Pub Date : 2024-07-30DOI: 10.1080/1744666X.2024.2386427
Prabal Barman, Suprit Basu, Taru Goyal, Saniya Sharma, Sangeetha Siniah, Rahul Tyagi, Kaushal Sharma, Ankur K Jindal, Rakesh K Pilania, Pandiarajan Vignesh, Manpreet Dhaliwal, Deepti Suri, Amit Rawat, Surjit Singh
Introduction: Inborn errors of immunity (IEI) are a group of genetically heterogeneous disorders with a wide-ranging clinical phenotype, varying from increased predisposition to infections to dysregulation of the immune system, including autoimmune phenomena, autoinflammatory disorders, lymphoproliferation, and malignancy. Lymphoproliferative disorder (LPD) in IEI refers to the nodal or extra-nodal and persistent or recurrent clonal or non-clonal proliferation of lymphoid cells in the clinical context of an inherited immunodeficiency or immune dysregulation. The Epstein-Barr virus (EBV) plays a significant role in the etiopathogenesis of LPD in IEIs. In patients with specific IEIs, lack of immune surveillance can lead to an uninhibited proliferation of EBV-infected cells that may result in chronic active EBV infection, hemophagocytic lymphohistiocytosis, and LPD, particularly lymphomas.
Areas covered: We intend to discuss the pathogenesis, diagnosis, and treatment modalities directed toward EBV-associated LPD in patients with distinct IEIs.
Expert opinion: EBV-driven lymphoproliferation in IEIs presents a diagnostic and therapeutic problem that necessitates a comprehensive understanding of host-pathogen interactions, immune dysregulation, and personalized treatment approaches. A multidisciplinary approach involving immunologists, hematologists, infectious disease specialists, and geneticists is paramount to addressing the diagnostic and therapeutic challenges posed by this intriguing yet formidable clinical entity.
{"title":"Epstein-Barr virus-driven lymphoproliferation in inborn errors of immunity: a diagnostic and therapeutic challenge.","authors":"Prabal Barman, Suprit Basu, Taru Goyal, Saniya Sharma, Sangeetha Siniah, Rahul Tyagi, Kaushal Sharma, Ankur K Jindal, Rakesh K Pilania, Pandiarajan Vignesh, Manpreet Dhaliwal, Deepti Suri, Amit Rawat, Surjit Singh","doi":"10.1080/1744666X.2024.2386427","DOIUrl":"10.1080/1744666X.2024.2386427","url":null,"abstract":"<p><strong>Introduction: </strong>Inborn errors of immunity (IEI) are a group of genetically heterogeneous disorders with a wide-ranging clinical phenotype, varying from increased predisposition to infections to dysregulation of the immune system, including autoimmune phenomena, autoinflammatory disorders, lymphoproliferation, and malignancy. Lymphoproliferative disorder (LPD) in IEI refers to the nodal or extra-nodal and persistent or recurrent clonal or non-clonal proliferation of lymphoid cells in the clinical context of an inherited immunodeficiency or immune dysregulation. The Epstein-Barr virus (EBV) plays a significant role in the etiopathogenesis of LPD in IEIs. In patients with specific IEIs, lack of immune surveillance can lead to an uninhibited proliferation of EBV-infected cells that may result in chronic active EBV infection, hemophagocytic lymphohistiocytosis, and LPD, particularly lymphomas.</p><p><strong>Areas covered: </strong>We intend to discuss the pathogenesis, diagnosis, and treatment modalities directed toward EBV-associated LPD in patients with distinct IEIs.</p><p><strong>Expert opinion: </strong>EBV-driven lymphoproliferation in IEIs presents a diagnostic and therapeutic problem that necessitates a comprehensive understanding of host-pathogen interactions, immune dysregulation, and personalized treatment approaches. A multidisciplinary approach involving immunologists, hematologists, infectious disease specialists, and geneticists is paramount to addressing the diagnostic and therapeutic challenges posed by this intriguing yet formidable clinical entity.</p>","PeriodicalId":12175,"journal":{"name":"Expert Review of Clinical Immunology","volume":null,"pages":null},"PeriodicalIF":3.9,"publicationDate":"2024-07-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141765859","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}
Pub Date : 2024-07-29DOI: 10.1080/1744666X.2024.2384705
Fadi Kharouf, Dafna D Gladman
Introduction: There are several treatment controversies that have emerged in spondyloarthritis and psoriatic arthritis. These are related to the nature of the conditions as well as to the use of medications.
