Pub Date : 2025-01-27DOI: 10.1186/s13223-024-00925-4
Persia Pourshahnazari, Stephen D Betschel, Vy H D Kim, Susan Waserman, Rongbo Zhu, Harold Kim
The field of medicine is constantly changing and, as healthcare providers, we are fortunate to be practicing in a time when patients are living longer and novel therapeutic options continue to evolve. However, these new advances may be associated with adverse effects that practitioners need to be aware of. Some of these impair the immune system leading to secondary immunodeficiencies (SID) that increase host susceptibility to infections and other complications. The causes and consequences of these SID are extremely broad, and a detailed review is beyond the scope of this article. The goal of this primer is to provide a general overview and understanding of common conditions and therapies leading to SID, as well as a guide to the assessment and management of patients with SID.
{"title":"Secondary Immunodeficiency.","authors":"Persia Pourshahnazari, Stephen D Betschel, Vy H D Kim, Susan Waserman, Rongbo Zhu, Harold Kim","doi":"10.1186/s13223-024-00925-4","DOIUrl":"10.1186/s13223-024-00925-4","url":null,"abstract":"<p><p>The field of medicine is constantly changing and, as healthcare providers, we are fortunate to be practicing in a time when patients are living longer and novel therapeutic options continue to evolve. However, these new advances may be associated with adverse effects that practitioners need to be aware of. Some of these impair the immune system leading to secondary immunodeficiencies (SID) that increase host susceptibility to infections and other complications. The causes and consequences of these SID are extremely broad, and a detailed review is beyond the scope of this article. The goal of this primer is to provide a general overview and understanding of common conditions and therapies leading to SID, as well as a guide to the assessment and management of patients with SID.</p>","PeriodicalId":51302,"journal":{"name":"Allergy Asthma and Clinical Immunology","volume":"20 Suppl 3","pages":"80"},"PeriodicalIF":2.6,"publicationDate":"2025-01-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11773690/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143054064","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2025-01-27DOI: 10.1186/s13223-024-00944-1
Harold Kim, Anne K Ellis, Wade Watson
{"title":"This supplement is dedicated to the late Dr. Richard Warrington.","authors":"Harold Kim, Anne K Ellis, Wade Watson","doi":"10.1186/s13223-024-00944-1","DOIUrl":"10.1186/s13223-024-00944-1","url":null,"abstract":"","PeriodicalId":51302,"journal":{"name":"Allergy Asthma and Clinical Immunology","volume":"20 Suppl 3","pages":"79"},"PeriodicalIF":2.6,"publicationDate":"2025-01-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11773688/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143054065","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2025-01-22DOI: 10.1186/s13223-025-00951-w
Yiwei Yin, Moshe Ben Shoshan, Marcus Shaker, Matthew Greenhawt, Kate M Johnson
Background: Until recently, immediate emergency department (ED) transfer after food-related anaphylactic reactions was recommended regardless of symptom resolution following use of an epinephrine autoinjector (EAI). We evaluated the cost-effectiveness of delayed ED transfer after EAI use in non-medical settings (watchful waiting) compared to immediate ED transfer among pediatric patients with food allergies in Canada.
Methods: We developed a probabilistic Markov model of individuals starting at age of one year who are at risk of severe food-related allergic reactions requiring epinephrine. We evaluated medical costs (in 2022 Canadian dollars) and quality-adjusted life years (QALY) of each strategy over a 20-year horizon. In the base case, we assumed a tenfold increase in food allergy fatality for patients under watchful waiting, which we increased to 100- to 1,000-fold in sensitivity analysis. The analysis was conducted from the Canadian healthcare system perspective with a 1.5% annual discount rate and a willingness-to-pay (WTP) threshold of $50,000 per QALY.
Results: Immediate ED transfer following EAI use resulted in a decreased risk of food allergy fatality of 9.2 × 10- 5 over 20 years, which is equivalent to < 1 fatality per 200,000 patient-years. Watchful waiting resulted in cost savings of $1,157 per patient and a QALY loss of 7.28 × 10- 4; an incremental cost per QALY saved of $1,589,854. The incremental cost per death prevented with immediate ED transfer was $12,586,613. Watchful waiting remained cost-effective in all sensitivity and scenario analyses, except under extreme increases in fatality risk of 500-fold and 1,000-fold.
