Clinical and experimental immunology最新文献_第4页

Intrinsic functional defects in B cells of patients with NFKB2 mutations. NFKB2 基因突变患者 B 细胞的内在功能缺陷。

IF 3.4 3区医学

Clinical and experimental immunology Pub Date : 2024-10-15 DOI: 10.1093/cei/uxae090

Qing Min, Yaxuan Li, Xuzhe Wu, Meiping Yu, Wenjing Ying, Qinhua Zhou, Jia Hou, Bijun Sun, Xiaoying Hui, Lulu Dong, Xin Meng, Hai Zhang, Ziying Hu, Xiaoqian Feng, Jinqiao Sun, Wenjie Wang, Xiaochuan Wang, Ji-Yang Wang

{"title":"Intrinsic functional defects in B cells of patients with NFKB2 mutations.","authors":"Qing Min, Yaxuan Li, Xuzhe Wu, Meiping Yu, Wenjing Ying, Qinhua Zhou, Jia Hou, Bijun Sun, Xiaoying Hui, Lulu Dong, Xin Meng, Hai Zhang, Ziying Hu, Xiaoqian Feng, Jinqiao Sun, Wenjie Wang, Xiaochuan Wang, Ji-Yang Wang","doi":"10.1093/cei/uxae090","DOIUrl":"https://doi.org/10.1093/cei/uxae090","url":null,"abstract":"Mutations in the human nuclear factor-κB2 gene (NFKB2) are associated with common variable immunodeficiency (CVID) or combined immunodeficiency diseases (CID), characterized by B-cell lymphopenia, hypogammaglobulinemia, and T cell dysfunction. This study investigated whether B cells with NFKB2 mutations exhibit intrinsic impairments in activation, class-switch recombination, and differentiation. We analyzed five patients from four unrelated families with CVID, each carrying a heterozygous NFKB2 mutation: P1 (C.2595_2614del, p.A867Gfs*12), P2 (C.2597G>A, p.S866N), P3 (C.2540dupT, p.R848Efs*38), and P4 and P5 (C.2570_2571insCAGCACA, p.A860Qfs*28). The patients with frameshift mutations (P1, P3, P4, and P5) exhibited truncated proteins detectable in their peripheral blood mononuclear cells, while P2 had a missense mutation. All identified mutations disrupted the processing of p100 into the active p52 form, resulting in NF-κB2 loss-of-function and IκBδ gain-of-function. Clinically, P1, P2, and P3 exhibited B-cell lymphopenia, and all five patients presented with hypogammaglobulinemia. Notably, P2 exhibited a markedly low B-cell count, associated with increased proportions of memory B and IgD-CD27- double negative B cells. In vitro experiments with naïve B cells from P1 and P4 demonstrated decreased survival, impaired activation, and reduced differentiation into CD27+IgD- cells and plasmablasts, while class switch recombination was unaffected. These findings reveal novel B cell-intrinsic functional defects in patients with NFKB2 mutations.","PeriodicalId":10268,"journal":{"name":"Clinical and experimental immunology","volume":null,"pages":null},"PeriodicalIF":3.4,"publicationDate":"2024-10-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142459409","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

Pneumococcal Serotype-Specific Antibodies in Children with Recurrent Oto-sinopulmonary Infections. 复发性耳鼻咽喉感染儿童的肺炎球菌血清型特异性抗体。

IF 3.4 3区医学

Clinical and experimental immunology Pub Date : 2024-10-10 DOI: 10.1093/cei/uxae086

Hanadys Ale, Jose G Calderon, Joshua Gruber, Thomas Taylor, William R Blouin, Vivian P Hernández Trujillo