Areas covered: This review, which included a search of PubMed database as well as the references within the articles provides an overview of the nature of spondyloarthritis, controversy over the inclusion of psoriatic arthritis (PsA) as a peripheral spondyloarthritis, and a summary of current treatments for both PsA and axial spondyloarthritis (axSpA), with special emphasis on targeted therapy. The review highlights the differences in response to certain medications, particularly biologic therapy and summarizes the randomized controlled trials in psoriatic arthritis and axial spondyloarthritis providing data about the responses in table format.
Expert opinion: There is a need for better outcome measures in axSpA. Currently, the measures are subjective. Imaging may be more appropriate but there is a need for research into the reliability and responsiveness of imaging techniques. In PsA, there may also be better response measures and research into the reliability and responsiveness of available measures is underway. There is also a need for novel therapies as well as biomarkers for response in both diseases.
{"title":"Treatment controversies in spondyloarthritis and psoriatic arthritis: focus on biologics and targeted therapies.","authors":"Fadi Kharouf, Dafna D Gladman","doi":"10.1080/1744666X.2024.2384705","DOIUrl":"https://doi.org/10.1080/1744666X.2024.2384705","url":null,"abstract":"<p><strong>Introduction: </strong>There are several treatment controversies that have emerged in spondyloarthritis and psoriatic arthritis. These are related to the nature of the conditions as well as to the use of medications.</p><p><strong>Areas covered: </strong>This review, which included a search of PubMed database as well as the references within the articles provides an overview of the nature of spondyloarthritis, controversy over the inclusion of psoriatic arthritis (PsA) as a peripheral spondyloarthritis, and a summary of current treatments for both PsA and axial spondyloarthritis (axSpA), with special emphasis on targeted therapy. The review highlights the differences in response to certain medications, particularly biologic therapy and summarizes the randomized controlled trials in psoriatic arthritis and axial spondyloarthritis providing data about the responses in table format.</p><p><strong>Expert opinion: </strong>There is a need for better outcome measures in axSpA. Currently, the measures are subjective. Imaging may be more appropriate but there is a need for research into the reliability and responsiveness of imaging techniques. In PsA, there may also be better response measures and research into the reliability and responsiveness of available measures is underway. There is also a need for novel therapies as well as biomarkers for response in both diseases.</p>","PeriodicalId":12175,"journal":{"name":"Expert Review of Clinical Immunology","volume":null,"pages":null},"PeriodicalIF":3.9,"publicationDate":"2024-07-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141787668","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}
Pub Date : 2024-07-24DOI: 10.1080/1744666X.2024.2384060
Raymund R Razonable
Introduction: Cytomegalovirus (CMV) is a common opportunistic infection after solid organ transplantation, with significant impact on morbidity and long-term survival. Despite advances in diagnostics and therapeutics, the management of CMV remains very challenging.
Areas covered: This article reviews emerging data on the clinical utility of laboratory assays that quantify cell-mediated immune responses to CMV. Observational studies have consistently demonstrated that a deficiency in pathogen-specific cell-mediated immunity is correlated with a heightened risk of primary, reactivation or recurrent CMV after transplantation. A limited number of interventional studies have recently investigated cell-mediated immune assays in guiding the prevention and treatment of CMV infection after solid organ transplantation.
