Clinical Reviews in Allergy & Immunology最新文献

Short-Chain Fatty Acids: Promising Therapeutic Targets for Respiratory Syncytial Virus Infection.

IF 8.4 2区医学

Clinical Reviews in Allergy & Immunology Pub Date : 2025-01-28 DOI: 10.1007/s12016-024-09018-x

Mingxin Liang, Qinqin Dong, Weiyi Wu, Juan Fan

引用次数: 0

TTC7A Variants Results in Gastrointestinal Defects and Immunodeficiency Syndrome: Case Series and Literature Review.

IF 8.4 2区医学

Clinical Reviews in Allergy & Immunology Pub Date : 2025-01-28 DOI: 10.1007/s12016-024-09017-y

Zhaowei Huang, Xiufang Zhi, Qiuyu Geng, Xiaoqi Yuan, Xiaoxia Zhao, Qi Qin, Jingan Lou, Fan Tong, Jinfa Tou, Dengming Lai

{"title":"TTC7A Variants Results in Gastrointestinal Defects and Immunodeficiency Syndrome: Case Series and Literature Review.","authors":"Zhaowei Huang, Xiufang Zhi, Qiuyu Geng, Xiaoqi Yuan, Xiaoxia Zhao, Qi Qin, Jingan Lou, Fan Tong, Jinfa Tou, Dengming Lai","doi":"10.1007/s12016-024-09017-y","DOIUrl":"https://doi.org/10.1007/s12016-024-09017-y","url":null,"abstract":"Gastrointestinal Defects and Immunodeficiency Syndrome-1 (GIDID-1), caused by abnormalities in TTC7A, is an autosomal recessive disorder characterized by multiple gastrointestinal malformations and immune deficiencies, often accompanied by inflammatory bowel disease (IBD). This condition typically results in poor treatment outcomes and is usually fatal in early infancy. This paper examined the genetic abnormalities and clinical features of GIDID by analyzing data from three children and one fetus with gastrointestinal dysfunction and immune deficiency associated with TTC7A abnormalities at our hospital, and reviewed reported cases worldwide. Genetic analysis of the four patients identified eight novel variants in the TTC7A, five of which were likely pathogenic variants, while three were of uncertain significance. Including the cases reported in this paper and through a literature review, there were 89 known cases globally, involving 79 TTC7A variants. Patients typically presented with multiple gastrointestinal malformations, immune deficiencies, or IBD. Thus, genetic testing is recommended for patients with multiple gastrointestinal malformations and recurrent infections to determine if GIDID is due to TTC7A abnormalities. The syndrome generally has a poor prognosis, and this information is crucial for treatment planning, prenatal screening, and genetic counseling.","PeriodicalId":10423,"journal":{"name":"Clinical Reviews in Allergy & Immunology","volume":"68 1","pages":"7"},"PeriodicalIF":8.4,"publicationDate":"2025-01-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143051762","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"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

Histone Modifications and DNA Methylation in Psoriasis: A Cellular Perspective.

IF 8.4 2区医学

Clinical Reviews in Allergy & Immunology Pub Date : 2025-01-27 DOI: 10.1007/s12016-024-09014-1

