Journal of Allergy and Clinical Immunology-In Practice最新文献

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Allergic Lymphangitis after Hymenoptera Sting. 膜翅目昆虫蜇伤后过敏性淋巴管炎。
IF 6.6 1区 医学
Journal of Allergy and Clinical Immunology-In Practice Pub Date : 2025-09-26 DOI: 10.1016/j.jaip.2025.08.030
Jyotsna Mullur
{"title":"Allergic Lymphangitis after Hymenoptera Sting.","authors":"Jyotsna Mullur","doi":"10.1016/j.jaip.2025.08.030","DOIUrl":"https://doi.org/10.1016/j.jaip.2025.08.030","url":null,"abstract":"","PeriodicalId":51323,"journal":{"name":"Journal of Allergy and Clinical Immunology-In Practice","volume":" ","pages":""},"PeriodicalIF":6.6,"publicationDate":"2025-09-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145150601","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}
引用次数: 0
Efficacy, Safety, and Quality-of-Life Outcomes of Remibrutinib in Chronic Spontaneous Urticaria: A Systematic Review and Meta-Analysis. 瑞米鲁替尼治疗慢性自发性荨麻疹的疗效、安全性和生活质量:系统回顾和荟萃分析。
IF 6.6 1区 医学
Journal of Allergy and Clinical Immunology-In Practice Pub Date : 2025-09-24 DOI: 10.1016/j.jaip.2025.09.023
Ahmed Ali Khan, Abdul Ahad Riaz, Faisal Naseer, Noor Fatima, Zuhair Abrar, Linta Malik, Jumana Khan, Raza Aslam, Ahmed Abdul Rab, Allahdad Khan
{"title":"Efficacy, Safety, and Quality-of-Life Outcomes of Remibrutinib in Chronic Spontaneous Urticaria: A Systematic Review and Meta-Analysis.","authors":"Ahmed Ali Khan, Abdul Ahad Riaz, Faisal Naseer, Noor Fatima, Zuhair Abrar, Linta Malik, Jumana Khan, Raza Aslam, Ahmed Abdul Rab, Allahdad Khan","doi":"10.1016/j.jaip.2025.09.023","DOIUrl":"https://doi.org/10.1016/j.jaip.2025.09.023","url":null,"abstract":"<p><strong>Background: </strong>Chronic spontaneous urticaria (CSU) is a mast cell-mediated condition affecting ∼1% of the population and is often refractory to antihistamines and omalizumab. Remibrutinib, a Bruton's tyrosine kinase (BTK) inhibitor, prevents mast cell activation independent of the IgE pathway.</p><p><strong>Objective: </strong>To assess the efficacy, safety, and quality-of-life outcomes (QoL) of remibrutinib compared to placebo in adults with refractory CSU.</p><p><strong>Methods: </strong>A systematic review was conducted per PRISMA guidelines. Three RCTs (n=997) and two single-arm studies (n=280) evaluating remibrutinib in CSU were included. Specific disease activity endpoints assessed included changes in Urticaria Activity Score (UAS7), Hives Severity Score (HSS7), Itch Severity Score (ISS7), Angioedema Activity Score (AAS7), and Dermatology Life Quality Index (DLQI). The risk of bias was evaluated using the Cochrane RoB-2 tool for RCTs and ROBINS-I for single-arm studies. Meta-analysis was performed using a random-effects model.</p><p><strong>Results: </strong>In the pooled analysis of RCTs, remibrutinib effectively decreased UAS7 at Week 12 compared to placebo (MD -7.81, 95% CI: -10.29 to -5.33), with improvements in itch and hives severity scores (MD -2.94, 95% CI: -3.73 to -2.15, and MD -4.05, 95% CI: -4.98 to -3.12, respectively). Remibrutinib increased the likelihood of achieving complete response (UAS7=0; RR 3.32, 95% CI: 2.34 to 4.71), controlled disease (UAS7≤6; RR 2.13, 95% CI: 1.73 to 2.62), and minimal quality-of-life impact (DLQI ≤ 1; RR 1.84, 95% CI: 1.47 to 2.30). REMIX-1 and REMIX-2 trials showed significantly better disease control (UAS7≤6) by week 2 (RR 6.81, 95% CI: 3.45 to 13.42). Adverse event rates with remibrutinib were similar to placebo, except for increased nasopharyngitis, upper respiratory tract infection, and petechiae (RR 1.88, 95% CI: 1.11 to 3.19; RR 2.88, 95% CI: 1.30 to 6.41; and RR=7.52, 95% Cl: 1.44 to 39.20, respectively) Evidence from single-arm studies (BISCUIT at 24 weeks and Jain 2024 at 52 weeks) suggested sustained long-term efficacy and tolerability.