Autoimmunity reviews最新文献

{"title":"STING-inflammasome axis in autoimmune diseases and inflammation-related disease","authors":"Shengjun Chai ,&nbsp;Haiming Xu ,&nbsp;Ruixin Liu ,&nbsp;Chunmei Cai","doi":"10.1016/j.autrev.2025.103898","DOIUrl":"10.1016/j.autrev.2025.103898","url":null,"abstract":"<div><div>The STING-inflammasome axis predominantly functions to transmit signals generated from cGAS-STING-mediated cytosolic DNA recognition, thereby inducing and potentiating inflammasome activation and amplifying immune responses. However, under specific circumstances, the inflammasome can reciprocally modulate STING activity. This dynamic relationship comprises a canonical upstream-downstream activation cascade complemented by bidirectional interactions and feedback loops within the intricate immune regulatory network. Accumulating evidence underscores the pivotal involvement of the STING-inflammasome axis in diverse host defense mechanisms and inflammatory disorders, including autoimmune diseases, sterile inflammatory conditions, carcinogenesis, and neurodegenerative diseases. In this comprehensive review, we systematically summarize the current understanding of the contributions and underlying mechanisms of the STING-inflammasome axis in physiological and pathological processes. Additionally, we conduct an in-depth exploration of the translational potential of targeting this axis for preventive and therapeutic interventions, offering novel insights into future research directions and clinical applications.</div></div>","PeriodicalId":8664,"journal":{"name":"Autoimmunity reviews","volume":"24 11","pages":"Article 103898"},"PeriodicalIF":8.3,"publicationDate":"2025-08-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144768337","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":"Cognitive impairment in systemic autoimmune and inflammatory diseases: A narrative review focused on ANCA-associated vasculitis, sarcoidosis, Sjögren's syndrome, systemic sclerosis, and Behçet's disease","authors":"Marion Camard ,&nbsp;Fanny Urbain ,&nbsp;Nicolas Noel","doi":"10.1016/j.autrev.2025.103899","DOIUrl":"10.1016/j.autrev.2025.103899","url":null,"abstract":"<div><div>Cognitive impairment is an increasingly recognized feature of systemic autoimmune and inflammatory diseases, yet remains underdiagnosed and insufficiently studied beyond systemic lupus erythematosus. In this narrative review, we explore the prevalence, clinical profiles, and pathophysiological mechanisms of cognitive dysfunction in five systemic diseases: ANCA-associated vasculitis (AAV), primary Sjögren's syndrome (pSS), systemic sclerosis (SSc), sarcoidosis, and Behçet's disease (BD). We conducted a structured literature search in PubMed and Embase to identify relevant studies published between 1954 and 2024. Reported prevalence varies widely: ∼30 % in AAV, 0–35 % in sarcoidosis, 10–80 % in pSS, 8–65 % in SSc, and 30–100 % in BD. Executive dysfunction and attention deficits predominate. In some cases—particularly in AAV and BD—cognitive decline may reflect direct central nervous system involvement, but many patients report cognitive complaints without overt neurological manifestations or imaging abnormalities. Proposed mechanisms include blood-brain barrier disruption, cytokine-driven neuroinflammation (notably IL-6, TNF-α, IFN-γ, BAFF), autoantibody-mediated synaptic toxicity, and cerebral hypoperfusion linked to small-vessel vasculopathy. Glial activation and neuroimmune dysregulation are recurring findings in animal models and functional imaging studies. White matter lesions and abnormal brain perfusion on MRI or SPECT are frequently observed in asymptomatic patients. Comorbid symptoms such as depression, fatigue, pain, and small fiber neuropathy may exacerbate or mimic cognitive dysfunction. Despite its prevalence and impact on quality of life, cognitive dysfunction is rarely screened, partly due to a lack of standardized tools. Harmonized neurocognitive assessment protocols and longitudinal studies are urgently needed to improve understanding, detection, and management of cognitive impairment, and support its integration into routine care.