{"title":"Pneumococcal Serotype-Specific Antibodies in Children with Recurrent Oto-sinopulmonary Infections.","authors":"Hanadys Ale, Jose G Calderon, Joshua Gruber, Thomas Taylor, William R Blouin, Vivian P Hernández Trujillo","doi":"10.1093/cei/uxae086","DOIUrl":"https://doi.org/10.1093/cei/uxae086","url":null,"abstract":"Low titers to pneumococcal vaccine are a frequent finding in pediatric patients with recurrent oto-sinopulmonary infections. To characterize the pre- and post-immunization antibody trend for each serotype included in the pneumococcal 13-valent conjugate vaccine (PCV13), in a cohort of pediatric patients with recurrent oto-sinopulmonary infections. This retrospective review identified 182 patients with recurrent oto-sinopulmonary infections (131 required an immune workup and 99 had low pneumococcal titers leading to a PCV13 vaccine booster). Baseline pneumococcal serotype-specific antibody titers at initial visit and 6 weeks after the vaccine booster were obtained. An adequate response to the pneumococcal conjugate vaccine was deemed to be a 4-fold increase over baseline and/or a post-immunization titer of 1.3 µg/ml or greater. Overall, PCV13 booster provided a significant improvement in the number of protective titers, increasing from 3.6 serotypes at baseline to 11.1 serotypes at 6 weeks (p < 0.001). This increase correlated with improved clinical outcomes (81% showed no signs of recurrent infection after the first booster and 94% after a second booster). Post-immunization antibody concentrations were significantly higher than at baseline for all serotypes (p< 0.05) and only 8, 9N, and 12F did not exhibit a greater than 4-fold increase (p> 0.05) 6 weeks following booster. There were no differences between patients at different ages in post-immunization titer levels for all serotypes. In pediatric patients with recurrent oto-sinopulmonary infections, an additional pneumococcal booster proved to be effective in the protection of these children against further infections, across all age groups.","PeriodicalId":10268,"journal":{"name":"Clinical and experimental immunology","volume":null,"pages":null},"PeriodicalIF":3.4,"publicationDate":"2024-10-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142388481","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

sB7-H4 is a diagnostic biomarker in epithelial ovarian cancer and correlates to platinum resistance. sB7-H4 是上皮性卵巢癌的诊断生物标志物，与铂类抗药性相关。

IF 3.4 3区医学

Clinical and experimental immunology Pub Date : 2024-10-07 DOI: 10.1093/cei/uxae084

Ying Zhou, Jingluan Tian, Yu Shen, Hansi Liang, Youguo Chen, Juan Wang, Yanzheng Gu

{"title":"sB7-H4 is a diagnostic biomarker in epithelial ovarian cancer and correlates to platinum resistance.","authors":"Ying Zhou, Jingluan Tian, Yu Shen, Hansi Liang, Youguo Chen, Juan Wang, Yanzheng Gu","doi":"10.1093/cei/uxae084","DOIUrl":"https://doi.org/10.1093/cei/uxae084","url":null,"abstract":"Ovarian cancer, with its high mortality rate among gynecological cancers, is often diagnosed late due to the lack of early diagnostic symptoms and biomarkers. The tumor immune microenvironment has become a focal point in cancer diagnostic and therapeutic research. Among these, B7-H4, a checkpoint protein, plays a crucial role in immune regulation and tumor suppression, contributing to immune evasion within the tumor microenvironment. This study aims to identify the concentration of soluble B7-H4(sB7-H4) in the plasma of patients with ovarian cancer and to evaluate its clinical significance. Through a comprehensive analysis involving enzyme-linked immunosorbent assay, immunohistochemistry, and multicolor immunofluorescence, we quantified sB7-H4 levels in patient plasma and ascites, correlating these findings with tissue expression and clinical outcomes. Results indicated a strong association between high sB7-H4 levels and advanced disease, surgical outcomes, lymphatic metastasis, and platinum resistance. When compared with traditional biomarkers CA125 and HE4, sB7-H4, especially in conjunction with these markers, enhances the diagnostic accuracy for epithelial ovarian cancer, offering insights into disease progression and therapeutic efficacy. This comprehensive analysis suggests that sB7-H4 is a promising biomarker for EOC, providing valuable insights into diagnosis, stage differentiation, treatment effectiveness, and prognosis.","PeriodicalId":10268,"journal":{"name":"Clinical and experimental immunology","volume":null,"pages":null},"PeriodicalIF":3.4,"publicationDate":"2024-10-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142380130","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

