Journal of autoimmunity最新文献

{"title":"Impact of coding risk variant IFNGR2 on the B cell-intrinsic IFN-γ signaling pathway in multiple sclerosis","authors":"Laurens Bogers ,&nbsp;Jasper Rip ,&nbsp;Liza Rijvers ,&nbsp;Jamie van Langelaar ,&nbsp;Steven C. Koetzier ,&nbsp;Kirsten L. Kuiper ,&nbsp;Veronique Meerdink ,&nbsp;Annet F. Wierenga-Wolf ,&nbsp;Marie-José Melief ,&nbsp;Ana M. Marques ,&nbsp;Joost Smolders ,&nbsp;Marvin M. van Luijn","doi":"10.1016/j.jaut.2024.103279","DOIUrl":"10.1016/j.jaut.2024.103279","url":null,"abstract":"<div><p>B cells of people with multiple sclerosis (MS) are more responsive to IFN-γ, corresponding to their brain-homing potential. We studied how a coding single nucleotide polymorphism (SNP) in <em>IFNGR2</em> (rs9808753) co-operates with Epstein-Barr virus (EBV) infection as MS risk factors to affect the IFN-γ signaling pathway in human B cells. In both cell lines and primary cells, EBV infection positively associated with IFN-γ receptor expression and STAT1 phosphorylation. The <em>IFNGR2</em> risk SNP selectively promoted downstream signaling via STAT1, particularly in transitional B cells. Altogether, EBV and the <em>IFNGR2</em> risk SNP independently amplify IFN-γ signaling, potentially driving B cells to enter the MS brain.</p></div>","PeriodicalId":15245,"journal":{"name":"Journal of autoimmunity","volume":"148 ","pages":"Article 103279"},"PeriodicalIF":7.9,"publicationDate":"2024-07-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S0896841124001136/pdfft?md5=af7d3ba7a970baa5d8f848df49c5c4dd&pid=1-s2.0-S0896841124001136-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141554893","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}

{"title":"Pro-fibrotic effect of the susceptible gene PCSK5 in vascular fibrosis of Takayasu arteritis via TGF-β and SMAD3 signaling pathway activation","authors":"Jinghua Wang ,&nbsp;Ying Sun ,&nbsp;Rongyi Chen ,&nbsp;Dan Meng ,&nbsp;Yuanyuan Wei ,&nbsp;Lindi Jiang ,&nbsp;Xiufang Kong","doi":"10.1016/j.jaut.2024.103277","DOIUrl":"10.1016/j.jaut.2024.103277","url":null,"abstract":"<div><h3>Background</h3><p>Vascular fibrosis directly causes vascular thickening in Takayasu arteritis (TAK), in which sustained transforming growth factor beta (TGF-β) activation is critical. Understanding TGF-β activation regulation and blocking it might yield a therapeutic effect in TAK. Proprotein convertase subtilisin/kexin type 5 (PCSK5) rs6560480 (T/C) is associated with TAK development. In this study, we assessed the association between the <em>PCSK5</em> rs6560480 genotype and PCSK5 expression in TAK and explored its molecular role in TGF-β activation and vascular fibrosis development.</p></div><div><h3>Methods</h3><p>In TAK patients, PCSK5 and TGF-β expression in plasma and aortic tissue was examined by ELISA and immunohistochemical staining, and <em>PCSK5</em> rs6560480 was genotyped. The correlation between PCSK5 and extracellular matrix (ECM) expression was examined by Western blotting (WB) and immunohistochemistry staining. Detection by co-immunoprecipitation was performed to detect the interaction between PCSK5 and TGF-β in adventitial fibroblasts (AAFs). Downstream signaling pathways were detected by WB and validated with appropriate inhibitors. Potential immunosuppressive agents to inhibit the effects of PCSK5 were explored in cell culture and TAK patients.</p></div><div><h3>Results</h3><p>Patients with <em>PCSK5</em> rs6560480 TT patients had significantly higher PCSK5 levels and more thickened vascular lesions than patients with <em>PCSK5</em> rs6560480 CT. PCSK5 expression was significantly increased in alpha smooth muscle actin (α-SMA)-positive myofibroblasts in TAK vascular lesions. Overexpressing PCSK5 facilitated TGF-β and downstream SMAD2/3 activation and ECM expression in AAFs and aorta in in-vitro culture. The mechanistic study supported that PCSK5 activated precursor TGF-β (pro-TGF-β) to the mature form by binding the pro-TGF-β cleavage site. Leflunomide inhibited PCSK5 and pro-TGF-β binding, decreasing TGF-β activation and ECM expression, which was also partially validated in leflunomide-treated patients.