Expert opinion: The pathogenesis and outcome of CMV after solid organ transplantion reflect the interplay between viral replication and CMV-specific immune reconstitution. Research in CMV-specific cell-mediated immunity paved way for the development of several laboratory assays that may assist clinicians in predicting the risk of CMV after transplantation, individualize the approach to CMV disease prevention, guide the need and duration of treatment of CMV infection, and predict the risk of relapse after treatment. More interventional studies are needed to further solidify the role of cell-mediated immune assays in various clinical situations after transplantation.
{"title":"Pathogen-specific cell-mediated immunity to guide the management of cytomegalovirus in solid organ transplantation: state of the art clinical review.","authors":"Raymund R Razonable","doi":"10.1080/1744666X.2024.2384060","DOIUrl":"10.1080/1744666X.2024.2384060","url":null,"abstract":"<p><strong>Introduction: </strong>Cytomegalovirus (CMV) is a common opportunistic infection after solid organ transplantation, with significant impact on morbidity and long-term survival. Despite advances in diagnostics and therapeutics, the management of CMV remains very challenging.</p><p><strong>Areas covered: </strong>This article reviews emerging data on the clinical utility of laboratory assays that quantify cell-mediated immune responses to CMV. Observational studies have consistently demonstrated that a deficiency in pathogen-specific cell-mediated immunity is correlated with a heightened risk of primary, reactivation or recurrent CMV after transplantation. A limited number of interventional studies have recently investigated cell-mediated immune assays in guiding the prevention and treatment of CMV infection after solid organ transplantation.</p><p><strong>Expert opinion: </strong>The pathogenesis and outcome of CMV after solid organ transplantion reflect the interplay between viral replication and CMV-specific immune reconstitution. Research in CMV-specific cell-mediated immunity paved way for the development of several laboratory assays that may assist clinicians in predicting the risk of CMV after transplantation, individualize the approach to CMV disease prevention, guide the need and duration of treatment of CMV infection, and predict the risk of relapse after treatment. More interventional studies are needed to further solidify the role of cell-mediated immune assays in various clinical situations after transplantation.</p>","PeriodicalId":12175,"journal":{"name":"Expert Review of Clinical Immunology","volume":null,"pages":null},"PeriodicalIF":3.9,"publicationDate":"2024-07-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141747842","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}
Pub Date : 2024-07-24DOI: 10.1080/1744666X.2024.2384057
Vadim Gorodetskiy, Andrey Sudarikov
Introduction: The typical clinical manifestations of T-cell large granular lymphocyte (T-LGL) leukemia are an increase in the number of large granular lymphocytes (LGLs) in the blood > 2000 cells/μL, neutropenia, and splenomegaly. In rare cases of so-called 'aleukemic' T-LGL leukemia, the number of LGLs is <400-500 cells/μL. In patients with rheumatoid arthritis (RA), distinguishing T-LGL leukemia with low tumor burden in the blood and bone marrow from Felty syndrome (FS) poses diagnostic challenges.
Areas covered: This review aimed to describe the basic characteristics and variants of aleukemic T-LGL leukemia, with a special focus on aleukemic T-LGL leukemia with massive splenomegaly (splenic variant of T-LGL leukemia) and differential diagnosis of such cases with hepatosplenic T-cell lymphoma. The significance of mutations in the signal transducer and activator of transcription 3 (STAT3) gene for distinguishing aleukemic RA-associated T-LGL leukemia from FS is discussed, along with the evolution of the T-LGL leukemia diagnostic criteria. PubMed database was used to search for the most relevant literature.
Expert opinion: Evaluation of STAT3 mutations in the blood and bone marrow using next-generation sequencing, as well as a comprehensive spleen study, may be necessary to establish a diagnosis of aleukemic RA-associated T-LGL leukemia.