</div></div>","PeriodicalId":8664,"journal":{"name":"Autoimmunity reviews","volume":"24 11","pages":"Article 103899"},"PeriodicalIF":8.3,"publicationDate":"2025-08-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144793367","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":"Points to consider in the management of ANCA-associated vasculitis","authors":"Marina Papadopoulou,&nbsp;Anastasios Karamanakos","doi":"10.1016/j.autrev.2025.103894","DOIUrl":"10.1016/j.autrev.2025.103894","url":null,"abstract":"<div><div>Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is characterized by inflammation and necrosis of small to medium blood vessels that frequently present with organ- or life-threatening manifestations. Although significant therapeutic advances have improved outcomes, many areas in the management of AAV remain controversial or insufficiently defined. Key areas of debate include the choice of induction therapy—particularly the potential benefits of combining rituximab and cyclophosphamide—and the role of adjunctive therapies such as avacopan and plasma exchange. Additional challenges involve determining the optimal duration and dosing of maintenance therapy, strategies for relapse prediction and prevention, appropriate glucocorticoid tapering, the use of fixed versus biomarker-guided maintenance regimens, and the potential for withholding maintenance therapy in select patient populations. Despite multiple high-quality randomized controlled trials and international guidelines, clinical practice remains heterogeneous. The emergence of novel therapies is promising in helping to address these gaps and may provide effective treatment options for patients with resistant or refractory disease. This review summarizes current controversies and challenges in the treatment of AAV and provides a practical, evidence-based framework to support clinical decision-making.</div></div>","PeriodicalId":8664,"journal":{"name":"Autoimmunity reviews","volume":"24 11","pages":"Article 103894"},"PeriodicalIF":8.3,"publicationDate":"2025-08-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144774599","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":"Removal notice to “Relapsing polychondritis: A clinical update” [Autoimmunity Reviews 15_6 (2016) 539–543]","authors":"Lucia Longo ,&nbsp;Antonio Greco ,&nbsp;Andrea Rea ,&nbsp;Vincenza Rita Lo Vasco ,&nbsp;Armando De Virgilio ,&nbsp;Marco De Vincentiis","doi":"10.1016/j.autrev.2025.103890","DOIUrl":"10.1016/j.autrev.2025.103890","url":null,"abstract":"","PeriodicalId":8664,"journal":{"name":"Autoimmunity reviews","volume":"24 9","pages":"Article 103890"},"PeriodicalIF":8.3,"publicationDate":"2025-08-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144772575","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":"Neutrophil extracellular traps and neuropsychiatric lupus: Signaling pathways involved in NET formation and mechanisms affecting the central nervous system","authors":"Xin Guan,&nbsp;Xiaojie He,&nbsp;Lingjuan Liu","doi":"10.1016/j.autrev.2025.103897","DOIUrl":"10.1016/j.autrev.2025.103897","url":null,"abstract":"<div><div>Neuropsychiatric systemic lupus erythematosus (NPSLE) is a severe complication of systemic lupus erythematosus (SLE) that affects the central or peripheral nervous system. The clinical management of NPSLE faces significant challenges due to the absence of specific diagnostic biomarkers and the lack of standardized, evidence-based diagnostic and therapeutic protocols. Therefore, a comprehensive understanding of the pathogenesis of NPSLE and the exploration of novel therapeutic targets have become urgent priorities in current research. Recent studies have demonstrated that neutrophils and their hallmark product, neutrophil extracellular traps (NETs), play a central role in various pathological processes of NPSLE, with blood-brain barrier (BBB) disruption and the subsequent inflammatory cascade being particularly critical. This paper provides a systematic review of the molecular regulatory network of neutrophils in NPSLE, focusing on the mechanisms by which neutrophils mediate neuroimmune microenvironmental disorders through BBB damage, with the goal of offering a theoretical foundation for the precise diagnosis and treatment of this disease.</div></div>","PeriodicalId":8664,"journal":{"name":"Autoimmunity reviews","volume":"24 11","pages":"Article 103897"},"PeriodicalIF":8.3,"publicationDate":"2025-07-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144768336","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":"Monoclonal Gammopathy of Rheumatologic Significance (MGRhS): a systemic vision of clonal disorders with multiple organ involvement","authors":"Luca Quartuccio ,&nbsp;Valeria Manfrè ,&nbsp;Elena Treppo ,&nbsp;Fabio Perrotta ,&nbsp;Gaafar Ragab ,&nbsp;Andreas Goules ,&nbsp;Ennio Lubrano","doi":"10.1016/j.autrev.2025.103895","DOIUrl":"10.1016/j.autrev.2025.103895","url":null,"abstract":"<div><div>Monoclonal gammopathy (MG) encompasses a spectrum of conditions ranging from benign to malignant clonal B-cell proliferations. While MG is traditionally associated with hematologic malignancies, its role in autoimmune and rheumatologic diseases is increasingly recognized. This review proposes the novel concept of <em>“monoclonal gammopathy of rheumatologic significance (MGRhS)”</em> that refers to a non-malignant or pre-malignant systemic condition related to a monoclonal immunoglobulin and clonal B cells, capable of producing multi-organ damage or influencing the therapeutic management of rheumatologic diseases.