</p><p><strong>Conclusion: </strong>Remibrutinib shows rapid symptom improvement with an acceptable safety profile in refractory CSU and appears to be a promising oral option for antihistamine-refractory CSU based on short-term data.</p>","PeriodicalId":51323,"journal":{"name":"Journal of Allergy and Clinical Immunology-In Practice","volume":" ","pages":""},"PeriodicalIF":6.6,"publicationDate":"2025-09-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145180343","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}
引用次数: 0
Tolerability of Penicillins and Cephalosporins in Patients with Confirmed Cefazolin Immediate Hypersensitivity. 确诊头孢唑林立即过敏患者青霉素和头孢菌素的耐受性。
IF 6.6 1区 医学
Journal of Allergy and Clinical Immunology-In Practice Pub Date : 2025-09-24 DOI: 10.1016/j.jaip.2025.09.017
Joseph F De Luca, Sara Vogrin MBiostat, Suran L Fernando, Morgan T Rose, Sarah L Green, Jason A Trubiano, Jamma Li
{"title":"Tolerability of Penicillins and Cephalosporins in Patients with Confirmed Cefazolin Immediate Hypersensitivity.","authors":"Joseph F De Luca, Sara Vogrin MBiostat, Suran L Fernando, Morgan T Rose, Sarah L Green, Jason A Trubiano, Jamma Li","doi":"10.1016/j.jaip.2025.09.017","DOIUrl":"https://doi.org/10.1016/j.jaip.2025.09.017","url":null,"abstract":"","PeriodicalId":51323,"journal":{"name":"Journal of Allergy and Clinical Immunology-In Practice","volume":" ","pages":""},"PeriodicalIF":6.6,"publicationDate":"2025-09-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145180356","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}
引用次数: 0
Leukocytosis, monocytosis, and eosinophilia in systemic mastocytosis: analysis of phenotype, genetics and prognosis in 596 patients from the GREM registry. 系统性肥大细胞增多症中的白细胞增多症、单核细胞增多症和嗜酸性粒细胞增多症:来自GREM登记的596例患者的表型、遗传学和预后分析。
IF 6.6 1区 医学
Journal of Allergy and Clinical Immunology-In Practice Pub Date : 2025-09-24 DOI: 10.1016/j.jaip.2025.09.018
Johannes Lübke, Nicole Naumann, Vito Dangelo, Alice Fabarius, Georgia Metzgeroth, Hans-Peter Horny, Karl Sotlar, Wolf-Karsten Hofmann, Martina Rudelius, Juliana Schwaab, Andreas Reiter
{"title":"Leukocytosis, monocytosis, and eosinophilia in systemic mastocytosis: analysis of phenotype, genetics and prognosis in 596 patients from the GREM registry.","authors":"Johannes Lübke, Nicole Naumann, Vito Dangelo, Alice Fabarius, Georgia Metzgeroth, Hans-Peter Horny, Karl Sotlar, Wolf-Karsten Hofmann, Martina Rudelius, Juliana Schwaab, Andreas Reiter","doi":"10.1016/j.jaip.2025.09.018","DOIUrl":"https://doi.org/10.1016/j.jaip.2025.09.018","url":null,"abstract":"<p><strong>Background: </strong>Leukocytosis, monocytosis, and eosinophilia (L/M/E) are recurrent findings in systemic mastocytosis (SM).</p><p><strong>Objective: </strong>To investigate the prevalence of L/M/E in SM and assess their association with clinical phenotype, mutational profile, and overall survival (OS) in advanced SM (AdvSM).</p><p><strong>Methods: </strong>Within the German Registry on Disorders of Eosinophilia and Mast Cells (GREM), 596 SM patients (91% KIT D816V positive; 270 AdvSM, 326 non-AdvSM) were analyzed for L/M/E.