{"title":"Aleukemic variant of T-cell large granular lymphocyte leukemia in patients with rheumatoid arthritis - diagnostically challenging subtype.","authors":"Vadim Gorodetskiy, Andrey Sudarikov","doi":"10.1080/1744666X.2024.2384057","DOIUrl":"https://doi.org/10.1080/1744666X.2024.2384057","url":null,"abstract":"<p><strong>Introduction: </strong>The typical clinical manifestations of T-cell large granular lymphocyte (T-LGL) leukemia are an increase in the number of large granular lymphocytes (LGLs) in the blood > 2000 cells/μL, neutropenia, and splenomegaly. In rare cases of so-called 'aleukemic' T-LGL leukemia, the number of LGLs is <400-500 cells/μL. In patients with rheumatoid arthritis (RA), distinguishing T-LGL leukemia with low tumor burden in the blood and bone marrow from Felty syndrome (FS) poses diagnostic challenges.</p><p><strong>Areas covered: </strong>This review aimed to describe the basic characteristics and variants of aleukemic T-LGL leukemia, with a special focus on aleukemic T-LGL leukemia with massive splenomegaly (splenic variant of T-LGL leukemia) and differential diagnosis of such cases with hepatosplenic T-cell lymphoma. The significance of mutations in the signal transducer and activator of transcription 3 (<i>STAT3</i>) gene for distinguishing aleukemic RA-associated T-LGL leukemia from FS is discussed, along with the evolution of the T-LGL leukemia diagnostic criteria. PubMed database was used to search for the most relevant literature.</p><p><strong>Expert opinion: </strong>Evaluation of <i>STAT3</i> mutations in the blood and bone marrow using next-generation sequencing, as well as a comprehensive spleen study, may be necessary to establish a diagnosis of aleukemic RA-associated T-LGL leukemia.</p>","PeriodicalId":12175,"journal":{"name":"Expert Review of Clinical Immunology","volume":null,"pages":null},"PeriodicalIF":3.9,"publicationDate":"2024-07-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141758005","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}
Pub Date : 2024-07-21DOI: 10.1080/1744666X.2024.2377616
Niels Roelof Franciscus Sluijpers, Sarah Pringle, Hendrika Bootsma, Frederik Karst Lucien Spijkervet, Arjan Vissink, Konstantina Delli
Introduction: The complex nature of Sjögren's Disease (SjD) necessitates a comprehensive and patient-centered approach in both diagnosis and management. This narrative review emphasizes the need for a holistic understanding of the connection between salivary gland inflammation and oral symptoms in SjD.
Areas covered: The intricate relationship between salivary gland inflammation and dry mouth is explored, highlighting the variability in associations reported in studies. The association of the severity of xerostomia and degree of inflammation is also discussed. The frequent presence of recurrent sialadenitis in SjD further accentuates the connection of compromised salivary gland function and inflammation. The review additionally discusses local inflammatory factors assessed through salivary gland biopsies, which could potentially serve as predictors for lymphoma development in SjD. Insights into compromised quality of life and hypercoagulable state and their association with salivary gland inflammations are provided. Advancements in noninvasive imaging techniques, particularly salivary gland ultrasonography and color Doppler ultrasound, offer promising avenues for noninvasive assessment of inflammation.
Expert opinion: There is a need for longitudinal studies to unravel the connections between salivary gland inflammation and oral symptoms. This will enhance management strategies and optimize treatment outcomes for SjD patients.