</div><div>MG of clinical significance is characterized by the production of monoclonal proteins causing organ damage and modulating immune responses. These proteins contribute to rheumatologic conditions or peculiar phenotypes, including cryoglobulinemic vasculitis, Sjögren's disease, and other autoimmune disorders. The review explores pathogenic mechanisms linking MG with these diseases, emphasizing early detection and accurate classification to guide therapeutic strategies.</div><div>The manuscript addresses the implications of incidental MG detection in rheumatologic patients, particularly in the context of biologic therapies. Practical guidance is provided on identifying MGRhS, assessing its impact, and tailoring management to prevent complications.</div><div>This analysis aims to advance understanding of MGRhS as a distinct clinical entity, encouraging a multidisciplinary approach to its management. The integration of novel diagnostic tools and targeted therapies is essential to improve outcomes and address the complexity of this condition.</div></div>","PeriodicalId":8664,"journal":{"name":"Autoimmunity reviews","volume":"24 11","pages":"Article 103895"},"PeriodicalIF":8.3,"publicationDate":"2025-07-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144759028","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":"Specialized pro-resolving mediators and autoimmunity: Recent insights and future perspectives","authors":"Marta Vomero ,&nbsp;Ludovica Lamberti ,&nbsp;Erika Corberi ,&nbsp;Damiano Currado ,&nbsp;Annalisa Marino ,&nbsp;Onorina Berardicurti ,&nbsp;Marina Fava ,&nbsp;Alessandro Leuti ,&nbsp;Mauro Maccarrone ,&nbsp;Roberto Giacomelli ,&nbsp;Luca Navarini","doi":"10.1016/j.autrev.2025.103896","DOIUrl":"10.1016/j.autrev.2025.103896","url":null,"abstract":"<div><div>Inflammation is a response to injuries involving multiple cellular and molecular mechanisms. Different stimuli, such as trauma or microbial invasion, trigger an acute inflammatory response consisting, at least in principle, of two phases: initiation and resolution. Although the acute phase of inflammatory response represents a protective and usually self-limited mechanism, it can sometimes persist and evolve into chronic inflammation, a key driver in the development of many rheumatic and autoimmune diseases. The biosynthesis of specialized pro-resolving mediators (SPMs) orchestrates the resolution phase of inflammation, leading to the shutdown of phlogosis, tissue damage repair, and restoration of homeostasis. Dysregulation in SPMs biosynthesis or receptor expression and signalling are related to impaired viruses' clearance and adaptive immune response. Emerging knowledge on the involvement of SPMs in the development and progression of rheumatic diseases is arising. An altered SPM profile has been observed in different rheumatic diseases, including Rheumatoid Arthritis, Systemic Lupus Erythematosus, Adult-onset Still's Disease, Systemic Sclerosis, and Sjögren's Syndrome. Considering the anti-inflammatory and pro-resolving roles of these molecules, the possible use of synthetic SPMs and fish oil supplements, a crucial source of SPMs precursors DHA, DPA and EPA, in patients affected by chronic inflammatory diseases, is emerging.</div></div>","PeriodicalId":8664,"journal":{"name":"Autoimmunity reviews","volume":"24 11","pages":"Article 103896"},"PeriodicalIF":8.3,"publicationDate":"2025-07-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144759029","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":"Intersecting paths between autoimmunity and bone marrow fibrosis: the case of autoimmune myelofibrosis.","authors":"Francesco Carubbi ,&nbsp;Alessia Alunno ,&nbsp;Elisa Matone ,&nbsp;Alessandro Lucchesi ,&nbsp;Gerardo Musuraca ,&nbsp;Claudio Ferri","doi":"10.1016/j.autrev.2025.103893","DOIUrl":"10.1016/j.autrev.2025.103893","url":null,"abstract":"<div><div>Bone marrow fibrosis is frequently observed in patients with haematological malignancies including myeloproliferative neoplasm (MPNs). The most common cause of BMF is the BCR-ABL-negative MPN primary myelofibrosis (PMF) however, BMF may also be caused by a variety of non-neoplastic disorders such as infections, endocrine diseases and autoimmune diseases (ADs). Autoimmune myelofibrosis (AIMF) is a type of BMF associated with either a fully blown AD (secondary AIMF) or serological signs of autoimmunity without an overt AD (primary AIMF). Although isolated case reports and case series have been published, AIMF remains a poorly recognized disorder. Therefore, we performed a scoping review and critically appraised the literature on AIMF pathogenesis, diagnosis and treatment with a particular focus on similarities and differences between AIMF and PMF.