</p></div><div><h3>Conclusion</h3><p>The findings revealed a novel pro-fibrotic mechanism of PCSK5 in TAK vascular fibrosis via TGF-β and downstream SMAD2/3 pathway activation. Leflunomide might be anti-fibrotic by disrupting PCSK5 and pro-TGF-β binding, presenting a new TAK treatment approach.</p></div>","PeriodicalId":15245,"journal":{"name":"Journal of autoimmunity","volume":"148 ","pages":"Article 103277"},"PeriodicalIF":7.9,"publicationDate":"2024-07-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141554894","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":"VNN2-expressing circulating monocytes exhibit unique functional characteristics and are decreased in patients with primary Sjögren's syndrome","authors":"Ayibaota Bahabayi ,&nbsp;Xiayidan Alimu ,&nbsp;Guochong Wang ,&nbsp;Yiming Gao ,&nbsp;Yang Chen ,&nbsp;Junjie Zhao ,&nbsp;Xinran Lian ,&nbsp;Qi Li ,&nbsp;Ziqi Xiong ,&nbsp;Zhonghui Zhang ,&nbsp;Pingzhang Wang ,&nbsp;Chen Liu","doi":"10.1016/j.jaut.2024.103275","DOIUrl":"10.1016/j.jaut.2024.103275","url":null,"abstract":"<div><h3>Objective</h3><p>This study aims to elucidate the significance of VNN2 expression in peripheral blood monocytes and its clinical relevance in primary Sjögren's syndrome (pSS).</p></div><div><h3>Methods</h3><p>We investigated VNN2 expression by analyzing single-cell RNA sequencing (scRNA-seq) data from peripheral blood mononuclear cells. Flow cytometry was used to detect and compare VNN2 expression in total monocytes, classical monocytes (cMo), intermediate monocytes (iMo) and non-classical monocytes (ncMo). Additionally, we examined the expression of HLA, ICAM1, CD62L, ITGAM, S100A8, S100A9, CCR2, CCR6, CX3CR1 and CXCR3 in VNN2⁺ and VNN2^- cells. We analyzed the correlation between VNN2 expression and clinical indicators and assessed the clinical utility of VNN2⁺ monocytes in pSS diagnosis using receiver operating characteristic curves.</p></div><div><h3>Results</h3><p>We observed high VNN2 expression in monocytes, with significantly higher levels in CD14⁺⁺ monocytes compared to ncMo. VNN2⁺ monocytes exhibited decreased expression of HLA and CD62L and increased expression of ICAM1, ITGAM, S100A8, S100A9, CCR2, CCR6, CX3CR1 and CXCR3 compared to VNN2^- monocytes. Although scRNA-seq data showed that VNN2 mRNA was upregulated, cell surface expression of VNN2 was decreased in monocytes from pSS patients compared to healthy controls. The reduced levels of VNN2⁺ monocyte subpopulations in pSS patients were negatively correlated with anti-ribosome antibody levels and positively correlated with complement 4 levels. Detection of VNN2 expression in monocytes can aid in the auxiliary diagnosis of pSS.</p></div><div><h3>Conclusion</h3><p>Monocytes expressing cell surface VNN2 are significantly reduced in pSS patients. This suggests a potential role for VNN2 in pSS development and its potential use as a diagnostic marker for pSS.</p></div>","PeriodicalId":15245,"journal":{"name":"Journal of autoimmunity","volume":"147 ","pages":"Article 103275"},"PeriodicalIF":7.9,"publicationDate":"2024-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141468275","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":"Soluble form of immune checkpoints in autoimmune diseases","authors":"Li Yuan ,&nbsp;Yuxia Wang ,&nbsp;Xuxia Shen ,&nbsp;Fujun Ma ,&nbsp;Jun Wang ,&nbsp;Fang Yan","doi":"10.1016/j.jaut.2024.103278","DOIUrl":"10.1016/j.jaut.2024.103278","url":null,"abstract":"<div><p>Immune checkpoints are essential regulators of immune responses, either by activating or suppressing them. Consequently, they are regarded as pivotal elements in the management of infections, cancer, and autoimmune disorders. In recent years, researchers have identified numerous soluble immune checkpoints that are produced through various mechanisms and demonstrated biological