{"title":"Connecting salivary gland inflammation to specific symptoms in Sjögren's disease.","authors":"Niels Roelof Franciscus Sluijpers, Sarah Pringle, Hendrika Bootsma, Frederik Karst Lucien Spijkervet, Arjan Vissink, Konstantina Delli","doi":"10.1080/1744666X.2024.2377616","DOIUrl":"10.1080/1744666X.2024.2377616","url":null,"abstract":"<p><strong>Introduction: </strong>The complex nature of Sjögren's Disease (SjD) necessitates a comprehensive and patient-centered approach in both diagnosis and management. This narrative review emphasizes the need for a holistic understanding of the connection between salivary gland inflammation and oral symptoms in SjD.</p><p><strong>Areas covered: </strong>The intricate relationship between salivary gland inflammation and dry mouth is explored, highlighting the variability in associations reported in studies. The association of the severity of xerostomia and degree of inflammation is also discussed. The frequent presence of recurrent sialadenitis in SjD further accentuates the connection of compromised salivary gland function and inflammation. The review additionally discusses local inflammatory factors assessed through salivary gland biopsies, which could potentially serve as predictors for lymphoma development in SjD. Insights into compromised quality of life and hypercoagulable state and their association with salivary gland inflammations are provided. Advancements in noninvasive imaging techniques, particularly salivary gland ultrasonography and color Doppler ultrasound, offer promising avenues for noninvasive assessment of inflammation.</p><p><strong>Expert opinion: </strong>There is a need for longitudinal studies to unravel the connections between salivary gland inflammation and oral symptoms. This will enhance management strategies and optimize treatment outcomes for SjD patients.</p>","PeriodicalId":12175,"journal":{"name":"Expert Review of Clinical Immunology","volume":null,"pages":null},"PeriodicalIF":3.9,"publicationDate":"2024-07-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141554496","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}
Introduction: Intravenous immunoglobulin is the standard of care in Kawasaki disease. However, a subset of patients exhibits resistance to intravenous immunoglobulin treatment, even when Kawasaki disease is promptly diagnosed and managed. While intravenous immunoglobulin reduces the occurrence of coronary artery abnormalities from 15-25% to 3-5%, it does not entirely eliminate the risk. Besides, management guidelines for non-coronary complications of Kawasaki disease, for instance, myocarditis, remain speculative.
Areas covered: Recent literature suggests that a subset of patients with Kawasaki disease may benefit from treatment intensification with drugs, such as corticosteroids, infliximab, anakinra, and/or ciclosporin. In this manuscript, we have reviewed recent advances in the management of Kawasaki disease, especially with regard to preemptive intensification of therapy in children at high risk of cardiac complications. A comprehensive search was made using Web of Science, Scopus, and PubMed databases to gather English articles published from 1967 to 2023 on the treatment of Kawasaki disease. We incorporated the following words in the search strategy: 'Kawasaki disease,' 'intravenous immunoglobulin/IVIg,' 'intravenous immunoglobulin/IVIg-resistant Kawasaki disease,' 'treatment intensification,' or 'primary intensification of treatment/therapy.'
Expert opinion: The 'high-risk' group in Kawasaki disease needs to be identified with early intensification of primary therapy for better coronary and myocardial outcomes.
简介静脉注射免疫球蛋白是治疗川崎病的标准方法。然而,即使川崎病得到及时诊断和治疗,仍有一部分患者对静脉注射免疫球蛋白治疗表现出耐药性。虽然静脉注射免疫球蛋白可将冠状动脉异常的发生率从 15-25% 降至 3-5%,但并不能完全消除风险。此外,针对川崎病的非冠状动脉并发症(如心肌炎)的治疗指南仍处于推测阶段:最近的文献表明,一部分川崎病患者可能受益于皮质类固醇、英夫利昔单抗、阿那曲林和/或环孢素等药物的强化治疗。在这篇手稿中,我们回顾了川崎病治疗的最新进展,尤其是对有心脏并发症高风险的儿童进行先期强化治疗方面的进展。我们使用 Web of Science、Scopus 和 PubMed 数据库进行了全面检索,收集了 1967 年至 2023 年间发表的有关川崎病治疗的英文文章。我们在检索策略中加入了以下词条:"川崎病"、"静脉注射免疫球蛋白/IVIg"、"静脉注射免疫球蛋白/IVIg耐药川崎病"、"强化治疗 "或 "初级强化治疗/疗法":需要识别川崎病的 "高危 "人群,及早加强初级治疗,以改善冠状动脉和心肌的预后。
