Autoimmunity reviews最新文献

{"title":"Janus kinase inhibitors for psoriatic arthritis: Evidence from a systematic review and network meta-analysis","authors":"Sotirios G. Tsiogkas ,&nbsp;Arriana Gkouvi ,&nbsp;Katerina Maria Kontouli ,&nbsp;Theodora Simopoulou ,&nbsp;Maria G. Grammatikopoulou ,&nbsp;Dimitrios P. Bogdanos","doi":"10.1016/j.autrev.2025.103819","DOIUrl":"10.1016/j.autrev.2025.103819","url":null,"abstract":"<div><div>Psoriatic arthritis (PsA) constitutes a heterogeneous disease. Diagnosis is commonly made by identifying joint inflammation, dactylitis, enthesitis, or axial spine involvement in cutaneous or nail psoriasis. Janus kinases (JAKs) are intracellular kinases that mediate cytokine signaling. The present network meta-analysis aimed to provide a comprehensive summary regarding the use of JAK inhibitors (JAKis) in PsA. We systematically searched MEDLINE, CENTRAL, Web of Science databases, and the <span><span>clinicaltrials.gov</span><svg><path></path></svg></span> registry until October 2023 and included randomized controlled trials (RCTs). Examined outcomes consisted of the proportion of participants achieving ACR20, ACR50, ACR70, PASI75, resolution of enthesitis, resolution of dactylitis, and experiencing serious adverse events (SAEs). Changes in the Health Assessment Questionnaire Disability Index (HAQ-DI) from the baseline were also recorded. The revised Cochrane Risk of Bias 2.0 tool determined the risk of bias. Networks were constructed, and random-effects frequentist analyses were employed utilizing the placebo arms from each study. Most JAKis were superior to placebo for all examined outcomes. Statistical advantages of one JAKi over another were recorded, and all concerned upadacitinib. However, the safety profile of upadacitinib did not differ significantly from that of other JAKis. Our network meta-analysis is the first to concentrate on comparable, current outcome data of JAKis in PsA on clinical efficacy. It reveals statistically significant variations in the advantages and disadvantages among JAKis in PsA, which require further meticulous assessment.</div></div>","PeriodicalId":8664,"journal":{"name":"Autoimmunity reviews","volume":"24 6","pages":"Article 103819"},"PeriodicalIF":9.2,"publicationDate":"2025-04-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143881449","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Autophagy in myositis, a dysregulated pathway, and a target for therapy","authors":"A.S. Kamalanathan ,&nbsp;Vikas Agarwal ,&nbsp;Laura Talamini ,&nbsp;Sylviane Muller","doi":"10.1016/j.autrev.2025.103817","DOIUrl":"10.1016/j.autrev.2025.103817","url":null,"abstract":"<div><div>Corticosteroids and immunosuppressants are the mainstay of therapy for idiopathic inflammatory myopathies (IIMs). However, a significant therapeutic challenge extends beyond mitigating inflammation with these agents in achieving meaningful improvements in muscle strength and physical function, a goal that remains largely unmet. IIMs encompass a heterogeneous group of autoimmune disorders, including dermatomyositis, polymyositis, necrotizing autoimmune myopathy, inclusion body myositis, and others, characterized by chronic muscle inflammation, progressive weakness, and fatigue. The etiology of IIMs remains poorly understood, though potential contributors include environmental triggers (<em>e.g.</em>, infections, medications, or injury) and genetic predisposition. To advance the development of novel therapeutic strategies, it is critical to elucidate the dysfunctional molecular and cellular pathways underlying IIM pathogenesis. Among these, dysregulated autophagy pathways have emerged as a promising target for therapeutic intervention. Specifically, impairments in lysosomal autophagy and mitophagy have been implicated in IIMs, and modulating these processes through targeted regulatory mechanisms may offer therapeutic benefits. This review provides a comprehensive synthesis of clinical and biological features of IIMs, the current diagnostic approaches and emerging biomarkers, evaluates the utility of existing biomarkers, and examines the relevance of animal models in IIM research. Furthermore, we explore the role of autophagic dysregulation in disease pathogenesis and provide a critical appraisal of current treatment modalities. Finally, we highlight emerging therapeutic targets and regulatory molecules under investigation, with a particular focus on autophagy modulation. Notably, autophagy inhibitors represent a novel and potentially transformative therapeutic avenue for patients with IIMs, offering hope for improved clinical outcomes.