Conclusions: Watchful waiting for symptom re-occurrence following EAI administration in non-medical settings is cost-effective.
{"title":"Cost-effectiveness of watchful waiting versus immediate emergency department transfer after epinephrine autoinjector use in Canada.","authors":"Yiwei Yin, Moshe Ben Shoshan, Marcus Shaker, Matthew Greenhawt, Kate M Johnson","doi":"10.1186/s13223-025-00951-w","DOIUrl":"10.1186/s13223-025-00951-w","url":null,"abstract":"<p><strong>Background: </strong>Until recently, immediate emergency department (ED) transfer after food-related anaphylactic reactions was recommended regardless of symptom resolution following use of an epinephrine autoinjector (EAI). We evaluated the cost-effectiveness of delayed ED transfer after EAI use in non-medical settings (watchful waiting) compared to immediate ED transfer among pediatric patients with food allergies in Canada.</p><p><strong>Methods: </strong>We developed a probabilistic Markov model of individuals starting at age of one year who are at risk of severe food-related allergic reactions requiring epinephrine. We evaluated medical costs (in 2022 Canadian dollars) and quality-adjusted life years (QALY) of each strategy over a 20-year horizon. In the base case, we assumed a tenfold increase in food allergy fatality for patients under watchful waiting, which we increased to 100- to 1,000-fold in sensitivity analysis. The analysis was conducted from the Canadian healthcare system perspective with a 1.5% annual discount rate and a willingness-to-pay (WTP) threshold of $50,000 per QALY.</p><p><strong>Results: </strong>Immediate ED transfer following EAI use resulted in a decreased risk of food allergy fatality of 9.2 × 10<sup>- 5</sup> over 20 years, which is equivalent to < 1 fatality per 200,000 patient-years. Watchful waiting resulted in cost savings of $1,157 per patient and a QALY loss of 7.28 × 10<sup>- 4</sup>; an incremental cost per QALY saved of $1,589,854. The incremental cost per death prevented with immediate ED transfer was $12,586,613. Watchful waiting remained cost-effective in all sensitivity and scenario analyses, except under extreme increases in fatality risk of 500-fold and 1,000-fold.</p><p><strong>Conclusions: </strong>Watchful waiting for symptom re-occurrence following EAI administration in non-medical settings is cost-effective.</p>","PeriodicalId":51302,"journal":{"name":"Allergy Asthma and Clinical Immunology","volume":"21 1","pages":"5"},"PeriodicalIF":2.6,"publicationDate":"2025-01-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11755952/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143025719","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2025-01-22DOI: 10.1186/s13223-024-00936-1
Samira Jeimy, Tiffany Wong, Moshe Ben-Shoshan, Ana Maria Copaescu, Ghislaine A C Isabwe, Anne K Ellis
Drug allergy encompasses a spectrum of immunologically-mediated hypersensitivity reactions (HSRs) with varying mechanisms and clinical presentations. This type of adverse drug reaction (ADR) not only affects patient quality of life, but may also lead to delayed treatment, unnecessary investigations, and increased morbidity and mortality. Given the spectrum of symptoms associated with the condition, diagnosis can be challenging. Therefore, referral to an allergist experienced in the diagnosis and management of drug allergy is recommended if a drug-induced allergic reaction is suspected. Diagnosis relies on a careful history and physical examination and, in some instances, skin testing or in vitro testing and drug challenges. The most effective strategy for the management of allergist-confirmed drug allergy is avoidance or discontinuation of the offending drug. When available, alternative medications with unrelated chemical structures should be substituted. Cross-reactivity among drugs should also be taken into consideration when choosing alternative agents. Additional therapy for drug HSRs may include topical corticosteroids, oral antihistamines and, in severe cases, systemic corticosteroids and other immunomodulators. In the event of anaphylaxis, the treatment of choice is intramuscular epinephrine. If a patient with a history of anaphylaxis requires a specific drug and there is no acceptable alternative, desensitization to that drug may be considered. This article provides a background on drug allergy and strategies for the diagnosis and management of some of the most common drug-induced allergic reactions.