Jing Pan, Siji Chen, Xianzhen Chen, Yinjing Song, Hao Cheng

{"title":"Histone Modifications and DNA Methylation in Psoriasis: A Cellular Perspective.","authors":"Jing Pan, Siji Chen, Xianzhen Chen, Yinjing Song, Hao Cheng","doi":"10.1007/s12016-024-09014-1","DOIUrl":"https://doi.org/10.1007/s12016-024-09014-1","url":null,"abstract":"In recent years, epigenetic modifications have attracted significant attention due to their unique regulatory mechanisms and profound biological implications. Acting as a bridge between environmental stimuli and changes in gene activity, they reshape gene expression patterns, providing organisms with regulatory mechanisms to respond to environmental changes. A growing body of evidence indicates that epigenetic regulation plays a crucial role in the pathogenesis and progression of psoriasis. A deeper understanding of these epigenetic mechanisms not only helps unveil the molecular mechanisms underlying the initiation and progression of psoriasis but may also provide new insights into diagnostic and therapeutic strategies. Given the unique roles and significant contributions of various cell types involved in the process of psoriasis, a thorough analysis of specific epigenetic patterns in different cell types becomes a key entry point for elucidating the mechanisms of disease development. Although epigenetic modifications encompass multiple complex layers, this review will focus on histone modifications and DNA methylation, describing how they function in different cell types and subsequently impact the pathophysiological processes of psoriasis. Finally, we will summarize the current problems in research concerning histone modifications and DNA methylation in psoriasis and discuss the clinical application prospects and challenges of targeting epigenetic modifications as therapeutic strategies for psoriasis.","PeriodicalId":10423,"journal":{"name":"Clinical Reviews in Allergy & Immunology","volume":"68 1","pages":"6"},"PeriodicalIF":8.4,"publicationDate":"2025-01-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143051707","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"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

Exosomes in Autoimmune Diseases: A Review of Mechanisms and Diagnostic Applications. 自身免疫性疾病中的外泌体：机制和诊断应用综述

IF 8.4 2区医学

Clinical Reviews in Allergy & Immunology Pub Date : 2025-01-17 DOI: 10.1007/s12016-024-09013-2

Lina Duan, Wanying Lin, Yi Zhang, Lingyue Jin, Jie Xiao, Haifang Wang, Shuyin Pang, Hongxia Wang, Dehua Sun, Ying Gong, Haixia Li

{"title":"Exosomes in Autoimmune Diseases: A Review of Mechanisms and Diagnostic Applications.","authors":"Lina Duan, Wanying Lin, Yi Zhang, Lingyue Jin, Jie Xiao, Haifang Wang, Shuyin Pang, Hongxia Wang, Dehua Sun, Ying Gong, Haixia Li","doi":"10.1007/s12016-024-09013-2","DOIUrl":"https://doi.org/10.1007/s12016-024-09013-2","url":null,"abstract":"Exosomes, small extracellular vesicles secreted by various cell types, have emerged as key players in the pathophysiology of autoimmune diseases. These vesicles serve as mediators of intercellular communication, facilitating the transfer of bioactive molecules such as proteins, lipids, and nucleotide. In autoimmune diseases, exosomes have been implicated in modulating immune responses, oxidative stress, autophagy, gut microbes, and the cell cycle, contributing to disease initiation, progression, and immune dysregulation. Recent advancements in exosome isolation techniques and their molecular characterization have paved the way for exploring their clinical potential as biomarkers and therapeutic targets. This review focuses on the mechanisms by which exosomes influence autoimmune disease development and their potential clinical applications, particularly in diagnosis. The role of exosomes in autoimmune diseases, including systemic lupus erythematosus (SLE), rheumatoid arthritis (RA), type 1 diabetes mellitus (T1DM), inflammatory bowel disease (IBD), and Sjögren's syndrome (SS), is discussed in relation to their involvements in antigen presentation, T-cell activation, and the induction of inflammatory pathways. Additionally, exosome-based biomarkers offer promising non-invasive diagnostic tools for early diagnostic, disease monitoring, and therapeutic response assessment. However, challenges such as standardization of exosome isolation protocols and validation of their clinical significance remain. This review highlights the potential of exosomes as both diagnostic biomarkers and therapeutic targets in autoimmune diseases, emphasizing the need for further research to overcome current limitations and fully harness their clinical value.","PeriodicalId":10423,"journal":{"name":"Clinical Reviews in Allergy & Immunology","volume":"68 1","pages":"5"},"PeriodicalIF":8.4,"publicationDate":"2025-01-17","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143001014","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"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