activity. These soluble immune checkpoints can be produced and distributed in the bloodstream and various tissues, with their roles in immune response dysregulation and autoimmunity extensively documented. This review aims to provide a thorough overview of the generation of various soluble immune checkpoints, such as sPD-1, sCTLA-4, sTim-3, s4-1BB, sBTLA, sLAG-3, sCD200, and the B7 family, and their importance as indicators for the diagnosis and prediction of autoimmune conditions. Furthermore, the review will investigate the potential pathological mechanisms of soluble immune checkpoints in autoimmune diseases, emphasizing their association with autoimmune diseases development, prognosis, and treatment.</p></div>","PeriodicalId":15245,"journal":{"name":"Journal of autoimmunity","volume":"147 ","pages":"Article 103278"},"PeriodicalIF":7.9,"publicationDate":"2024-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141468274","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":"Cellular pathways and molecular events that shape autoantibody production in COVID-19","authors":"Gregory J. Tsay ,&nbsp;Moncef Zouali","doi":"10.1016/j.jaut.2024.103276","DOIUrl":"10.1016/j.jaut.2024.103276","url":null,"abstract":"<div><p>A hallmark of COVID-19 is the variety of complications that follow SARS-CoV-2 infection in some patients, and that target multiple organs and tissues. Also remarkable are the associations with several auto-inflammatory disorders and the presence of autoantibodies directed to a vast array of antigens. The processes underlying autoantibody production in COVID-19 have not been completed deciphered. Here, we review mechanisms involved in autoantibody production in COVID-19, multisystem inflammatory syndrome in children, and post-acute sequelae of COVID19. We critically discuss how genomic integrity, loss of B cell tolerance to self, superantigen effects of the virus, and extrafollicular B cell activation could underly autoantibody proaction in COVID-19. We also offer models that may account for the pathogenic roles of autoantibodies in the promotion of inflammatory cascades, thromboembolic phenomena, and endothelial and vascular deregulations.</p></div>","PeriodicalId":15245,"journal":{"name":"Journal of autoimmunity","volume":"147 ","pages":"Article 103276"},"PeriodicalIF":7.9,"publicationDate":"2024-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141468272","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":"Reconstituted CD74+ NK cells trigger chronic graft versus host disease after allogeneic bone marrow transplantation","authors":"Yingchao Dou ,&nbsp;Zhigang Nian ,&nbsp;Dongyao Wang ,&nbsp;Guangyu Sun ,&nbsp;Li Zhou ,&nbsp;Ziming Hu ,&nbsp;Jieqi Ke ,&nbsp;Xiaoyu Zhu ,&nbsp;Rui Sun ,&nbsp;Zhigang Tian ,&nbsp;Binqing Fu ,&nbsp;Yonggang Zhou ,&nbsp;Haiming Wei","doi":"10.1016/j.jaut.2024.103274","DOIUrl":"10.1016/j.jaut.2024.103274","url":null,"abstract":"<div><p>Chronic graft-versus-host disease (cGVHD) is the most common long-term complication after allogeneic hematopoietic stem cell transplantation (allo-HSCT). The patients with pulmonary cGVHD in particular have a very poor prognosis. NK cells are the first reconstituted lymphocyte subset after allo-HSCT; however, the impact of reconstituted NK cells on cGVHD is unclear. Here, we found allogeneic recipients showed obvious pulmonary cGVHD. Surprisingly, deletion of reconstituted NK cells resulted in maximal relief of pulmonary cGVHD. Mechanistically, reconstituted NK cells with donor profiles modulated the pulmonary inflammatory microenvironment to trigger cGVHD. Reconstituted NK cells secreted IFN-γ and TNF-α to induce CXCL10 production by epithelial cells, which recruited macrophages and CD4⁺ T cells to the lungs. Then macrophages and CD4⁺ T cells were activated by the inflammatory microenvironment, thereby mediating lung injury. Through assessment of differences in cellular energy, we found that CD74⁺ NK cells with high mitochondrial potential and pro-inflammatory activity triggered pulmonary cGVHD. Furthermore, targeted elimination of CD74⁺ NK cells using the anti-CD74 antibody significantly alleviated pulmonary cGVHD but preserved the CD74⁻ NK cells to exert graft-versus-leukemia (GVL) effects. Data from human samples corroborated our findings in mouse models. Collectively, our results reveal that reconstituted CD74⁺ NK cells trigger pulmonary cGVHD and suggest that administration of CD74 antibody was a potential therapeutic for patients with cGVHD.