{"title":"Drug allergy.","authors":"Samira Jeimy, Tiffany Wong, Moshe Ben-Shoshan, Ana Maria Copaescu, Ghislaine A C Isabwe, Anne K Ellis","doi":"10.1186/s13223-024-00936-1","DOIUrl":"10.1186/s13223-024-00936-1","url":null,"abstract":"<p><p>Drug allergy encompasses a spectrum of immunologically-mediated hypersensitivity reactions (HSRs) with varying mechanisms and clinical presentations. This type of adverse drug reaction (ADR) not only affects patient quality of life, but may also lead to delayed treatment, unnecessary investigations, and increased morbidity and mortality. Given the spectrum of symptoms associated with the condition, diagnosis can be challenging. Therefore, referral to an allergist experienced in the diagnosis and management of drug allergy is recommended if a drug-induced allergic reaction is suspected. Diagnosis relies on a careful history and physical examination and, in some instances, skin testing or in vitro testing and drug challenges. The most effective strategy for the management of allergist-confirmed drug allergy is avoidance or discontinuation of the offending drug. When available, alternative medications with unrelated chemical structures should be substituted. Cross-reactivity among drugs should also be taken into consideration when choosing alternative agents. Additional therapy for drug HSRs may include topical corticosteroids, oral antihistamines and, in severe cases, systemic corticosteroids and other immunomodulators. In the event of anaphylaxis, the treatment of choice is intramuscular epinephrine. If a patient with a history of anaphylaxis requires a specific drug and there is no acceptable alternative, desensitization to that drug may be considered. This article provides a background on drug allergy and strategies for the diagnosis and management of some of the most common drug-induced allergic reactions.</p>","PeriodicalId":51302,"journal":{"name":"Allergy Asthma and Clinical Immunology","volume":"20 Suppl 3","pages":"78"},"PeriodicalIF":2.6,"publicationDate":"2025-01-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11755868/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143025721","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Background: The incidence of allergic diseases has been increasing in Japan. In particular, a serious decline in the age of onset of allergic rhinitis has been observed. Passive smoking from parental smoking has a significant impact on children's health; however, it is difficult to restrict smoking in the home. While various studies have previously reported on the relationship between passive smoking and the development of allergic diseases in children. However, there have been no reports on passive smoking and allergic diseases on a national scale.
Methods: Using Japanese national longitudinal survey data (n = 38,444) for newborns born between May 10 and 24, 2010, we assessed parental smoking habits when their children were 6 months old and investigated the association with the development of allergic diseases until the age of 5.5 years. The risk ratios and 95% confidence intervals for the development of different allergic diseases were analyzed after adjusting for potential confounders using Poisson regression with a robust error variance.
Results: The risk ratio for developing allergic rhinitis/allergic conjunctivitis (AR/AC) in children was significantly higher in the maternal smoking groups ( ≦ 10 cigarettes/day; RR 1.15, 95% CI 1.02-1.30; ≧11 cigarettes/day; RR 1.16, 95% CI 0.93-1.44). Furthermore, associations were found between the maternal smoking group in the presence of paternal smoking and the risk of developing bronchial asthma ( ≦ 10, RR 1.33 95% CI 1.17-1.52; ≧11, RR 1.71 95% CI 1.38-2.1), food allergy ( ≦ 10, RR 1.36 95% CI 1.12-1.63; ≧11, RR 1.25 95% CI 0.84-1.86), atopic dermatitis ( ≦ 10, RR 1.42 95% CI 1.22-1.66; ≧11, RR 1.6 95% CI 1.2-2.13), and AR/AC ( ≦ 10, RR 1.21 95% CI 1.07-1.36; ≧11, RR 1.35 95% CI 1.09-1.67).
Conclusions: Maternal smoking during infancy increases the risk of developing AR/AC in children. Considering paternal smoking, maternal smoking further increased the risk of developing allergic diseases in children, suggesting that reducing parental smoking at home may reduce the risk of developing allergic diseases in children.