{"title":"Treatment intensification in Kawasaki disease - current perspectives.","authors":"Prabal Barman, Rakesh Kumar Pilania, Gayathri Cv, Abarna Thangaraj, Munish Arora, Surjit Singh","doi":"10.1080/1744666X.2024.2378900","DOIUrl":"10.1080/1744666X.2024.2378900","url":null,"abstract":"<p><strong>Introduction: </strong>Intravenous immunoglobulin is the standard of care in Kawasaki disease. However, a subset of patients exhibits resistance to intravenous immunoglobulin treatment, even when Kawasaki disease is promptly diagnosed and managed. While intravenous immunoglobulin reduces the occurrence of coronary artery abnormalities from 15-25% to 3-5%, it does not entirely eliminate the risk. Besides, management guidelines for non-coronary complications of Kawasaki disease, for instance, myocarditis, remain speculative.</p><p><strong>Areas covered: </strong>Recent literature suggests that a subset of patients with Kawasaki disease may benefit from treatment intensification with drugs, such as corticosteroids, infliximab, anakinra, and/or ciclosporin. In this manuscript, we have reviewed recent advances in the management of Kawasaki disease, especially with regard to preemptive intensification of therapy in children at high risk of cardiac complications. A comprehensive search was made using Web of Science, Scopus, and PubMed databases to gather English articles published from 1967 to 2023 on the treatment of Kawasaki disease. We incorporated the following words in the search strategy: 'Kawasaki disease,' 'intravenous immunoglobulin/IVIg,' 'intravenous immunoglobulin/IVIg-resistant Kawasaki disease,' 'treatment intensification,' or 'primary intensification of treatment/therapy.'</p><p><strong>Expert opinion: </strong>The 'high-risk' group in Kawasaki disease needs to be identified with early intensification of primary therapy for better coronary and myocardial outcomes.</p>","PeriodicalId":12175,"journal":{"name":"Expert Review of Clinical Immunology","volume":null,"pages":null},"PeriodicalIF":3.9,"publicationDate":"2024-07-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141558437","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}
Pub Date : 2024-07-12DOI: 10.1080/1744666X.2024.2368195
Federica Pulvirenti, Annalisa Villa, Matteo D'Ambrosi, Gabriella Cusa, Patricia Quijada-Morales, Eduardo de la Fuente-Munoz, Maddalena Sciannamea, Giulia Garzi, Isabella Quinti
Background: Personalized medicine requires the assessment of the impact of health care interventions on Health-Related Quality of Life.
Research design and methods: We run an observational study of HRQoL in 140 CVID patients with biannual assessments over 8 years using a disease-specific tool, the CVID_QoL, and the GHQ questionnaires. Factors influencing changes in HRQoL scores were identified using multiple linear regression models with a stepwise procedure.
Results: Infections frequency, female gender, and chronic enteropathy were associated with worse global CVID_QoL scores. The presence of permanent organ damage and older age contributed to the perception of being at risk of health deterioration, while chronic enteropathy was associated with fatigue. The presence of permanent organ damage was also associated with perceived difficulties in usual activities. The frequency of infections was the main risk factor for difficulties in long-term planning and perceptions of vulnerability. Before COVID-19, improved HRQoL scores were associated with reduced respiratory infections and changes in immunoglobulin replacement route and setting. The COVID-19 pandemic caused a sudden deterioration in all HRQoL dimensions, and a further deterioration in the emotional dimension was observed during the pandemic period. Patients who died during the study had worse CVID_QoL scores at all time points, confirming that HRQoL performance is strongly related to patient outcome.
Conclusions: Periodic HRQoL assessments are needed to capture relevant issues that change over time in patients affected by long-term chronic conditions such CVID, possibly identifying areas of intervention.