</div></div>","PeriodicalId":8664,"journal":{"name":"Autoimmunity reviews","volume":"24 7","pages":"Article 103817"},"PeriodicalIF":9.2,"publicationDate":"2025-04-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143878335","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"The entrenchment of NLRP3 inflammasomes in autoimmune disease-related inflammation","authors":"Valeria Carnazzo ,&nbsp;Donato Rigante ,&nbsp;Giuliana Restante ,&nbsp;Valerio Basile ,&nbsp;Krizia Pocino ,&nbsp;Umberto Basile","doi":"10.1016/j.autrev.2025.103815","DOIUrl":"10.1016/j.autrev.2025.103815","url":null,"abstract":"<div><div>Autoinflammation and autoimmunity are almost “opposite” phenomena characterized by chronic activation of the immune system, ‘innate’ in the first and ‘adaptive’ in the second, leading to inflammation of several tissues with specific protean effectors of tissue damage. The mechanism of involvement of multiprotein complexes called ‘inflammasomes’ within autoimmune pictures, differently from autoinflammatory conditions, is yet undeciphered. In this review we provide a comprehensive overview on NLRP3 inflammasome contribution into the pathogenesis of some autoimmune diseases. In response to autoantibodies against nucleic acids or tissue-specific antigens the NLRP3 inflammasome is activated within dendritic cells and macrophages of patients with systemic lupus erythematosus. Crucial is NLRP3 inflammasome to amplify tissue inflammation with interleukin-1 overexpression and matrix metalloproteinase production at the joint level in rheumatoid arthritis. A deregulated NLRP3 inflammasome activation occurs in the serous acini of salivary and lacrimal glands prone to Sjogren's syndrome, but also in the inflammatory process involving endothelial cells, leucocyte recruitment, and platelet plugging of vasculitides. Furthermore, organ-specific autoimmune diseases such as thyroiditis and hepatitis may display hyperactive NLRP3 inflammasomes at the level of resident immune cells within thyroid or liver, respectively. Therefore, it is not unexpected that preclinical studies have shown how specific inflammasome inhibitors may significantly overthrow the severity of different autoimmune diseases and slow down their trend towards an ominous progression. Specific markers of inflammasome activation could also reveal subclinical inflammatory components escaping conventional diagnostic approaches or improve monitoring of autoimmune diseases and personalizing their treatment.</div></div>","PeriodicalId":8664,"journal":{"name":"Autoimmunity reviews","volume":"24 7","pages":"Article 103815"},"PeriodicalIF":9.2,"publicationDate":"2025-04-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143833289","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"The role of CRISPR-Cas9 and CRISPR interference technologies in the treatment of autoimmune diseases","authors":"Zahra Khademi ,&nbsp;Negar Mottaghi-Dastjerdi ,&nbsp;Hamed Morad ,&nbsp;Amirhossein Sahebkar","doi":"10.1016/j.autrev.2025.103816","DOIUrl":"10.1016/j.autrev.2025.103816","url":null,"abstract":"<div><div>Autoimmune disorders can be described as inappropriate immune responses directed against self-antigens, which account for substantial healthcare concerns around the world. Immunosuppression or immune modulation are the main therapeutic modalities for autoimmune disorders. These modalities, however, impair the ability of the immune system to fight against infections, thereby predisposing to opportunistic diseases. This review explores existing therapies for autoimmune disorders, highlighting their limitations and challenges. Additionally, it describes the potential of CRISPR-Cas9 technology as a novel therapeutic approach to address these challenges.</div></div>","PeriodicalId":8664,"journal":{"name":"Autoimmunity reviews","volume":"24 7","pages":"Article 103816"},"PeriodicalIF":9.2,"publicationDate":"2025-04-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143829427","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Cochlear T cells and their role in health and disease: A systematic review","authors":"Evi De Backer ,&nbsp;Dorien Verdoodt ,&nbsp;Peter Ponsaerts ,&nbsp;Emanuela Pasciuto ,&nbsp;Vincent Van Rompaey","doi":"10.1016/j.autrev.2025.103814","DOIUrl":"10.1016/j.autrev.2025.103814","url":null,"abstract":"<div><h3>Background</h3><div>The role of T cells in health and disease has already been studied extensively in many organs, yet their activity in the cochlea and involvement in hearing loss remains less explored. This review aims to summarize current existing literature on the presence and activity of T cells in the cochlea and the link between T-cell activity and the development of hearing loss.