背景:日本变态反应性疾病的发病率呈上升趋势。特别是,已经观察到过敏性鼻炎发病年龄的严重下降。父母吸烟造成的被动吸烟对儿童健康有重大影响;然而,在家里限制吸烟是很困难的。虽然之前有各种研究报告了被动吸烟与儿童过敏性疾病发展之间的关系。然而,在全国范围内尚无关于被动吸烟和过敏性疾病的报道。方法:利用2010年5月10日至24日出生的日本全国纵向调查数据(n = 38,444),评估父母在孩子6个月大时的吸烟习惯,并调查其在5.5岁之前与过敏性疾病发展的关系。在校正了潜在混杂因素后,使用误差方差较强的泊松回归分析不同变态反应性疾病发生的风险比和95%置信区间。结果:母亲吸烟组儿童发生变应性鼻炎/变应性结膜炎(AR/AC)的风险比明显高于母亲吸烟组(≦10支/天;Rr 1.15, 95% ci 1.02-1.30;≧11香烟/天;Rr 1.16, 95% ci 0.93-1.44)。此外,在父亲吸烟的情况下,母亲吸烟组与患支气管哮喘的风险相关(≦10,RR 1.33 95% CI 1.17-1.52;≧11,RR 1.71 95% CI 1.38-2.1),食物过敏(≦10,RR 1.36 95% CI 1.12-1.63;≧11,RR 1.25 95% CI 0.84-1.86),特应性皮炎(≦10,RR 1.42 95% CI 1.22-1.66;≧11,RR 1.6 95% CI 1.2-2.13), AR/AC(≦10,RR 1.21 95% CI 1.07-1.36);≧11,rr 1.35 (95% ci 1.09-1.67)。结论:母亲在婴儿期吸烟会增加儿童发生AR/AC的风险。考虑到父亲吸烟,母亲吸烟进一步增加了儿童发生过敏性疾病的风险,提示减少父母在家吸烟可能会降低儿童发生过敏性疾病的风险。
{"title":"Maternal smoking during infancy increases the risk of allergic diseases in children: a nationwide longitudinal survey in Japan.","authors":"Kenji Shigehara, Naomi Matsumoto, Mitsuru Tsuge, Kazuhiro Uda, Yukie Saito, Masato Yashiro, Takashi Yorifuji, Masanori Ikeda, Hirokazu Tsukahara","doi":"10.1186/s13223-025-00952-9","DOIUrl":"https://doi.org/10.1186/s13223-025-00952-9","url":null,"abstract":"<p><strong>Background: </strong>The incidence of allergic diseases has been increasing in Japan. In particular, a serious decline in the age of onset of allergic rhinitis has been observed. Passive smoking from parental smoking has a significant impact on children's health; however, it is difficult to restrict smoking in the home. While various studies have previously reported on the relationship between passive smoking and the development of allergic diseases in children. However, there have been no reports on passive smoking and allergic diseases on a national scale.</p><p><strong>Methods: </strong>Using Japanese national longitudinal survey data (n = 38,444) for newborns born between May 10 and 24, 2010, we assessed parental smoking habits when their children were 6 months old and investigated the association with the development of allergic diseases until the age of 5.5 years. The risk ratios and 95% confidence intervals for the development of different allergic diseases were analyzed after adjusting for potential confounders using Poisson regression with a robust error variance.</p><p><strong>Results: </strong>The risk ratio for developing allergic rhinitis/allergic conjunctivitis (AR/AC) in children was significantly higher in the maternal smoking groups ( ≦ 10 cigarettes/day; RR 1.15, 95% CI 1.02-1.30; ≧11 cigarettes/day; RR 1.16, 95% CI 0.93-1.44). Furthermore, associations were found between the maternal smoking group in the presence of paternal smoking and the risk of developing bronchial asthma ( ≦ 10, RR 1.33 95% CI 1.17-1.52; ≧11, RR 1.71 95% CI 1.38-2.1), food allergy ( ≦ 10, RR 1.36 95% CI 1.12-1.63; ≧11, RR 1.25 95% CI 0.84-1.86), atopic dermatitis ( ≦ 10, RR 1.42 95% CI 1.22-1.66; ≧11, RR 1.6 95% CI 1.2-2.13), and AR/AC ( ≦ 10, RR 1.21 95% CI 1.07-1.36; ≧11, RR 1.35 95% CI 1.09-1.67).</p><p><strong>Conclusions: </strong>Maternal smoking during infancy increases the risk of developing AR/AC in children. Considering paternal smoking, maternal smoking further increased the risk of developing allergic diseases in children, suggesting that reducing parental smoking at home may reduce the risk of developing allergic diseases in children.</p>","PeriodicalId":51302,"journal":{"name":"Allergy Asthma and Clinical Immunology","volume":"21 1","pages":"4"},"PeriodicalIF":2.6,"publicationDate":"2025-01-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11740415/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143015905","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2025-01-13DOI: 10.1186/s13223-024-00943-2
Harold Kim, Anne K Ellis, Wade Watson
{"title":"Introduction from the editors.","authors":"Harold Kim, Anne K Ellis, Wade Watson","doi":"10.1186/s13223-024-00943-2","DOIUrl":"10.1186/s13223-024-00943-2","url":null,"abstract":"","PeriodicalId":51302,"journal":{"name":"Allergy Asthma and Clinical Immunology","volume":"20 Suppl 3","pages":"77"},"PeriodicalIF":2.6,"publicationDate":"2025-01-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11726953/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142980634","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2025-01-12DOI: 10.1186/s13223-024-00945-0