Investigating Concomitant RAG-2 and LRBA Mutations in SCID and Autoimmunity. 研究SCID和自身免疫中同时存在的RAG-2和LRBA突变

IF 3.4 3区医学

Clinical and experimental immunology Pub Date : 2024-10-03 DOI: 10.1093/cei/uxae083

Ilia Spivak, Shirly Frizinsky, Amarilla Mandola, Atar Lev, Amos J Simon, Ortal Barel, Vicktoria Vishnevskia-Dai, Raz Somech, Ido Somekh

{"title":"Investigating Concomitant RAG-2 and LRBA Mutations in SCID and Autoimmunity.","authors":"Ilia Spivak, Shirly Frizinsky, Amarilla Mandola, Atar Lev, Amos J Simon, Ortal Barel, Vicktoria Vishnevskia-Dai, Raz Somech, Ido Somekh","doi":"10.1093/cei/uxae083","DOIUrl":"https://doi.org/10.1093/cei/uxae083","url":null,"abstract":"Inborn errors of immunity (IEI) are a large heterogenous group of diseases characterized by immunodeficiency, immune dysregulation, allergy, auto-inflammation and predisposition for malignancies. Most are inherited in an autosomal recessive trait. We studied a patient with severe combined immunodeficiency (SCID) and immune dysregulation who harbored two distinct bi-allelic IEI-associated genetic mutations. Clinical, immunological and genetic data were collected. Genetic investigation included whole exome sequencing on DNA extracted from skin fibroblasts. Family segregation was performed by Sanger sequencing. Immunological evaluation included absolute and functional evaluation of lymphocytes and chimerism analysis post hematopoietic stem cell transplantation (HSCT). Treg subsets, LRBA and CTLA4 expression levels were measured by flow-cytometric analysis. A nineteen-year-old female patient from a consanguine background underwent unconditioned matched sibling related HSCT during infancy due to clinical presentation of SCID with an Omenn phenotype. At that time her underlying genetic defect was not defined. Years after HSCT, severe auto-immune phenomena were noted, including a systemic lupus erythematosus-like syndrome and ophthalmic manifestations. Genetic evaluation revealed bi-allelic homozygous mutations in RAG-2 (c.685C>T, p.Arg229Trp) and a previously undescribed mutation in LRBA (c.3325G>T, p.Asp1109Tyr). LRBA and CTLA4 expression levels were normal, suggesting that the LRBA variant identified in these kindred is unlikely to be pathogenic. Multiple genetic defects causing complex IEIs may be identified in the same individual in highly consanguineous populations. Functional immunological testing is essential for evaluation of novel genetic variants.","PeriodicalId":10268,"journal":{"name":"Clinical and experimental immunology","volume":null,"pages":null},"PeriodicalIF":3.4,"publicationDate":"2024-10-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142364630","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

Investigating immune profile by CyTOF in patients with eosinophilic esophagitis after treatment with orodispersible budesonide. 通过 CyTOF 研究嗜酸性粒细胞食管炎患者在使用口崩布地奈德治疗后的免疫状况。

IF 3.4 3区医学

Clinical and experimental immunology Pub Date : 2024-09-16 DOI: 10.1093/cei/uxae065

John Plate, Sofie Albinsson Högberg, Hardis Rabe, Helen Larsson, Christine Lingblom