Copy Number Variation in Asthma: An Integrative Review. 哮喘的拷贝数变异：一项综合综述。

IF 8.4 2区医学

Clinical Reviews in Allergy & Immunology Pub Date : 2025-01-04 DOI: 10.1007/s12016-024-09015-0

Fernanda Mariano Garcia, Valdemir Pereira de Sousa, Priscila Pinto E Silva-Dos-Santos, Izadora Silveira Fernandes, Faradiba Sarquis Serpa, Flávia de Paula, José Geraldo Mill, Maria Rita Passos Bueno, Flávia Imbroisi Valle Errera

{"title":"Copy Number Variation in Asthma: An Integrative Review.","authors":"Fernanda Mariano Garcia, Valdemir Pereira de Sousa, Priscila Pinto E Silva-Dos-Santos, Izadora Silveira Fernandes, Faradiba Sarquis Serpa, Flávia de Paula, José Geraldo Mill, Maria Rita Passos Bueno, Flávia Imbroisi Valle Errera","doi":"10.1007/s12016-024-09015-0","DOIUrl":"https://doi.org/10.1007/s12016-024-09015-0","url":null,"abstract":"Asthma is a complex disease with varied clinical manifestations resulting from the interaction between environmental and genetic factors. While chronic airway inflammation and hyperresponsiveness are central features, the etiology of asthma is multifaceted, leading to a diversity of phenotypes and endotypes. Although most research into the genetics of asthma focused on the analysis of single nucleotide polymorphisms (SNPs), studies highlight the importance of structural variations, such as copy number variations (CNVs), in the inheritance of complex characteristics, but their role has not yet been fully elucidated in asthma. In this context, an integrative review was conducted to identify the genes and pathways involved, the location, size, and classes of CNVs, as well as their contribution to asthma risk, severity, control, and response to treatment. As a result of the review, 16 articles were analyzed, from different types of observational studies, such as case-control, cohort studies and genotyped-proband or trios design, that have been carried out in populations from different countries, ethnicities, and ages. Chromosomes 12 and 17 were the most studied in three publications each. CNVs located on 12 chromosomes were associated with asthma, the majority being found on chromosome 6p and 17q, of the deletion type, encompassing 30 different coding-protein genes and one pseudogene region. Six genes with CNVs were identified as significant expression quantitative locus (eQTLs) with mean expression in asthma-related tissues, such as the lung and whole blood. The phenotypic variability of asthma may hinder the clinical application of these findings, but the research shows the importance of investigating these genetic variations as possible biomarkers in asthma patients.","PeriodicalId":10423,"journal":{"name":"Clinical Reviews in Allergy & Immunology","volume":"68 1","pages":"4"},"PeriodicalIF":8.4,"publicationDate":"2025-01-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142926549","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"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

Regulatory Roles of SWI/SNF Chromatin Remodeling Complexes in Immune Response and Inflammatory Diseases. SWI/SNF染色质重塑复合物在免疫反应和炎症性疾病中的调节作用。

IF 8.4 2区医学

Clinical Reviews in Allergy & Immunology Pub Date : 2024-12-30 DOI: 10.1007/s12016-024-09011-4