</p><p><strong>Results: </strong>In comparison to non-AdvSM, AdvSM patients had significantly higher leukocyte (median 9.4 vs. 6.9×10<sup>9</sup>/L), monocyte (median 0.7 vs. 0.5×10<sup>9</sup>/L), and eosinophil counts (median 0.3 vs. 0.1×10<sup>9</sup>/L; all P<0.001) with highest counts (leukocytes: 10.2×10<sup>9</sup>/L, monocytes: 0.9×10<sup>9</sup>/L, eosinophils: 0.3×10<sup>9</sup>/L; all P<0.001) being observed in SM with associated hematologic neoplasm (SM-AHN). High counts of L/M/E correlated with an increased number of additional somatic mutations (P=0.012, P<0.001, P=0.020) with monocytosis being specially associated with mutations in ASXL1 (odds ratio [OR] 2.91; 95% CI: 1.5-5.8), SRSF2 (OR 2.2; 95% CI: 1.2-4.0), and TET2 (OR 2.2; 95% CI: 1.2-4.0). In AdvSM, optimal OS cut-off values based on maximally selected rank statistics were ≥16.8×10<sup>9</sup>/L for leukocytosis (median OS: 1.6 vs. 4.7 years, P<0.001), ≥1.1×10<sup>9</sup>/L for monocytosis (2.9 vs. 4.8 years, P<0.001), and ≥1.5×10<sup>9</sup>/L for eosinophilia (1.7 vs. 5.0 years, P<0.001). Monocytosis and/or eosinophilia defined a three-tiered risk model (median OS: 1.60 vs. 3.04 vs. 6.94 years, P<0.001).</p><p><strong>Conclusions: </strong>Elevated counts of L/M/E are indicative of AdvSM and within AdvSM associated with additional somatic mutations, a subtype of SM-AHN and poor prognosis.</p>","PeriodicalId":51323,"journal":{"name":"Journal of Allergy and Clinical Immunology-In Practice","volume":" ","pages":""},"PeriodicalIF":6.6,"publicationDate":"2025-09-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145180328","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}
引用次数: 0
Hepatic Manifestations in Common Variable Immunodeficiency Associated with Mortality and Worse Hospital Outcomes in Nationwide Analysis using National Readmission Database. 在使用国家再入院数据库的全国分析中,常见可变免疫缺陷的肝脏表现与死亡率和较差的医院预后相关。
IF 6.6 1区 医学
Journal of Allergy and Clinical Immunology-In Practice Pub Date : 2025-09-23 DOI: 10.1016/j.jaip.2025.09.021
Ahmed Elmoursi, Daniel V DiGiacomo, Jocelyn R Farmer, Sara Barmettler
{"title":"Hepatic Manifestations in Common Variable Immunodeficiency Associated with Mortality and Worse Hospital Outcomes in Nationwide Analysis using National Readmission Database.","authors":"Ahmed Elmoursi, Daniel V DiGiacomo, Jocelyn R Farmer, Sara Barmettler","doi":"10.1016/j.jaip.2025.09.021","DOIUrl":"https://doi.org/10.1016/j.jaip.2025.09.021","url":null,"abstract":"<p><strong>Background: </strong>Liver disease is associated with increased mortality in patients with common variable immunodeficiency (CVID), yet we lack population-level data on its impact on CVID-associated hospitalizations in the United States.</p><p><strong>Objective: </strong>To conduct a nationwide analysis evaluating the prevalence, spectrum, clinical correlates, and outcomes of hepatic manifestations among admitted adults with CVID.</p><p><strong>Methods: </strong>We performed a retrospective cross-sectional analysis using the National Readmission Database, part of the Healthcare Cost and Utilization Project (HCUP) by the Agency for Healthcare Research and Quality. International Classification of Diseases (ICD)-10 codes were used to assess hepatic complications, comorbidities, risk factors, and outcomes in CVID-associated admissions with liver involvement.