Edyta Krzych-Fałta, Andrzej Namysłowski, Sławomir Białek, Monika E Czerwińska, Konrad Furmańczyk, Aleksandra Tylewicz, Adam Sybilski, Bolesław Samoliński, Oksana Wojas
Background: Nasal allergen provocation tests are an important part of the diagnostics of allergic diseases triggered by environmental factors. Recently, increased attention has been paid to the potential use of this method in the diagnosis of food allergy. The objective of the study was to evaluate the usefulness of the nasal allergen provocation test in a group of subjects allergic to hen's egg white allergens.
Methods: The material consisted of a group of 57 subjects (32 subjects with hen's egg white allergy and 25 healthy controls). The method consisted in a nasal allergen provocation test carried out with the use of hen's egg white allergen and assessed using the visual analog scale and optical rhinometry as well as by determination of sIgE and tryptase levels in nasal lavage fluid.
Results: Subjective nasal symptoms and objective evaluations following the application of 100 µg of hen's egg white allergen revealed a moderately positive nasal mucosal response in optical rhinometry tests (ΔE = 0.34 OD).
Conclusions: Nasal food challenge with hen's egg white allergen is a good diagnostic alternative in the group of food allergy patients. Due to the insufficient number of studies carried out so far, further attempts at standardization of the method are required.
{"title":"Nasal food challenge with hen's egg white allergen.","authors":"Edyta Krzych-Fałta, Andrzej Namysłowski, Sławomir Białek, Monika E Czerwińska, Konrad Furmańczyk, Aleksandra Tylewicz, Adam Sybilski, Bolesław Samoliński, Oksana Wojas","doi":"10.1186/s13223-024-00945-0","DOIUrl":"10.1186/s13223-024-00945-0","url":null,"abstract":"<p><strong>Background: </strong>Nasal allergen provocation tests are an important part of the diagnostics of allergic diseases triggered by environmental factors. Recently, increased attention has been paid to the potential use of this method in the diagnosis of food allergy. The objective of the study was to evaluate the usefulness of the nasal allergen provocation test in a group of subjects allergic to hen's egg white allergens.</p><p><strong>Methods: </strong>The material consisted of a group of 57 subjects (32 subjects with hen's egg white allergy and 25 healthy controls). The method consisted in a nasal allergen provocation test carried out with the use of hen's egg white allergen and assessed using the visual analog scale and optical rhinometry as well as by determination of sIgE and tryptase levels in nasal lavage fluid.</p><p><strong>Results: </strong>Subjective nasal symptoms and objective evaluations following the application of 100 µg of hen's egg white allergen revealed a moderately positive nasal mucosal response in optical rhinometry tests (ΔE = 0.34 OD).</p><p><strong>Conclusions: </strong>Nasal food challenge with hen's egg white allergen is a good diagnostic alternative in the group of food allergy patients. Due to the insufficient number of studies carried out so far, further attempts at standardization of the method are required.</p>","PeriodicalId":51302,"journal":{"name":"Allergy Asthma and Clinical Immunology","volume":"21 1","pages":"3"},"PeriodicalIF":2.6,"publicationDate":"2025-01-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11727154/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142973219","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2025-01-08DOI: 10.1186/s13223-024-00938-z
Vy H D Kim, Julia E M Upton, Beata Derfalvi, Kyla J Hildebrand, Christine McCusker