Shunan Sun, Yu Chen, Yuzhen Ouyang, Zhenwei Tang

{"title":"Regulatory Roles of SWI/SNF Chromatin Remodeling Complexes in Immune Response and Inflammatory Diseases.","authors":"Shunan Sun, Yu Chen, Yuzhen Ouyang, Zhenwei Tang","doi":"10.1007/s12016-024-09011-4","DOIUrl":"10.1007/s12016-024-09011-4","url":null,"abstract":"The switch/sucrose non-fermentable (SWI/SNF) chromatin remodeling complexes (also referred to as BAF complexes) are composed of multiple subunits, which regulate the nucleosome translocation and chromatin accessibility. In recent years, significant advancements have been made in understanding mutated genes encoding subunits of the SWI/SNF complexes in cancer biology. Nevertheless, the role of SWI/SNF complexes in immune response and inflammatory diseases continues to attract significant attention. This review presents a summary of the significant functions of SWI/SNF complexes during the overall process from the development to the activation of innate and adaptive immune cells. In addition, the correlation between various SWI/SNF subunits and diverse inflammatory diseases is explored. Further investigations are warranted in terms of the mechanism of SWI/SNF complexes' preference for binding sites and opposite pro-/anti-inflammatory effects. In conclusion, further efforts are needed to evaluate the druggability of targeting SWI/SNF complexes in inflammatory diseases, and we hope this review will inspire the development of novel immune modulators in clinical practice.","PeriodicalId":10423,"journal":{"name":"Clinical Reviews in Allergy & Immunology","volume":"68 1","pages":"2"},"PeriodicalIF":8.4,"publicationDate":"2024-12-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142920968","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"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

Group 3 Innate Lymphoid Cells: A Potential Therapeutic Target for Steroid Resistant Asthma. 先天淋巴样细胞：类固醇抵抗性哮喘的潜在治疗靶点

IF 8.4 2区医学

Clinical Reviews in Allergy & Immunology Pub Date : 2024-12-27 DOI: 10.1007/s12016-024-09012-3

Marzhan Berkinbayeva, Wenjing Gu, Zhifeng Chen, Peisong Gao

{"title":"Group 3 Innate Lymphoid Cells: A Potential Therapeutic Target for Steroid Resistant Asthma.","authors":"Marzhan Berkinbayeva, Wenjing Gu, Zhifeng Chen, Peisong Gao","doi":"10.1007/s12016-024-09012-3","DOIUrl":"10.1007/s12016-024-09012-3","url":null,"abstract":"Asthma is a chronic airway inflammatory disease that affects millions globally. Although glucocorticoids are a mainstay of asthma treatment, a subset of patients show resistance to these therapies, resulting in poor disease control and increased morbidity. The complex mechanisms underlying steroid-resistant asthma (SRA) involve Th1 and Th17 lymphocyte activity, neutrophil recruitment, and NLRP3 inflammasome activation. Recent studies provided evidence that innate lymphoid cells type 3 (ILC3s) might be potential therapeutic targets for non-eosinophilic asthma (NEA) and SRA. Like Th17 cells, ILC3s play crucial roles in immune responses, inflammation, and tissue homeostasis, contributing to disease severity and corticosteroid resistance in NEA. Biologics targeting ILC3-related pathways have shown promise in managing Th2-low asthma, suggesting new avenues for SRA treatment. This review aims to explore the risk factors for SRA, discuss the challenges and mechanisms underlying SRA, consolidate current findings on innate lymphoid cells, and elucidate their role in respiratory conditions. We present the latest findings on the involvement of ILC3s in human diseases and explore their potential mechanisms in SRA development. Furthermore, we review emerging therapeutic biologics targeting ILC3-related pathways in managing NEA and SRA. This review highlights current challenges, and emerging therapeutic strategies, and addresses a significant gap in asthma research, with implications for improving the management of steroid-resistant asthma.","PeriodicalId":10423,"journal":{"name":"Clinical Reviews in Allergy & Immunology","volume":"68 1","pages":"1"},"PeriodicalIF":8.4,"publicationDate":"2024-12-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11698894/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142920966","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"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

Olfactory Dysfunction in Allergic Rhinitis. 过敏性鼻炎的嗅觉功能障碍。

IF 8.4 2区医学

Clinical Reviews in Allergy & Immunology Pub Date : 2024-12-26 DOI: 10.1007/s12016-024-09016-z