</p><p><strong>Results: </strong>Of 181,288 CVID-related index hospitalizations, 10% (18,823) had a co-diagnosed hepatic complication, specifically hepatic steatosis (38%), cirrhosis (24%), nonalcoholic fatty liver disease (17%), portal hypertension (PH) (15%), and nodular regenerative hyperplasia (NRH) (14%). CVID-associated admissions with hepatic disease had longer median lengths of stay (7 vs. 5 days, P<0.0001), higher in-hospital mortality (11% vs. 4%, P<0.0001), and higher median hospital charges ($68,114 vs. $44,757, P<0.0001). They also had a higher prevalence of hypertension, chronic kidney disease, diabetes, obesity, and autoimmune cytopenia (P<0.0001 for all). Alcohol use, hepatitis C, and hepatitis B were strong predictors of hepatic manifestations in CVID admissions (P<0.0001). CVID patients with NRH (HR: 1.3; 95% CI: 1.2-1.5, P<0.0001) and PH (HR: 1.4; 95% CI: 1.2-1.5, P<0.0001) had lower survival.</p><p><strong>Conclusions: </strong>CVID-associated admissions with liver disease, particularly those with NRH and PH, were longer, more costly, and associated with increased mortality. Further studies are needed to improve early detection and long-term outcomes.</p>","PeriodicalId":51323,"journal":{"name":"Journal of Allergy and Clinical Immunology-In Practice","volume":" ","pages":""},"PeriodicalIF":6.6,"publicationDate":"2025-09-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145150598","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}
引用次数: 0
Relationships of type 2 biomarkers with spirometry in response to tezepelumab in uncontrolled severe asthma. 2型生物标志物与不受控制的严重哮喘患者对tezepelumab反应的肺活量测定的关系
IF 6.6 1区 医学
Journal of Allergy and Clinical Immunology-In Practice Pub Date : 2025-09-23 DOI: 10.1016/j.jaip.2025.09.019
Robert Greig, Philipp Suter, Rory Chan, Brian Lipworth
{"title":"Relationships of type 2 biomarkers with spirometry in response to tezepelumab in uncontrolled severe asthma.","authors":"Robert Greig, Philipp Suter, Rory Chan, Brian Lipworth","doi":"10.1016/j.jaip.2025.09.019","DOIUrl":"https://doi.org/10.1016/j.jaip.2025.09.019","url":null,"abstract":"","PeriodicalId":51323,"journal":{"name":"Journal of Allergy and Clinical Immunology-In Practice","volume":" ","pages":""},"PeriodicalIF":6.6,"publicationDate":"2025-09-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145151615","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}
引用次数: 0
Real world adherence to biologic therapy for severe asthma in children. 现实世界中对儿童严重哮喘生物治疗的依从性。
IF 6.6 1区 医学
Journal of Allergy and Clinical Immunology-In Practice Pub Date : 2025-09-23 DOI: 10.1016/j.jaip.2025.09.020
Steven J Rose, Aisaku Nakamura, Md Monir Hossain, Theresa W Guilbert, Rachelle R Ramsey
{"title":"Real world adherence to biologic therapy for severe asthma in children.","authors":"Steven J Rose, Aisaku Nakamura, Md Monir Hossain, Theresa W Guilbert, Rachelle R Ramsey","doi":"10.1016/j.jaip.2025.09.020","DOIUrl":"https://doi.org/10.1016/j.jaip.2025.09.020","url":null,"abstract":"<p><strong>Background: </strong>Biologic medications improve asthma symptoms, spirometry, and exacerbation rates in patients with severe asthma. However, little is known about adherence to biologic medications in children.</p><p><strong>Objectives: </strong>To describe adherence to biologic medications in pediatric asthma and evaluate cofactors and outcomes related to adherence.</p><p><strong>Methods: </strong>A retrospective chart review was performed of patients at a single medical center with severe asthma to calculate adherence during the first year on biologic therapy. The relationship of adherence with method of administration (i.e. home nursing, clinic, or independent), medication dosing frequency, and insurance was examined using multiple regression models. The effect of biologic therapy on asthma outcomes was analyzed using generalized linear regression models.