{"title":"Investigating immune profile by CyTOF in patients with eosinophilic esophagitis after treatment with orodispersible budesonide.","authors":"John Plate, Sofie Albinsson Högberg, Hardis Rabe, Helen Larsson, Christine Lingblom","doi":"10.1093/cei/uxae065","DOIUrl":"10.1093/cei/uxae065","url":null,"abstract":"Eosinophilic esophagitis (EoE) is a chronic Th2-mediated inflammatory disease of the esophagus driven by dietary or inhalant allergens which if left untreated, leads to fibrosis and poor esophageal function. Although the inflammation in the esophagus is dominated by eosinophils, there are also elevated levels of T and B cells. Blood samples from ten patients with EoE before and after treatment with orodispersible budesonide and 10 healthy controls were compared using cytometry by time-of-flight. An antibody panel was designed that covers the major immunological cell populations with a particular focus on eosinophils. The data was analyzed with multivariate methods and cluster analysis. Correlation analysis was done between immune markers and endoscopic, histological, and symptomatologic assessments. Our analysis revealed that patients with EoE had lower levels of effector memory T cells after treatment with orodispersible budesonide to the same level as healthy subjects. In addition, more suppressive eosinophils were present in the circulation of EoE patients before treatment and more immature eosinophils were present after treatment. Furthermore, levels of galectin-10+ eosinophils correlated with histological findings in esophageal tissue from EoE patients. In all patients, the peak eosinophils were decreased after treatment with orodispersible budesonide. Intriguingly, 90% of the patients had remission in the histological assessment and 50% improved in the endoscopic assessment. This study reports a detailed immune profile in patients with EoE before and after treatment with orodispersible budesonide and it is a step toward finding blood-based immune parameters that could be useful to monitor response to treatment.","PeriodicalId":10268,"journal":{"name":"Clinical and experimental immunology","volume":null,"pages":null},"PeriodicalIF":3.4,"publicationDate":"2024-09-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11404122/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141733681","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

Expanding organoid complexity to advance immunology research. 扩大类器官的复杂性，推进免疫学研究。

IF 3.4 3区医学

Clinical and experimental immunology Pub Date : 2024-09-16 DOI: 10.1093/cei/uxae067

Joana F Neves

引用次数: 0

Inhibition of the TIM-1 and -3 signaling pathway ameliorates disease in a murine model of rheumatoid arthritis. 抑制 TIM-1 和 -3 信号通路可改善类风湿性关节炎小鼠模型的病情

IF 3.4 3区医学

Clinical and experimental immunology Pub Date : 2024-09-16 DOI: 10.1093/cei/uxae056

Yuji Nozaki, Hisaya Akiba, Hiroki Akazawa, Hirotaka Yamazawa, Kaori Ishimura, Koji Kinoshita, Itaru Matsumura

{"title":"Inhibition of the TIM-1 and -3 signaling pathway ameliorates disease in a murine model of rheumatoid arthritis.","authors":"Yuji Nozaki, Hisaya Akiba, Hiroki Akazawa, Hirotaka Yamazawa, Kaori Ishimura, Koji Kinoshita, Itaru Matsumura","doi":"10.1093/cei/uxae056","DOIUrl":"10.1093/cei/uxae056","url":null,"abstract":"Members of the T-cell immunoglobulin and mucin (TIM) family, which is crucial for T-cell function, are implicated in autoimmunity. TIM-1 and -3 play distinct roles in autoimmunity, with TIM-1 acting as a costimulatory molecule and TIM-3 regulating Th1 responses. We investigated the therapeutic potential of anti-TIM-1 (RMT1-10) and anti-TIM-3 (RMT3-23) antibodies in an autoimmune arthritis model. Zymosan A was used to induce arthritis in female SKG mice. The arthritis scores, histology, mRNA expression, cytokine levels, micro-computed tomography, and flow cytometry results were obtained. The application of RMT1-10 reduced the arthritis scores, histological damage, and CD4+ T-cell infiltrations, and it suppressed interleukin (IL)-6 and -17A and reduced TIM-3 mRNA expressions. RMT3-23 also lowered arthritis severity, improved histology, and reduced serum levels of tumor necrosis factor (TNF)-α and IL-17A. RMT3-23 inhibited intracellular TNF-α and IL-6 and early apoptosis. An amelioration of autoimmune arthritis was achieved by blocking the TIM-1 and -3 signaling pathways via RMT1-10 and RMT3-23 administration, leading to a widespread decrease in inflammatory cytokines. Both antibodies exhibited therapeutic effects, suggesting TIM-1 and -3 as potential targets for rheumatoid arthritis.","PeriodicalId":10268,"journal":{"name":"Clinical and experimental immunology","volume":null,"pages":null},"PeriodicalIF":3.4,"publicationDate":"2024-09-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11404125/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141554237","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