</div></div><div><h3>Methods</h3><div>A systematic review of the literature was performed on PubMed and Web of Science on the 4th of December 2024 using the following search term: (“T-cell” OR “T cells” OR “T-lymphocyte*”) AND (“cochlea*” OR “spiral ligament” OR “spiral limbus”).</div></div><div><h3>Results</h3><div>The literature search revealed 20 studies that explored the presence and activity of T cells in the cochlea, as well as associations between T cells and hearing loss. The presence of cochlear T cells was compared between steady-state conditions and stimulated environments, which suggested an increase in cochlear T cells post-stimulation. Additionally, the role of T cells in hearing loss, both causal as protective, are described in 12 studies. Finally, three studies introduce cochlin as an inner ear-specific antigen triggering autoimmunity.</div></div><div><h3>Conclusion</h3><div>This review highlights the critical role of the immune balance in maintaining cochlear homeostasis. Both protective and detrimental T-cell functions have been linked to hearing, reflecting the dual role of T cells in cochlear health. Future therapies for hearing loss should aim to restore the immune balance to support normal hearing functions.</div></div>","PeriodicalId":8664,"journal":{"name":"Autoimmunity reviews","volume":"24 7","pages":"Article 103814"},"PeriodicalIF":9.2,"publicationDate":"2025-04-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143838235","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Molecular/antigenic mimicry and immunological cross-reactivity explains SARS-CoV-2-induced autoimmunity","authors":"Yekbun Adiguzel ,&nbsp;Dimitros P. Bogdanos ,&nbsp;Yehuda Shoenfeld","doi":"10.1016/j.autrev.2025.103811","DOIUrl":"10.1016/j.autrev.2025.103811","url":null,"abstract":"<div><div>COVID-19 pandemic is over, but its effects on chronic illnesses remain a challenging issue. Understanding the influence of SARS-COV-2-mediated autoimmunity and overt autoimmune disease is of paramount importance, as it can provide a critical mass of information regarding both infection-mediated (and vaccination-induced) autoimmune phenomena in susceptible individuals during the disease course, and short or long-term post-disease sequelae. The high prevalence of organ and non-organ specific autoantibody positivity in patients with COVID-19 led to studies attempting to delineate the origin and the underlying mechanism responsible for their induction nature, identifying novel autoantigens, and the self-epitope sequences which could be the impetus for the initiating autoreactive responses. Herein, we provide a meticulous review of the studies reporting those mimicking sequences that have been experimentally validated, based on the assumption that molecular mimicry and immunological crossreactivity may account for autoantibody development. Most reports are based on bioinformatics approaches, and only a disproportionally small number of studies currently demonstrate immunological crossreactivity. We took the opportunity to further review and searched for the linear human epitope sequences of human, through the epitopes deposited at the Immune Epitope Database. This included an analysis of autoimmune disease as the disease data to comprehensively understand the subject matter. The critical overview of the findings underscore the urgent and immense need for further research to gain a comprehensive understanding of the mechanisms involved and the anticipated appraisal that molecular mimicry and immunological crossreactivity is indeed central to the loss of immunological tolerance during SARS-COV-2 infection.</div></div>","PeriodicalId":8664,"journal":{"name":"Autoimmunity reviews","volume":"24 7","pages":"Article 103811"},"PeriodicalIF":9.2,"publicationDate":"2025-04-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143833178","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"The impact of pregnancy on future health in Rheumatoid Arthritis: A systematic review of the literature","authors":"Bethan Goulden ,&nbsp;George Woodward ,&nbsp;Sophie Leiner ,&nbsp;Zahra Ahmed ,&nbsp;Sophie Covington ,&nbsp;Diane Nzelu ,&nbsp;Radboud Dolhain ,&nbsp;Ian Giles","doi":"10.1016/j.autrev.2025.103808","DOIUrl":"10.1016/j.autrev.2025.103808","url":null,"abstract":"<div><h3>Objectives</h3><div>To assess whether obstetric history predicts future rheumatoid arthritis (RA) diagnosis, severity, and/or maternal health beyond the immediate postpartum period.