</p><p><strong>Results: </strong>Patients demonstrated 84% adherence to biologic therapies during the first 6 months of therapy (SD = 19, n=186) and 76% over 12 months (SD = 23, n=155). Method of administration, insurance type, and dosing frequency impacted adherence. Outcomes improved on biologic therapy such that asthma exacerbations decreased by 0.8 (SD=1.9) exacerbations per year, FVC z score improved by 0.26 (SD=0.7), and FEV1 z score improved by 0.3 (SD = 0.8). Decreased exacerbations rates and increased FVC and FEV1 were associated with adherence to therapy over the first 12 months.</p><p><strong>Conclusions: </strong>Patients with severe asthma demonstrated higher rates of adherence to asthma biologic medications than has been demonstrated for inhaled corticosteroids. Biologic therapies were associated with decreased asthma exacerbations and improved spirometry, and these changes were associated with adherence. Medication administration strategy, insurance type, and dosing frequency impacted adherence.</p>","PeriodicalId":51323,"journal":{"name":"Journal of Allergy and Clinical Immunology-In Practice","volume":" ","pages":""},"PeriodicalIF":6.6,"publicationDate":"2025-09-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145151260","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}
引用次数: 0
Big Data and Artificial Intelligence: Current State and Future Opportunities in Allery and Immunology. 大数据和人工智能:过敏和免疫学的现状和未来机遇。
IF 6.6 1区 医学
Journal of Allergy and Clinical Immunology-In Practice Pub Date : 2025-09-22 DOI: 10.1016/j.jaip.2025.09.011
Kim Kamphorst, Jamila de Jong, Nicholas L Rider, Jay M Portnoy
{"title":"Big Data and Artificial Intelligence: Current State and Future Opportunities in Allery and Immunology.","authors":"Kim Kamphorst, Jamila de Jong, Nicholas L Rider, Jay M Portnoy","doi":"10.1016/j.jaip.2025.09.011","DOIUrl":"https://doi.org/10.1016/j.jaip.2025.09.011","url":null,"abstract":"<p><p>Artificial intelligence (AI) and big data are reshaping the field of Allergy and Immunology, offering new opportunities to improve patient care, accelerate research, and inform clinical decision-making. The increasing availability of diverse data sources, including electronic health records, wearable devices, multi-omic profiles, environmental sensors, and patient-reported outcomes, has created an environment ready for innovation. AI techniques, particularly machine learning, are being applied to identify complex disease phenotypes, predict exacerbations, personalize treatment strategies, automate diagnostic tests interpretation, and streamline clinical documentation. Real-world examples already demonstrate the potential of AI and big data to support earlier diagnosis, optimize selection of biologics, and generate real-world evidence on treatment effectiveness and safety. However, several challenges remain, including the need for standardized data integration, protection of patient privacy, avoidance of algorithmic bias, and development of explainable, trustworthy AI systems. Ethical and practical considerations, such as equity in model development, transparency, and workflow integration, are critical for successful and responsible adoption in clinical practice. Lessons from other specialties, such as radiology and oncology, provide valuable models for implementation and highlight the importance of multidisciplinary collaboration. As the field moves forward, deliberate investment in technical infrastructure, governance, and clinician training will be essential to realize the promise of these technologies. In this context, this review provides an overview of these developments and highlights key considerations for their integration into clinical practice.