current cut-offs, ACPA has a higher specificity for RA than RF, and testing for ACPA alone is recommended when investigating patients with clinically suspect RA. The diagnostic utility of RF may improve with adjusted reference range/upper limit of normal.</div><div>RF and ACPA both predict rapid radiographic progression, severe extra-articular manifestations and mortality, whereas other outcomes, such as osteoporosis, pain and disability are not as clearly related to seropositivity. RF/ACPA positive patients respond better to some targeted therapies, in particular rituximab, compared to seronegative RA patients. Recent studies indicate that treatment of ACPA-positive arthralgia with abatacept may delay and perhaps sometimes even prevent development of arthritis. Available evidence does not support an added value of repeated RF or ACPA testing.</div></div><div><h3>Conclusions</h3><div>Testing for ACPA in patients with arthralgia or suspected early RA, and for ACPA and RF at RA diagnosis, provides useful diagnostic and prognostic information, which has implications for follow-up and treatment. Repeated testing for ACPA and RF is not recommended. Potential future developments include treatment of ACPA-positive individuals for prevention of arthritis.</div></div>","PeriodicalId":36425,"journal":{"name":"Journal of Translational Autoimmunity","volume":"10 ","pages":"Article 100291"},"PeriodicalIF":4.7,"publicationDate":"2025-05-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143907781","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Immune characterization of lupus nephritis patients undergoing dialysis","authors":"Quentin Simon ,&nbsp;François Gaillard ,&nbsp;John Tchen ,&nbsp;Delphine Bachelet ,&nbsp;Karim Sacré ,&nbsp;Katell Peoc'h ,&nbsp;Noémie Jourde-Chiche ,&nbsp;Eric Daugas ,&nbsp;Nicolas Charles","doi":"10.1016/j.jtauto.2025.100290","DOIUrl":"10.1016/j.jtauto.2025.100290","url":null,"abstract":"<div><div>Systemic lupus erythematosus (SLE) activity decreases in some patients with end-stage kidney disease (ESKD). The impact of ESKD on the immune cell profile of SLE patients and lupus activity remains unclear. In this study, we aimed at characterizing immunologically inactive and active SLE patients undergoing dialysis therapy. Based on multi-parametric flow cytometry assays, an extensive immunophenotyping was performed on blood samples from 47 SLE patients undergoing hemodialysis, 10 non-dialyzed SLE patients with active lupus nephritis (aLN), 6 non-dialyzed patients with a history of LN currently in remission (rLN), and 20 healthy volunteers (HV) as controls (<span><span>ClinicalTrials.gov</span><svg><path></path></svg></span> Identifier: NCT03921398). The hemodialysis group was composed of 16 SLE patients with inactive disease (iHD), 22 with sustained low disease activity with a non-renal SLEDAI ≤4 (aHD≤4), and 9 highly active SLE patients (aHD&gt;4). A factorial discriminant analysis was performed to validate the association between immune cell signatures and lupus activity. By compiling 12 cellular variables, we describe immune profiles related to highly active SLE patients or associated with both inactive and low-disease activity groups. As non-dialyzed active SLE patients, active patients undergoing hemodialysis showed a specific combination of increased numbers of circulating CD19^hi CD27^– “atypical naive” B cells, plasmablasts, CD16⁺ inflammatory monocytes and a basopenia. This study brings a comprehensive overview of immune cell signatures observed in SLE patients undergoing dialysis. We propose a simple immunophenotypic approach for the assessment of lupus activity that may provide help to data-driven personalized medicine in hemodialyzed SLE patients.</div></div>","PeriodicalId":36425,"journal":{"name":"Journal of Translational Autoimmunity","volume":"10 ","pages":"Article 100290"},"PeriodicalIF":4.7,"publicationDate":"2025-05-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143904191","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}