Revolutionizing immune research with organoid-based co-culture and chip systems. 利用类器官共培养和芯片系统革新免疫研究。

IF 3.4 3区医学

Clinical and experimental immunology Pub Date : 2024-09-16 DOI: 10.1093/cei/uxae004

Diana Papp, Tamas Korcsmaros, Isabelle Hautefort

{"title":"Revolutionizing immune research with organoid-based co-culture and chip systems.","authors":"Diana Papp, Tamas Korcsmaros, Isabelle Hautefort","doi":"10.1093/cei/uxae004","DOIUrl":"10.1093/cei/uxae004","url":null,"abstract":"The intertwined interactions various immune cells have with epithelial cells in our body require sophisticated experimental approaches to be studied. Due to the limitations of immortalized cell lines and animal models, there is an increasing demand for human in vitro model systems to investigate the microenvironment of immune cells in normal and in pathological conditions. Organoids, which are self-renewing, 3D cellular structures that are derived from stem cells, have started to provide gap-filling tissue modelling solutions. In this review, we first demonstrate with some of the available examples how organoid-based immune cell co-culture experiments can advance disease modelling of cancer, inflammatory bowel disease, and tissue regeneration. Then, we argue that to achieve both complexity and scale, organ-on-chip models combined with cutting-edge microfluidics-based technologies can provide more precise manipulation and readouts. Finally, we discuss how genome editing techniques and the use of patient-derived organoids and immune cells can improve disease modelling and facilitate precision medicine. To achieve maximum impact and efficiency, these efforts should be supported by novel infrastructures such as organoid biobanks, organoid facilities, as well as drug screening and host-microbe interaction testing platforms. All these together or in combination can allow researchers to shed more detailed, and often patient-specific, light on the crosstalk between immune cells and epithelial cells in health and disease.","PeriodicalId":10268,"journal":{"name":"Clinical and experimental immunology","volume":null,"pages":null},"PeriodicalIF":3.4,"publicationDate":"2024-09-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11404127/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139570016","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

Organoids as a tool to study homeostatic and pathological immune-epithelial interactions in the gut. 将器官组织作为研究肠道内免疫-上皮相互作用的稳态和病态的工具。

IF 3.4 3区医学

Clinical and experimental immunology Pub Date : 2024-09-16 DOI: 10.1093/cei/uxad118