</div></div>","PeriodicalId":8664,"journal":{"name":"Autoimmunity reviews","volume":"24 11","pages":"Article 103893"},"PeriodicalIF":8.3,"publicationDate":"2025-07-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144739565","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":"Understanding the effects of Janus kinase inhibitors on the cardiovascular system in comparison to main biological DMARDs in rheumatoid arthritis","authors":"Aliki Zavoriti,&nbsp;Pierre Miossec","doi":"10.1016/j.autrev.2025.103892","DOIUrl":"10.1016/j.autrev.2025.103892","url":null,"abstract":"<div><div>Rheumatoid arthritis (RA) is linked to increased cardiovascular (CV) risk, as is typical for systemic inflammation. The systemic effects of inflammatory cytokines induce vascular dysfunction, leading to thrombosis and other CV diseases. Reducing inflammation should attenuate this risk. For several decades, biologics against a single cytokine, such as TNF inhibitors and IL-6 receptor blockers seem to support this, demonstrating excellent control of RA and reduction of CV events. The Janus kinase inhibitors (JAKi) have been approved more recently. By inhibiting the JAK-STAT pathway, they can simultaneously block the function of multiple cytokines. Unlike biologics, JAKi are associated with increased risk of adverse CV events in high-risk RA patients. This review suggests biological mechanisms that could explain the worse CV outcomes with JAKi compared to biologics. Among the key pro-inflammatory cytokines, IFNγ, IL-6 and IL-23 use directly the JAK-STAT pathway, whereas IL-17, TNF and IL-1β do not, lacking JAK-related receptors. Nevertheless, these non-JAK-dependent cytokines can still influence the JAK-STAT pathway to promote vascular effects in an indirect fashion. Moreover, IL-17 and TNF specifically when combined, exert major pro-coagulant and pro-thrombotic effects on vessels independently of JAKs. As a result, JAKi might not block these pathways and even upregulate them, at high concentrations, leading to increased thrombotic risk. Finally, new research shows that JAKi cannot prevent the increase in adhesion and coagulation molecules as well as the deficiency in physiological anti-coagulant proteins triggered by TNF and IL-17 on endothelial cells. Increased efforts to control CV risk factors are critical in this context.</div></div>","PeriodicalId":8664,"journal":{"name":"Autoimmunity reviews","volume":"24 11","pages":"Article 103892"},"PeriodicalIF":8.3,"publicationDate":"2025-07-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144726966","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":"Primordial and primary prevention in rheumatological diseases: The time has come","authors":"Laura Scagnellato ,&nbsp;Mariangela Salvato ,&nbsp;Luca Iorio,&nbsp;Beatrice Moccaldi,&nbsp;Alessandro Giollo,&nbsp;Elisabetta Zanatta,&nbsp;Roberto Padoan,&nbsp;Roberta Ramonda,&nbsp;Andrea Doria","doi":"10.1016/j.autrev.2025.103891","DOIUrl":"10.1016/j.autrev.2025.103891","url":null,"abstract":"<div><div>Rheumatological, immune-mediated diseases (RDs) impose a substantial global burden, especially on women of working age, resulting in chronic disability and escalating healthcare utilisation. Despite therapeutic advances, the incidence of RDs is rising, and no definitive cure exists. Emerging evidence highlights the potential of early-stage interventions, such as addressing preclinical phases of diseases to delay or prevent disease onset. Modifiable risk factors, such as tobacco use, obesity, diet, infections, mechanical strain, and other environmental exposures (e.g., air pollution and ultraviolet radiation), offer clear targets for intervention. Despite increasing efforts in producing high-quality studies for each rheumatological condition, current evidence supports the global promotion of a healthy lifestyle with a balanced diet, regular physical activity and vaccinations, vitamin D supplementation, and minimisation of excessive infectious, mechanical and psychosocial strain. However, to stimulate stakeholders and policymakers in investing and developing strategies for primary prevention, further interventional trials are warranted on at-risk populations such as first-degree relatives of rheumatological patients and asymptomatic autoantibody carriers. Indeed, besides encouraging experiments in rheumatoid arthritis and psoriatic arthritis, where research has reached the adolescent stage, research in disease interception is still in its infancy for most rheumatological conditions.</div></div>","PeriodicalId":8664,"journal":{"name":"Autoimmunity reviews","volume":"24 9","pages":"Article 103891"},"PeriodicalIF":8.3,"publicationDate":"2025-07-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144722863","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}