</div></div><div><h3>Methods</h3><div>A systematic literature search was conducted on 01/07/24 (PubMed, Embase); PROSPERO ID CRD42024559893. Primary research examining health outcomes in RA-affected females, stratified on obstetric history pre- or post-RA onset, were selected for inclusion. Studies of overlapping cohorts were included if differing exposures/outcomes reported.</div></div><div><h3>Results</h3><div>Out of 3333 articles screened, 95 studies were selected. Future health outcomes analysed included RA diagnosis (<em>n</em> = 66 studies), severity (<em>n</em> = 11), cardiovascular disease (<em>n</em> = 2), immunity (<em>n</em> = 9), and microchimerism (<em>n</em> = 7). Parity/gravidity (<em>n</em> = 67), infertility (n = 7), and pregnancy loss (<em>n</em> = 22) were not reliable predictors of subsequent RA. High parity (n = 2) was linked to increased cardiovascular disease risk in RA-affected females. Both pre-eclampsia (<em>n</em> = 4) and delivery of a low birthweight infant (n = 2) were associated with RA diagnosis/severity. A trend suggested increased RA risk after preterm birth (<em>n</em> = 3) and severe hyperemesis gravidarum (n = 3), but not for gestational diabetes (<em>n</em> = 1). No significant differences in post-translational modification of serum proteins were noted beyond 6 months postpartum, though persistent differences in anti-HLA antibodies and microchimerism were observed.</div></div><div><h3>Conclusions</h3><div>Research indicates that parity, gravidity, infertility, and pregnancy loss do not adversely affect RA development. Conversely, low birthweight delivery was associated with RA diagnosis and severity, while pre-eclampsia correlated with subsequent RA diagnosis. Differences in immune responses, as indicated by anti-HLA and microchimerism, may indicate immune sensitisation relevant to RA pathogenesis. The predictive impact of pre-eclampsia and gestational diabetes on cardiovascular health in RA-affected females remains unstudied.</div></div>","PeriodicalId":8664,"journal":{"name":"Autoimmunity reviews","volume":"24 7","pages":"Article 103808"},"PeriodicalIF":9.2,"publicationDate":"2025-04-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143816447","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Emerging roles of mechanically activated ion channels in autoimmune disease","authors":"Zhiru Zeng ,&nbsp;Engeng Chen ,&nbsp;Jing Xue","doi":"10.1016/j.autrev.2025.103813","DOIUrl":"10.1016/j.autrev.2025.103813","url":null,"abstract":"<div><div>Mechanically activated (MA) ion channels have rapidly gained prominence as vital conduits bridging aberrant mechanical cues in tissues with the dysregulated immune responses at the core of autoimmune diseases. Once regarded as peripheral players in inflammation, these channels, exemplified by PIEZO1, TRPV4, and specific K2P family members, now play a central role in modulating T-cell effector functions, B- cell activation and the activity of macrophages and dendritic cells. Their gating is intimately tied to physical distortions such as increased tissue stiffness, osmotic imbalances, or fluid shear, triggering a cascade of ionic fluxes that elevate proinflammatory signaling and drive tissue-destructive loops. Recognition of these channels as central mediators of mechanical stress-induced inflammation responses in autoimmune pathogenesis is rapidly expanding. In parallel, the emerging therapeutic strategies aim to restrain overactive mechanosensors or selectively harness them in affected tissues. Small molecules, peptide blockers, and gene-targeting approaches show preclinical promise, although off-target effects and the broader homeostatic roles of these channels warrant caution. This review explores how integrating mechanobiological concepts with established immunological paradigms enables a more detailed understanding of autoimmune pathogenesis. By elucidating how mechanical forces potentiate or dampen pathological immunity, we propose innovative strategies that exploit mechanosensitivity to recalibrate immune responses across a spectrum of autoimmune conditions.</div></div>","PeriodicalId":8664,"journal":{"name":"Autoimmunity reviews","volume":"24 7","pages":"Article 103813"},"PeriodicalIF":9.2,"publicationDate":"2025-04-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143791335","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"The burden of extracutaneous manifestations in juvenile localized scleroderma: A literature review","authors":"Ilaria Liguoro ,&nbsp;Gabriele Simonini ,&nbsp;Giorgia Martini","doi":"10.1016/j.autrev.2025.103812","DOIUrl":"10.1016/j.autrev.2025.103812","url":null,"abstract":"<div><h3>Objectives</h3><div>Juvenile Localized Scleroderma (JLS) is an autoimmune disease leading to fibrosis of skin and subcutaneous tissues affecting children, that is characterized by extracutaneous manifestations (ECM) in about 20 % of patients. JLS and ECM can cause severe disabilities, potentially impacting patients' quality of life (QoL). We aimed to systematically review studies reporting ECM in young patients with JLS.