Xinyu Zhang, Yian Zhou, Zheng Liu, Yang Liu

{"title":"Olfactory Dysfunction in Allergic Rhinitis.","authors":"Xinyu Zhang, Yian Zhou, Zheng Liu, Yang Liu","doi":"10.1007/s12016-024-09016-z","DOIUrl":"10.1007/s12016-024-09016-z","url":null,"abstract":"Olfactory dysfunction (OD) can have serious consequences as it hinders individuals from detecting important warning signals like smoke, spoiled food, and gas leaks. This can significantly impact their nutritional status, eating satisfaction, and overall quality of life. Allergic rhinitis (AR) is a common disease that greatly affects the quality of life and can lead to a decrease, distortion, or complete loss of olfactory ability. There are various tools available for diagnosing OD, ranging from simple screening tests to more detailed and complex methods such as electrophysiological and imaging procedures not available in everyday practice but reserved for experimental studies. The underlying mechanisms by which AR impacts olfactory ability are still not fully understood and are likely to be multifactorial. Current therapeutic options for OD resulting from AR are limited and only provide partial or temporary relief from olfactory impairment. However, there are promising treatments investigated such as biologics, olfactory training, neural stem cell transplantation, and various novel target therapies that might help improve OD in AR in the future. This review aims to evaluate the epidemiology, mechanisms, and detection of OD in AR, as well as provide an overview of the management strategies for OD secondary to AR.","PeriodicalId":10423,"journal":{"name":"Clinical Reviews in Allergy & Immunology","volume":"68 1","pages":"3"},"PeriodicalIF":8.4,"publicationDate":"2024-12-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142920967","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"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

Non-allergic Hypersensitivity Reactions to Immunoglobulin Preparations in Antibody Deficiencies: What Role for Anti-IgA IgG and Complement Activation? 抗体缺乏症患者对免疫球蛋白制剂的非过敏性超敏反应：抗IgA IgG和补体激活的作用是什么？

IF 8.4 2区医学

Clinical Reviews in Allergy & Immunology Pub Date : 2024-12-01 Epub Date: 2024-10-22 DOI: 10.1007/s12016-024-09007-0

Aurore Collet, Diane Pelletier de Chambure, Emmanuelle Moitrot, Gaëlle Breyne, Floriane Mirgot, Stéphanie Rogeau, Mathieu Tronchon, Amélie Nicolas, Sébastien Sanges, Sarah Stabler, Emmanuel Ledoult, Louis Terriou, David Launay, Eric Hachulla, Myriam Labalette, Sylvain Dubucquoi, Guillaume Lefèvre

{"title":"Non-allergic Hypersensitivity Reactions to Immunoglobulin Preparations in Antibody Deficiencies: What Role for Anti-IgA IgG and Complement Activation?","authors":"Aurore Collet, Diane Pelletier de Chambure, Emmanuelle Moitrot, Gaëlle Breyne, Floriane Mirgot, Stéphanie Rogeau, Mathieu Tronchon, Amélie Nicolas, Sébastien Sanges, Sarah Stabler, Emmanuel Ledoult, Louis Terriou, David Launay, Eric Hachulla, Myriam Labalette, Sylvain Dubucquoi, Guillaume Lefèvre","doi":"10.1007/s12016-024-09007-0","DOIUrl":"10.1007/s12016-024-09007-0","url":null,"abstract":"The presence of IgG anti-IgA in the serum of primary immunodeficiency (PID) patients has long been considered responsible for hypersensitivity (HS) to immunoglobulin preparations (IgPs), but this link is increasingly being questioned. The aim of this work was to describe the prevalence of IgG anti-IgA and its association with HS, and to explore a new pathophysiological hypothesis involving the complement system. We measured IgG anti-IgA, using a standardised commercial technique, in controls and PID patients, and compared our results to a systematic literature review. We measured complement activation in PID patients before and after IgP infusion, and in vitro after incubation of IgP with serum from controls and PID patients. IgG anti-IgA was detected in 6% (n = 2/32) of PID patients, 30% (n = 3/10) of selective IgA deficiency patients and 2% (n = 1/46) of healthy controls. In the literature and our study, 38 PID patients had IgG anti-IgA and HS to IgPs and 9 had IgG anti-IgA but good tolerance to IgPs. In our patients, we observed a constant complement activation after IgP infusion compared to baseline. In vitro, IgP induced significant complement activation with all sera from tested individuals. IgA immunisation is not rare in PID, higher in selective IgA deficiency, but may also occur in healthy controls. Our results question the clinical relevance and pathophysiological implication of IgG anti-IgA in the context of HS with IgPs. Complement activation-related pseudoallergy could explain the clinical characteristics and natural history of HS symptoms.","PeriodicalId":10423,"journal":{"name":"Clinical Reviews in Allergy & Immunology","volume":" ","pages":"47-57"},"PeriodicalIF":8.4,"publicationDate":"2024-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11638320/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142459647","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"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