</p></div>","PeriodicalId":15245,"journal":{"name":"Journal of autoimmunity","volume":"147 ","pages":"Article 103274"},"PeriodicalIF":7.9,"publicationDate":"2024-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S0896841124001082/pdfft?md5=2e392b4331bc2156fe4a990a2503a2b4&pid=1-s2.0-S0896841124001082-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141468273","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}

{"title":"Inflammation dynamically regulates steroid hormone metabolism and action within macrophages in rheumatoid arthritis","authors":"C.S Martin ,&nbsp;A. Crastin ,&nbsp;M.S. Sagmeister ,&nbsp;M.S. Kalirai ,&nbsp;J.D. Turner ,&nbsp;L. MacDonald ,&nbsp;M. Kurowska-Stolarska ,&nbsp;D. Scheel-Toellner ,&nbsp;A.E. Taylor ,&nbsp;L.C. Gilligan ,&nbsp;K. Storbeck ,&nbsp;M. Price ,&nbsp;C.M. Gorvin ,&nbsp;Filer A ,&nbsp;R. Mahida ,&nbsp;A.R. Clark ,&nbsp;S.W. Jones ,&nbsp;K. Raza ,&nbsp;M. Hewison ,&nbsp;R.S. Hardy","doi":"10.1016/j.jaut.2024.103263","DOIUrl":"https://doi.org/10.1016/j.jaut.2024.103263","url":null,"abstract":"<div><h3>Rationale</h3><p>In inflammatory diseases such as rheumatoid arthritis (RA), steroid metabolism is a central component mediating the actions of immuno-modulatory glucocorticoids and sex steroids. However, the regulation and function of cellular steroid metabolism within key leukocyte populations such as macrophages remain poorly defined. In this study, the inflammatory regulation of global steroid metabolism was assessed in RA macrophages.</p></div><div><h3>Methods</h3><p>Bulk RNA-seq data from RA synovial macrophages was used to assess transcripts encoding key enzymes in steroid metabolism and signalling. Changes in metabolism were assessed in synovial fluids, correlated to measures of disease activity and functionally validated in primary macrophage cultures.</p></div><div><h3>Results</h3><p>RNA-seq revealed a unique pattern of differentially expressed genes, including changes in genes encoding the enzymes 11β-HSD1, SRD5A1, AKR1C2 and AKR1C3. These correlated with disease activity, favouring increased glucocorticoid and androgen levels. Synovial fluid 11β-HSD1 activity correlated with local inflammatory mediators (TNFα, IL-6, IL-17), whilst 11β-HSD1, SRD5A1 and AKR1C3 activity correlated with systemic measures of disease and patient pain (ESR, DAS28 ESR, global disease activity). Changes in enzyme activity were evident in inflammatory activated macrophages <em>in vitro</em> and revealed a novel androgen activating role for 11β-HSD1. Together, increased glucocorticoids and androgens were able to suppress inflammation in macrophages and fibroblast-like-synoviocytes.</p></div><div><h3>Conclusions</h3><p>This study underscores the significant increase in androgen and glucocorticoid activation within inflammatory polarized macrophages of the synovium, contributing to local suppression of inflammation. The diminished profile of inactive steroid precursors in postmenopausal women may contribute to disturbances in this process, leading to increased disease incidence and severity.</p></div>","PeriodicalId":15245,"journal":{"name":"Journal of autoimmunity","volume":"147 ","pages":"Article 103263"},"PeriodicalIF":12.8,"publicationDate":"2024-06-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S0896841124000970/pdfft?md5=fbf43a91254aa6b252f689124460d92d&pid=1-s2.0-S0896841124000970-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141292204","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}