{"title":"Changes in health-related quality of life in common variable immunodeficiency: an eight-year journey, including the COVID-19 pandemic.","authors":"Federica Pulvirenti, Annalisa Villa, Matteo D'Ambrosi, Gabriella Cusa, Patricia Quijada-Morales, Eduardo de la Fuente-Munoz, Maddalena Sciannamea, Giulia Garzi, Isabella Quinti","doi":"10.1080/1744666X.2024.2368195","DOIUrl":"https://doi.org/10.1080/1744666X.2024.2368195","url":null,"abstract":"<p><strong>Background: </strong>Personalized medicine requires the assessment of the impact of health care interventions on Health-Related Quality of Life.</p><p><strong>Research design and methods: </strong>We run an observational study of HRQoL in 140 CVID patients with biannual assessments over 8 years using a disease-specific tool, the CVID_QoL, and the GHQ questionnaires. Factors influencing changes in HRQoL scores were identified using multiple linear regression models with a stepwise procedure.</p><p><strong>Results: </strong>Infections frequency, female gender, and chronic enteropathy were associated with worse global CVID_QoL scores. The presence of permanent organ damage and older age contributed to the perception of being at risk of health deterioration, while chronic enteropathy was associated with fatigue. The presence of permanent organ damage was also associated with perceived difficulties in usual activities. The frequency of infections was the main risk factor for difficulties in long-term planning and perceptions of vulnerability. Before COVID-19, improved HRQoL scores were associated with reduced respiratory infections and changes in immunoglobulin replacement route and setting. The COVID-19 pandemic caused a sudden deterioration in all HRQoL dimensions, and a further deterioration in the emotional dimension was observed during the pandemic period. Patients who died during the study had worse CVID_QoL scores at all time points, confirming that HRQoL performance is strongly related to patient outcome.</p><p><strong>Conclusions: </strong>Periodic HRQoL assessments are needed to capture relevant issues that change over time in patients affected by long-term chronic conditions such CVID, possibly identifying areas of intervention.</p>","PeriodicalId":12175,"journal":{"name":"Expert Review of Clinical Immunology","volume":null,"pages":null},"PeriodicalIF":3.9,"publicationDate":"2024-07-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141589972","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}
Pub Date : 2024-07-02DOI: 10.1080/1744666X.2024.2373886
Marcela Santaolalla, José Arias-Irigoyen, Jose Miguel Soler, José María Duque, Rosario Escudero, José Luis Pérez-Formoso, Teófilo Lobera, María Rueda, César Alias, Helena Hermida, Catalina Vela, Leire Begoña, Alexander Vazquez, Begoña Madariaga
Background: Administration of allergen mixtures of many components comprises the most common approach for American allergists regarding the management of polyallergic patients. European allergists, however, are more reluctant to this type of treatment due to the potential drawbacks of mixing extracts.
Research design and methods: To assess the efficacy and safety of subcutaneous immunotherapy (SCIT) with polymerized allergen mixtures without dilutional effect in polyallergic patients.This observational, prospective, multicenter study included patients (between 5 and 60 years) with respiratory allergic diseases that had been prescribed with SCIT with mixtures of two pollen or mite extracts. Changes in Symptoms and Medication Score (SMS) and in rhinitis quality of life questionnaire (RQLQ), subjective clinical improvement, treatment satisfaction and tolerability were assessed after the 1-year treatment.
Results: A total of 115 patients were included in the assessment. Mean global SMS decreased from 3.5 (SD = 1.1) to 1.6 (SD = 1.2) points, with a mean absolute reduction of 1.6 (SD = 1.3) points in the RQLQ score (p < 0.001, Wilcoxon test). General subjective clinical improvements and a good treatment satisfaction and tolerability were observed.
Conclusion: SCIT with polymerized allergen mixtures from either pollen or mite extracts proved to be an effective and safe treatment option for polyallergic patients suffering from allergic respiratory diseases.