Hereditary Angioedema Attacks in Patients Receiving Long-Term Prophylaxis: A Systematic Review. 长期接受预防性治疗的患者中的遗传性血管性水肿发作：系统回顾。

IF 8.4 2区医学

Clinical Reviews in Allergy & Immunology Pub Date : 2024-12-01 Epub Date: 2024-11-07 DOI: 10.1007/s12016-024-09006-1

Hilary J Longhurst, Mauro Cancian, Vesna Grivcheva-Panovska, Majed Koleilat, Markus Magerl, Sinisa Savic, Marcin Stobiecki, Raffi Tachdjian, Bridget Healy, Christopher M Yea, Paul K Audhya, Laurence Bouillet

{"title":"Hereditary Angioedema Attacks in Patients Receiving Long-Term Prophylaxis: A Systematic Review.","authors":"Hilary J Longhurst, Mauro Cancian, Vesna Grivcheva-Panovska, Majed Koleilat, Markus Magerl, Sinisa Savic, Marcin Stobiecki, Raffi Tachdjian, Bridget Healy, Christopher M Yea, Paul K Audhya, Laurence Bouillet","doi":"10.1007/s12016-024-09006-1","DOIUrl":"10.1007/s12016-024-09006-1","url":null,"abstract":"Long-term prophylaxis (LTP) has been shown to reduce the frequency of hereditary angioedema (HAE) attacks; however, attacks occurring in patients receiving LTP have not been well characterized. The objective of this systematic review was to evaluate the proportion of type I/II HAE (HAE-C1INH) patients who experience attacks while receiving LTP, the characteristics of these attacks, and associated on-demand therapy use. A systematic search was conducted in PubMed to identify studies reporting LTP use with plasma-derived C1 inhibitor (pdC1INH), lanadelumab, berotralstat, androgens, or antifibrinolytics in patients with HAE-C1INH. Forty-five primary studies met the inclusion criteria. In phase 3 trials, attack-free rates were 40% for subcutaneous pdC1INH 60 IU/kg twice weekly at 16 weeks, and 44% for lanadelumab 300 mg every second week at 6 months (77% during steady-state [days 70-182]); there was no difference in attack-free rate for berotralstat 150 mg versus placebo at 24 weeks. Phase 3 studies reported a lower average attack severity with subcutaneous and intravenous pdC1INH versus placebo. With lanadelumab and berotralstat, the prophylactic treatment effect was more pronounced in peripheral attacks than in abdominal and laryngeal attacks. Laryngeal attacks accounted for 2%-7% of all attacks in observational and interventional studies, regardless of the LTP agent received. On-demand therapy was used in 49%-94% of attacks occurring in the presence of LTP. In conclusion, patients receiving LTP experienced attacks in all anatomic locations, including the larynx. Most attacks were treated with on-demand therapy, although outcomes were not reported. Access to on-demand therapy remains essential for all people with HAE-C1INH.","PeriodicalId":10423,"journal":{"name":"Clinical Reviews in Allergy & Immunology","volume":" ","pages":"83-95"},"PeriodicalIF":8.4,"publicationDate":"2024-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11638394/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142603592","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"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