</p>","PeriodicalId":51323,"journal":{"name":"Journal of Allergy and Clinical Immunology-In Practice","volume":" ","pages":""},"PeriodicalIF":6.6,"publicationDate":"2025-09-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145139424","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}
引用次数: 0
Sensitizations to all nonionic iodinated contrast media; a particular presentation after delayed hypersensitivity reactions. 对所有非离子碘化造影剂的敏化;迟发性超敏反应后的特殊表现。
IF 6.6 1区 医学
Journal of Allergy and Clinical Immunology-In Practice Pub Date : 2025-09-22 DOI: 10.1016/j.jaip.2025.09.016
Angèle Soria, Evelyne Collet, Camille Leleu, Marie Tauber, Justine Pasteur, Claire Bernier, Haudrey Assier, Emmanuelle Amsler, Annick Barbaud
{"title":"Sensitizations to all nonionic iodinated contrast media; a particular presentation after delayed hypersensitivity reactions.","authors":"Angèle Soria, Evelyne Collet, Camille Leleu, Marie Tauber, Justine Pasteur, Claire Bernier, Haudrey Assier, Emmanuelle Amsler, Annick Barbaud","doi":"10.1016/j.jaip.2025.09.016","DOIUrl":"https://doi.org/10.1016/j.jaip.2025.09.016","url":null,"abstract":"","PeriodicalId":51323,"journal":{"name":"Journal of Allergy and Clinical Immunology-In Practice","volume":" ","pages":""},"PeriodicalIF":6.6,"publicationDate":"2025-09-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145139480","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}
引用次数: 0
Development and validation of the FIP-Score for the screening of FIP1L1::PDGFRA-associated hypereosinophilic syndrome. FIP-Score用于筛选FIP1L1:: pdgfr相关的嗜酸性粒细胞增多综合征的开发和验证。
IF 6.6 1区 医学
Journal of Allergy and Clinical Immunology-In Practice Pub Date : 2025-09-22 DOI: 10.1016/j.jaip.2025.09.009
Romain Stammler, Alexandre Vallée, Julien Rohmer, Nathalie Grardel, Noémie Abisror, Wadih Abou Chahla, Félix Ackermann, Antoine Baudet, Nabil Belfeki, Raouf Ben Abdelali, Audrey Bidet, Jean-Sébastien Bladé, Séverine Bleuse, Bernard Bonnotte, Mohammed Azzedine Bouderbala, Jean-Michel Cayuela, Safia Chebrek, Jérémie Dion, Antoine Dossier, Nicolas Duployez, Stanislas Faguer, Pascale Flandrin-Gresta, Lionel Galicier, Catherine Godon, Maximilien Grall, Vincent Jachiet, Mathieu Jouvray, Irina Latu, Emmanuel Ledoult, Etienne Lengline, Amélie Leurs, Ludovic Lhermitte, François Lifermann, Nicolas Limal, Bertrand Lioger, Maxime Lugosi, Irène Machelart, Mickaël Martin, Nihal Martis, Laurent Mauvieux, Sara Melboucy-Belkhir, Catherine Mohr, Guillaume Moulis, Marie Joelle Mozicconacci, Dina Naguib, Antoine Néel, Franck E Nicolini, Roderau Outh, Kewin Panel, Claude Preudhomme, Mathieu Puyade, Thomas Quemeneur, Viviane Queyrel Moranne, Jérôme Rey, Philippe Rousselot, Nicolas Schleinitz, Borhane Slama, Delphine Staumont-Salle, Camille Taillé, Louis Terriou, Ludovic Tréfond, Jean François Viallard, Jean-Emmanuel Kahn, Guillaume Lefèvre, Matthieu Groh