{"title":"Efficacy and safety of subcutaneous immunotherapy with polymerized allergen mixtures in polyallergic patients - ARES observational study.","authors":"Marcela Santaolalla, José Arias-Irigoyen, Jose Miguel Soler, José María Duque, Rosario Escudero, José Luis Pérez-Formoso, Teófilo Lobera, María Rueda, César Alias, Helena Hermida, Catalina Vela, Leire Begoña, Alexander Vazquez, Begoña Madariaga","doi":"10.1080/1744666X.2024.2373886","DOIUrl":"10.1080/1744666X.2024.2373886","url":null,"abstract":"<p><strong>Background: </strong>Administration of allergen mixtures of many components comprises the most common approach for American allergists regarding the management of polyallergic patients. European allergists, however, are more reluctant to this type of treatment due to the potential drawbacks of mixing extracts.</p><p><strong>Research design and methods: </strong>To assess the efficacy and safety of subcutaneous immunotherapy (SCIT) with polymerized allergen mixtures without dilutional effect in polyallergic patients.This observational, prospective, multicenter study included patients (between 5 and 60 years) with respiratory allergic diseases that had been prescribed with SCIT with mixtures of two pollen or mite extracts. Changes in Symptoms and Medication Score (SMS) and in rhinitis quality of life questionnaire (RQLQ), subjective clinical improvement, treatment satisfaction and tolerability were assessed after the 1-year treatment.</p><p><strong>Results: </strong>A total of 115 patients were included in the assessment. Mean global SMS decreased from 3.5 (SD = 1.1) to 1.6 (SD = 1.2) points, with a mean absolute reduction of 1.6 (SD = 1.3) points in the RQLQ score (<i>p</i> < 0.001, Wilcoxon test). General subjective clinical improvements and a good treatment satisfaction and tolerability were observed.</p><p><strong>Conclusion: </strong>SCIT with polymerized allergen mixtures from either pollen or mite extracts proved to be an effective and safe treatment option for polyallergic patients suffering from allergic respiratory diseases.</p>","PeriodicalId":12175,"journal":{"name":"Expert Review of Clinical Immunology","volume":null,"pages":null},"PeriodicalIF":3.9,"publicationDate":"2024-07-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141456145","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}
Pub Date : 2024-07-02DOI: 10.1080/1744666X.2024.2372335
Niloufar Yazdanpanah, Nima Rezaei
Introduction: Congenital immunodeficiency is named primary immunodeficiency (PID), and more recently inborn errors of immunity (IEI). There are more than 485 conditions classified as IEI, with a wide spectrum of clinical and laboratory manifestations.
Areas covered: Regardless of the developing knowledge of IEI, many physicians do not think of IEI when approaching the patient's complaint, which leads to delayed diagnosis, misdiagnosis, serious infectious and noninfectious complications, permanent end-organ damage, and even death. Due to the various manifestations of IEI and the wide spectrum of associated conditions, patients refer to specialists in different disciplines of medicine and undergo - mainly symptomatic - treatments, and because IEI are not included in physicians' differential diagnosis, the main disease remains undiagnosed.
Expert opinion: A multidisciplinary approach may be a proper solution. Manifestations and the importance of a multidisciplinary approach in the diagnosis of main groups of IEI are discussed in this article.
{"title":"The multidisciplinary approach to diagnosing inborn errors of immunity: a comprehensive review of discipline-based manifestations.","authors":"Niloufar Yazdanpanah, Nima Rezaei","doi":"10.1080/1744666X.2024.2372335","DOIUrl":"10.1080/1744666X.2024.2372335","url":null,"abstract":"<p><strong>Introduction: </strong>Congenital immunodeficiency is named primary immunodeficiency (PID), and more recently inborn errors of immunity (IEI). There are more than 485 conditions classified as IEI, with a wide spectrum of clinical and laboratory manifestations.</p><p><strong>Areas covered: </strong>Regardless of the developing knowledge of IEI, many physicians do not think of IEI when approaching the patient's complaint, which leads to delayed diagnosis, misdiagnosis, serious infectious and noninfectious complications, permanent end-organ damage, and even death. Due to the various manifestations of IEI and the wide spectrum of associated conditions, patients refer to specialists in different disciplines of medicine and undergo - mainly symptomatic - treatments, and because IEI are not included in physicians' differential diagnosis, the main disease remains undiagnosed.</p><p><strong>Expert opinion: </strong>A multidisciplinary approach may be a proper solution. Manifestations and the importance of a multidisciplinary approach in the diagnosis of main groups of IEI are discussed in this article.</p>","PeriodicalId":12175,"journal":{"name":"Expert Review of Clinical Immunology","volume":null,"pages":null},"PeriodicalIF":3.9,"publicationDate":"2024-07-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141440379","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}