</div></div><div><h3>Methods</h3><div>Pubmed, Cochrane and Scopus databases were approached to identify studies evaluating ECM in children with LS. Selected papers focusing on QoL and multidisciplinary approach were separately analysed.</div></div><div><h3>Results</h3><div>At the end of the selection process, 15 papers (encompassing 3604 children) focused on the description of ECM were included. Overall, ECM were reported in 958/3604 (26.5 %) children, and the 3 most frequent ones were musculoskeletal (24 %), neurological (10.3 %) and odontostomatological (7.6 %). Six papers (435 patients) focusing on QoL in children with JLS resulted comparable. Three studies focusing on the role of a multidisciplinary team in the management of children and adolescents with JLS and ECM were also selected (216 children).</div></div><div><h3>Conclusions</h3><div>Almost one-third of patients with JLS may present several clinical problems other than skin lesions that should be managed by a multidisciplinary team. However, evidence on the efficacy of a multispecialty management is still lacking. The impact of ECM on QoL of these patients may be underestimated, as no specifically developed assessment tool has been applied so far, but recently proposed overall disease severity and disease-specific patient-reported outcome measures may improve the evaluation of this important clinical aspect.</div></div>","PeriodicalId":8664,"journal":{"name":"Autoimmunity reviews","volume":"24 7","pages":"Article 103812"},"PeriodicalIF":9.2,"publicationDate":"2025-04-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143794621","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Lupus nephritis trials network (LNTN) repeat kidney biopsy-based definitions of treatment response: A systematic literature review-based proposal","authors":"Ioannis Parodis ,&nbsp;Nursen Cetrez ,&nbsp;Leonardo Palazzo ,&nbsp;Valeria Alberton ,&nbsp;Hans-Joachim Anders ,&nbsp;Ingeborg M. Bajema ,&nbsp;Nathalie Costedoat-Chalumeau ,&nbsp;Ana Malvar ,&nbsp;Brad H. Rovin ,&nbsp;Jorge Sanchez-Guerrero ,&nbsp;Ming-Hui Zhao ,&nbsp;Julia Weinmann-Menke ,&nbsp;Maria G. Tektonidou ,&nbsp;Frédéric A. Houssiau","doi":"10.1016/j.autrev.2025.103810","DOIUrl":"10.1016/j.autrev.2025.103810","url":null,"abstract":"<div><div>Within the frame of the Lupus Nephritis Trials Network (LNTN), we conducted a systematic literature review (SLR) to propose kidney tissue-based definitions of treatment outcomes in lupus nephritis (LN). Given the limitations of clinical markers like proteinuria in predicting immunological, histological, and long-term outcomes, our work emphasises the importance of repeat kidney biopsies. Such biopsies help identify discordance between clinical and histological response, which has implications for long-term kidney outcomes. The research objectives of this SLR focused on defining repeat biopsy-based treatment response and histological remission, and their associations with long-term outcomes. The SLR reviewed studies published from 2000 to 2022, identifying 20 eligible works. Histological response was commonly defined by changes in the National Institutes of Health (NIH) Activity Index (AI), with response indicated by a decrease of ≥50 % and to ≤3. Remission was most commonly defined as an AI score of 0. These benchmarks were associated with improved long-term renal outcomes, such as reduced flare rates and preserved kidney function. Conversely, NIH AI scores ≥4 and NIH Chronicity Index (CI) scores ≥4 were associated with poor prognosis, highlighting their predictive utility. Consensus definitions were established through expert panel deliberation, setting a foundation for standardising LN treatment evaluation in clinical trials and observational studies. These definitions are not intended for routine clinical decisions but aim to enhance uniformity and comparability in research, especially when repeat kidney biopsies are performed, an approach strongly advocated by our work. Further validation through ongoing initiatives and molecular characterisation efforts will refine these criteria, fostering advances in LN management and patient outcomes.</div></div>","PeriodicalId":8664,"journal":{"name":"Autoimmunity reviews","volume":"24 7","pages":"Article 103810"},"PeriodicalIF":9.2,"publicationDate":"2025-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143768634","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}