{"title":"Regulatory T cells effectively downregulate the autoimmune anti-MPO response and ameliorate anti-MPO induced glomerulonephritis in mice","authors":"Peiqi Hu ,&nbsp;Hong Xiao ,&nbsp;Sandra Elmore ,&nbsp;Christian Agosto-Burgos ,&nbsp;Yichun Hu ,&nbsp;Susan L. Hogan ,&nbsp;Dominic J. Ciavatta ,&nbsp;Ronald J. Falk ,&nbsp;J. Charles Jennette ,&nbsp;Meghan E. Free","doi":"10.1016/j.jaut.2024.103266","DOIUrl":"https://doi.org/10.1016/j.jaut.2024.103266","url":null,"abstract":"<div><p>Regulation of autoreactive cells is key for both prevention and amelioration of autoimmune disease. A better understanding of the key cell population(s) responsible for downregulation of autoreactive cells would provide necessary foundational insight for cellular-based therapies in autoimmune disease. Utilizing a mouse model of anti-myeloperoxidase (MPO) glomerulonephritis, we sought to understand which immune cells contribute to downregulation of the anti-MPO autoimmune response. MPO−/− mice were immunized with whole MPO to induce an anti-MPO response. Anti-MPO splenocytes were then transferred into recipient mice (Rag2−/− mice or WT mice). Anti-MPO titers were followed over time. After anti-MPO splenocyte transfer, WT mice are able to downregulate the anti-MPO response while anti-MPO titers persist in Rag2−/− recipients. Reconstitution with WT splenocytes into Rag2−/− recipients prior to anti-MPO splenocyte transfer enabled mice to downregulate the anti-MPO immune response. Therefore, wildtype splenocytes contain a cellular population that is capable of downregulating the autoimmune response. Through splenocyte transfer, antibody depletion experiments, and purified cell population transfers, we confirmed that the regulatory T cell (Treg) population is responsible for the downregulation of the anti-MPO autoimmune response. Further investigation revealed that functional Tregs from WT mice are capable of downregulating anti-MPO antibody production and ameliorate anti-MPO induced glomerulonephritis. These data underscore the importance of functional Tregs for control of autoimmune responses and prevention of end-organ damage due to autoimmunity.</p></div>","PeriodicalId":15245,"journal":{"name":"Journal of autoimmunity","volume":"147 ","pages":"Article 103266"},"PeriodicalIF":12.8,"publicationDate":"2024-06-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S0896841124001008/pdfft?md5=6ac2e44873cca339cd969e68c0a0d9ee&pid=1-s2.0-S0896841124001008-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141292203","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}

{"title":"Prevalence and clinical profiles of primary sclerosing cholangitis in China: Data from electronic medical records and systematic literature retrieval","authors":"Xiaoqian Xu ,&nbsp;Tongtong Meng ,&nbsp;Lichen Shi ,&nbsp;Weijia Duan ,&nbsp;Junqi Niu ,&nbsp;Huiguo Ding ,&nbsp;Wen Xie ,&nbsp;Lu Zhou ,&nbsp;Bangmao Wang ,&nbsp;Jie Li ,&nbsp;Lingyi Zhang ,&nbsp;Yu Wang ,&nbsp;Xiaojuan Ou ,&nbsp;Xinyan Zhao ,&nbsp;Hong You ,&nbsp;Jidong Jia ,&nbsp;Yuanyuan Kong","doi":"10.1016/j.jaut.2024.103264","DOIUrl":"https://doi.org/10.1016/j.jaut.2024.103264","url":null,"abstract":"<div><h3>Background &amp; aims</h3><p>Epidemiology of primary sclerosing cholangitis (PSC) is lacking in China. We aimed to estimate the period prevalence and depict the clinical features of PSC in China.</p></div><div><h3>Methods</h3><p>We identified and included PSC cases between 2000 and 2023 from two sources: electronic medical records (EMR) and systematical literature retrieval (SLR). The period prevalence of PSC was estimated by the multiplier method. Rate ratios (RRs) for PSC prevalence in relation to macroeconomic indicators were calculated by the negative binomial regression model.