Primary immunodeficiencies (PID), now often referred to as inborn errors of immunity (IEI), are a large heterogeneous group of disorders that result from deficiencies in immune system development and/or function. IEIs can be broadly classified as disorders of adaptive immunity (e.g., combined or humoral immunodeficiencies) or of innate immunity (e.g., phagocyte and complement disorders). Although the clinical manifestations of IEIs are highly variable, traditionally many disorders involve an increased susceptibility to infection. Research in recent years has underscored how IEI can present with features other than infection such as: severe atopy, autoimmunity, autoinflammation, lymphoproliferation, and/or malignancy resulting from immune dysregulation. Early consultation with a clinical immunologist is essential, as timely diagnosis and treatment are imperative for preventing significant disease-associated morbidity and mortality. The treatment of IEIs is complex and generally requires both supportive and definitive strategies, including but not limited to, immunoglobulin replacement therapy, antibiotic prophylaxis, immune response modifiers, and hematopoietic stem cell transplantation. This article provides an overview of the major categories of IEIs and strategies for the appropriate diagnosis and management of these disorders.
{"title":"Inborn errors of immunity (primary immunodeficiencies).","authors":"Vy H D Kim, Julia E M Upton, Beata Derfalvi, Kyla J Hildebrand, Christine McCusker","doi":"10.1186/s13223-024-00938-z","DOIUrl":"10.1186/s13223-024-00938-z","url":null,"abstract":"<p><p>Primary immunodeficiencies (PID), now often referred to as inborn errors of immunity (IEI), are a large heterogeneous group of disorders that result from deficiencies in immune system development and/or function. IEIs can be broadly classified as disorders of adaptive immunity (e.g., combined or humoral immunodeficiencies) or of innate immunity (e.g., phagocyte and complement disorders). Although the clinical manifestations of IEIs are highly variable, traditionally many disorders involve an increased susceptibility to infection. Research in recent years has underscored how IEI can present with features other than infection such as: severe atopy, autoimmunity, autoinflammation, lymphoproliferation, and/or malignancy resulting from immune dysregulation. Early consultation with a clinical immunologist is essential, as timely diagnosis and treatment are imperative for preventing significant disease-associated morbidity and mortality. The treatment of IEIs is complex and generally requires both supportive and definitive strategies, including but not limited to, immunoglobulin replacement therapy, antibiotic prophylaxis, immune response modifiers, and hematopoietic stem cell transplantation. This article provides an overview of the major categories of IEIs and strategies for the appropriate diagnosis and management of these disorders.</p>","PeriodicalId":51302,"journal":{"name":"Allergy Asthma and Clinical Immunology","volume":"20 Suppl 3","pages":"76"},"PeriodicalIF":2.6,"publicationDate":"2025-01-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11714877/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142958544","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2025-01-07DOI: 10.1186/s13223-024-00939-y
Rajiv Mallick, Noemi Hahn, Christopher Scalchunes
Background: Immunoglobulin replacement therapy (IgRT) is the current standard of care for primary antibody deficiency patients (majority of all primary immunodeficiency (PID) diseases), with growing real-world evidence supporting use for secondary immunodeficiency (SID) patients. Infusion methods and practices can affect patients' satisfaction with their treatment and perception of their health-related quality of life.
Methods: An online survey of US patients with PID and SID was conducted. This research investigates primarily the impact of two IgRT infusion methods, intravenous immunoglobulin therapy (IVIG) and subcutaneous immunoglobulin (SCIG), on the patient reported outcome (PRO) Life Quality Index (LQI) tool. Patient reported infusion time efficiency, physical and mental health (PROMIS GPH-2 and PROMIS GMH-2 respectively), patient acceptability of their symptom state (PASS), upper extremity disability (Quick DASH) and general health perception (via the GHP) are also investigated.