{"title":"Development and validation of the FIP-Score for the screening of FIP1L1::PDGFRA-associated hypereosinophilic syndrome.","authors":"Romain Stammler, Alexandre Vallée, Julien Rohmer, Nathalie Grardel, Noémie Abisror, Wadih Abou Chahla, Félix Ackermann, Antoine Baudet, Nabil Belfeki, Raouf Ben Abdelali, Audrey Bidet, Jean-Sébastien Bladé, Séverine Bleuse, Bernard Bonnotte, Mohammed Azzedine Bouderbala, Jean-Michel Cayuela, Safia Chebrek, Jérémie Dion, Antoine Dossier, Nicolas Duployez, Stanislas Faguer, Pascale Flandrin-Gresta, Lionel Galicier, Catherine Godon, Maximilien Grall, Vincent Jachiet, Mathieu Jouvray, Irina Latu, Emmanuel Ledoult, Etienne Lengline, Amélie Leurs, Ludovic Lhermitte, François Lifermann, Nicolas Limal, Bertrand Lioger, Maxime Lugosi, Irène Machelart, Mickaël Martin, Nihal Martis, Laurent Mauvieux, Sara Melboucy-Belkhir, Catherine Mohr, Guillaume Moulis, Marie Joelle Mozicconacci, Dina Naguib, Antoine Néel, Franck E Nicolini, Roderau Outh, Kewin Panel, Claude Preudhomme, Mathieu Puyade, Thomas Quemeneur, Viviane Queyrel Moranne, Jérôme Rey, Philippe Rousselot, Nicolas Schleinitz, Borhane Slama, Delphine Staumont-Salle, Camille Taillé, Louis Terriou, Ludovic Tréfond, Jean François Viallard, Jean-Emmanuel Kahn, Guillaume Lefèvre, Matthieu Groh","doi":"10.1016/j.jaip.2025.09.009","DOIUrl":"https://doi.org/10.1016/j.jaip.2025.09.009","url":null,"abstract":"<p><strong>Background: </strong>FIP1L1-PDFGRA (F/P)-associated Hypereosinophilic Syndrome (HES) is a rare condition. F/P fusion gene testing is one of the first-line investigations in patients with unexplained eosinophilia and yields poor diagnostic performance.</p><p><strong>Objective: </strong>To build and validate the FIP-Score: a set of weighted criteria warranting testing for the F/P fusion gene.</p><p><strong>Methods: </strong>We merged data from 151 patients with F/P-associated HES and 320 patients with either F/P-negative HES (n=279) or hypereosinophilia of undetermined significance (n=41). Training and validation cohorts (comprising respectively 90% and 10% of all patients) were randomly dichotomized. Variables with a p-value <0.20 in univariate analysis were included in the multivariable forward-backward logistic regression model to assess their independent contribution to testing positive for the F/P fusion gene. Beta coefficients from multivariable logistic regression were used to assign points for the construction of the score.</p><p><strong>Results: </strong>Age under 66 years, male sex, splenomegaly, lymphomatoid papulosis, absence of gastrointestinal involvement, high serum vitamin B12, high serum tryptase and normal serum immunoglobulin E levels were the eight variables retained in the model. The best cutoff value was greater than 48. The model yielded a sensitivity, specificity, positive predictive value, negative predictive value and area under the curve respectively of 88.3%, 93.7%, 87.1%, 94.4%, and 0.962 in the training dataset and of 85.7%, 97.0%, 85.7% and 97.0%, 0.986 in the validation dataset.</p><p><strong>Conclusion: </strong>The FIP-score highlights the need for closely selecting patients with hypereosinophilia for whom F/P fusion gene testing should be performed, resulting in medical time reduction and substantial cost-savings.</p>","PeriodicalId":51323,"journal":{"name":"Journal of Allergy and Clinical Immunology-In Practice","volume":" ","pages":""},"PeriodicalIF":6.6,"publicationDate":"2025-09-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145139475","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}
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