</p></div><div><h3>Results</h3><p>A total of 1358 PSC cases were retrieved from 299 hospitals (162 from EMR and 1196 from SLR). Males accounted for 55.7 % of the PSC cases and 25.7 % had concomitant inflammatory bowel disease (IBD). The estimated period prevalence of PSC from 2000 to 2023 was 2.36 (95 % CI: 1.82, 3.34) per 100,000. Males had a numerically higher PSC prevalence than females (2.56, 95 % CI: 1.97, 3.63 vs. 2.14, 95 % CI: 1.65, 3.04 per 100,000). The highest prevalence of PSC was in East China at 4.87 (95 % CI: 3.44, 7.18) per 100,000, followed by North China at 2.94 (95 % CI: 2.33, 3.74) per 100,000, and the lowest in South China at 0.92 (95 % CI: 0.66, 1.30) per 100,000. Regional per capita GDP (RR 1.65, 95 % CI: 1.03, 2.65) and healthcare expenditure (RR 1.94, 95 % CI: 1.13, 3.38) were identified to be associated with PSC prevalence.</p></div><div><h3>Conclusion</h3><p>Our study showed the estimated PSC prevalence varied within China, but was generally lower than that in Western countries.</p></div>","PeriodicalId":15245,"journal":{"name":"Journal of autoimmunity","volume":"147 ","pages":"Article 103264"},"PeriodicalIF":12.8,"publicationDate":"2024-06-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141249669","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 HyperPed-COVID international registry: Impact of age of onset, disease presentation and geographical distribution on the final outcome of MIS-C","authors":"Roberta Caorsi ,&nbsp;Alessandro Consolaro ,&nbsp;Camilla Speziani ,&nbsp;Betul Sozeri ,&nbsp;Kadir Ulu ,&nbsp;Enrique Faugier-Fuentes ,&nbsp;Hector Menchaca-Aguayo ,&nbsp;Seza Ozen ,&nbsp;Seher Sener ,&nbsp;Shahana Akhter Rahman ,&nbsp;Mohammad Imnul Islam ,&nbsp;Filomeen Haerynck ,&nbsp;Gabriele Simonini ,&nbsp;Mariel Viviana Mastri ,&nbsp;Tadej Avcin ,&nbsp;Saša Sršen ,&nbsp;Taciana de Albuquerque Pedrosa Fernandes ,&nbsp;Valda Stanevicha ,&nbsp;Jelena Vojinovic ,&nbsp;Ali Sobh ,&nbsp;Svitlana Samsonenko","doi":"10.1016/j.jaut.2024.103265","DOIUrl":"https://doi.org/10.1016/j.jaut.2024.103265","url":null,"abstract":"<div><h3>Objectives</h3><p>The aim of the study was to establish an international multicenter registry to collect data on patients with Multisystem Inflammatory Syndrome in Children (MIS-C), in order to highlight a relationship between clinical presentation, age of onset and geographical distribution on the clinical outcome.</p></div><div><h3>Study design</h3><p>Multicenter retrospective study involving different international societies for rare immunological disorders.1009 patients diagnosed with MIS-C between March and September 2022, from 48 centers and 22 countries were collected. Five age groups (&lt;1, 1–4, 5–11, 12–16, &gt;16 years) and four geographic macro-areas, Western Europe, Central-Eastern Europe, Latin America, Asian-African resource-limited countries (LRC), were identified.</p></div><div><h3>Results</h3><p>Time to referral was significantly higher in LRC. Intensive anti-inflammatory treatment, including biologics, respiratory support and mechanic ventilation were more frequently used in older children and in European countries. The mortality rate was higher in very young children (&lt;1 year), in older patients (&gt;16 years of age) and in LRC. Multivariate analysis identified the residence in LRC, presence of severe cardiac involvement, renal hypertension, lymphopenia and non-use of heparin prophylaxis, as the factors most strongly associated with unfavorable outcomes.</p></div><div><h3>Conclusions</h3><p>The stratification of patients by age and geographic macro-area provided insights into the clinical presentation, treatment and outcome of MIS-C. The mortality and sequelae rates exhibited a correlation with the age and geographical areas. Patients admitted and treated in LRC displayed more severe outcomes, possibly due to delays in hospital admission and limited access to biologic drugs and to intensive care facilities.</p></div>","PeriodicalId":15245,"journal":{"name":"Journal of autoimmunity","volume":"147 ","pages":"Article 103265"},"PeriodicalIF":12.8,"publicationDate":"2024-06-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141242866","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}