Results: Responses of 990 patients (391 IVIG and 598 SCIG) were analyzed. The median total LQI score amongst SCIG patients (84.7) was higher than IVIG patients (81.9) (p < 0.001), and was significantly higher on 3 out of 4 sub-domains of the LQI. SCIG patients scored higher on items that are related to convenience and reported less interference with everyday life: "Are convenient", "Are scheduled according to my convenience", "Do not interfere with my work/school" and "Require very little time and cost". However, there was no significant difference between the two patient cohorts on other, non-IG specific PROs (PASS, PROMIS GPH-2 and GMH-2 and Quick DASH). Patient reported time per infusion was lower for SCIG infusions than IVIG infusions (pre-infusion time; 22 min vs. 63 min, p < 0.001, infusion time; 120 min vs. 240 min, p < 0.001, post-infusion time; 9 min vs. 31 min, p < 0.001). IVIG patients also reported more interference with everyday life than SCIG patients (82 vs. 86, p < 0.001).
Conclusions: The significantly higher LQI scores for patients receiving SCIG than those receiving IVIG confirms existing evidence that substitution of SCIG for IVIG may favorably impact immunoglobulin specific perceptions of quality of life and treatment satisfaction for appropriately selected patients. Our evidence on infusion times indicates similar improvement may be possible on infusion time efficiency.
{"title":"Immunoglobulin replacement therapy in patients with primary and secondary immunodeficiencies: impact of infusion method on immunoglobulin-specific perceptions of quality of life and treatment satisfaction.","authors":"Rajiv Mallick, Noemi Hahn, Christopher Scalchunes","doi":"10.1186/s13223-024-00939-y","DOIUrl":"https://doi.org/10.1186/s13223-024-00939-y","url":null,"abstract":"<p><strong>Background: </strong>Immunoglobulin replacement therapy (IgRT) is the current standard of care for primary antibody deficiency patients (majority of all primary immunodeficiency (PID) diseases), with growing real-world evidence supporting use for secondary immunodeficiency (SID) patients. Infusion methods and practices can affect patients' satisfaction with their treatment and perception of their health-related quality of life.</p><p><strong>Methods: </strong>An online survey of US patients with PID and SID was conducted. This research investigates primarily the impact of two IgRT infusion methods, intravenous immunoglobulin therapy (IVIG) and subcutaneous immunoglobulin (SCIG), on the patient reported outcome (PRO) Life Quality Index (LQI) tool. Patient reported infusion time efficiency, physical and mental health (PROMIS GPH-2 and PROMIS GMH-2 respectively), patient acceptability of their symptom state (PASS), upper extremity disability (Quick DASH) and general health perception (via the GHP) are also investigated.</p><p><strong>Results: </strong>Responses of 990 patients (391 IVIG and 598 SCIG) were analyzed. The median total LQI score amongst SCIG patients (84.7) was higher than IVIG patients (81.9) (p < 0.001), and was significantly higher on 3 out of 4 sub-domains of the LQI. SCIG patients scored higher on items that are related to convenience and reported less interference with everyday life: \"Are convenient\", \"Are scheduled according to my convenience\", \"Do not interfere with my work/school\" and \"Require very little time and cost\". However, there was no significant difference between the two patient cohorts on other, non-IG specific PROs (PASS, PROMIS GPH-2 and GMH-2 and Quick DASH). Patient reported time per infusion was lower for SCIG infusions than IVIG infusions (pre-infusion time; 22 min vs. 63 min, p < 0.001, infusion time; 120 min vs. 240 min, p < 0.001, post-infusion time; 9 min vs. 31 min, p < 0.001). IVIG patients also reported more interference with everyday life than SCIG patients (82 vs. 86, p < 0.001).</p><p><strong>Conclusions: </strong>The significantly higher LQI scores for patients receiving SCIG than those receiving IVIG confirms existing evidence that substitution of SCIG for IVIG may favorably impact immunoglobulin specific perceptions of quality of life and treatment satisfaction for appropriately selected patients. Our evidence on infusion times indicates similar improvement may be possible on infusion time efficiency.</p>","PeriodicalId":51302,"journal":{"name":"Allergy Asthma and Clinical Immunology","volume":"21 1","pages":"2"},"PeriodicalIF":2.6,"publicationDate":"2025-01-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11706029/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142958539","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}