Emma Højmose Kromann, Ainize Peña Cearra, Joana F Neves

{"title":"Organoids as a tool to study homeostatic and pathological immune-epithelial interactions in the gut.","authors":"Emma Højmose Kromann, Ainize Peña Cearra, Joana F Neves","doi":"10.1093/cei/uxad118","DOIUrl":"10.1093/cei/uxad118","url":null,"abstract":"The intestine hosts the largest immune cell compartment in the body as a result of its continuous exposure to exogenous antigens. The intestinal barrier is formed by a single layer of epithelial cells which separate immune cells from the gut lumen. Bidirectional interactions between the epithelium and the immune compartment are critical for maintaining intestinal homeostasis by limiting infection, preventing excessive immune activation, and promoting tissue repair processes. However, our understanding of epithelial-immune interactions incomplete as the complexity of in vivo models can hinder mechanistic studies, cell culture models lack the cellular heterogeneity of the intestine and when established from primary cell can be difficult to maintain. In the last decade, organoids have emerged as a reliable model of the intestine, recapitulating key cellular and architectural features of native tissues. Herein, we provide an overview of how intestinal organoids are being co-cultured with immune cells leading to substantial advances in our understanding of immune-epithelial interactions in the gut. This has enabled new discoveries of the immune contribution to epithelial maintenance and regeneration both in homeostasis and in disease such as chronic inflammation, infection and cancer. Organoids can additionally be used to generate immune cells with a tissue-specific phenotype and to investigate the impact of disease associated risk genes on the intestinal immune environment. Accordingly, this review demonstrates the multitude of applications for intestinal organoids in immunological research and their potential for translational approaches.","PeriodicalId":10268,"journal":{"name":"Clinical and experimental immunology","volume":null,"pages":null},"PeriodicalIF":3.4,"publicationDate":"2024-09-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11404120/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140326470","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

Exploring S100A8/A9, neopterin, and MMP3 in familial Mediterranean fever. 探索家族性地中海热中的 S100A8/A9、Neopterin 和 MMP3。

IF 3.4 3区医学

Clinical and experimental immunology Pub Date : 2024-09-16 DOI: 10.1093/cei/uxae049

Ozgur C Kilinc, Yonca S Akdeniz, Zuleyha Taskin, Mehmet Karabulut, Arif Kaya, Ibrahim Murat Bolayırlı, Gunay Can, Serdal Ugurlu

{"title":"Exploring S100A8/A9, neopterin, and MMP3 in familial Mediterranean fever.","authors":"Ozgur C Kilinc, Yonca S Akdeniz, Zuleyha Taskin, Mehmet Karabulut, Arif Kaya, Ibrahim Murat Bolayırlı, Gunay Can, Serdal Ugurlu","doi":"10.1093/cei/uxae049","DOIUrl":"10.1093/cei/uxae049","url":null,"abstract":"Familial Mediterranean fever (FMF) is characterized by inflammatory attacks due to overactivation of pyrin inflammasome. This study aimed to investigate the reliability of S100A8/A9, neopterin, and matrix metalloproteinase 3 (MMP3) at monitoring subclinical inflammation and disease activity, and at differentiating FMF attacks from appendicitis, the most common misdiagnosis among FMF patients. Blood samples (n = 75), comprising from FMF patients during an attack (n = 20), the same FMF patients during the attack-free period (n = 14), patients with appendicitis (n = 24), and healthy volunteers (n = 17) were obtained. Duplicate determinations of S100A8/A9, neopterin, and MMP-3 levels were conducted using the enzyme-linked immunosorbent assay (ELISA). FMF patients with and without attack and patients with appendicitis had significantly elevated S100A8/A9 levels compared to healthy volunteers (P-values: < 0.001, 0.036, 0.002, respectively). Patients with appendicitis and FMF patients with and without attack had significantly increased serum neopterin levels compared to healthy volunteers (P-value: < 0.001). MMP3 levels were significantly higher among patients with appendicitis and FMF patients during attack compared to healthy controls (P-values: < 0.001, 0.001). Serum levels of S100A8/A9, neopterin, and MMP3 were increased significantly during attacks compared to attack-free periods among FMF patients (P-values: 0.03, 0.047, 0.007). S100A8/A9 emerges as a valuable marker for monitoring disease activity. Neopterin and S100A8/A9 might help physicians to monitor subclinical inflammation during the attack-free periods of FMF patients. MMP3 might aid in diagnosing FMF attacks when distinguishing between attack and attack-free periods is challenging.","PeriodicalId":10268,"journal":{"name":"Clinical and experimental immunology","volume":null,"pages":null},"PeriodicalIF":3.4,"publicationDate":"2024-09-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11404116/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141305576","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0