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

{"title":"Spontaneous Cervical Swelling Syndrome Mimicking Angioedema","authors":"Victor Coste MD , Florence Castelain MD , Angéline Charansol MD , Fabien Pelletier MD, PhD , François Aubin MD, PhD , Eve Puzenat MD","doi":"10.1016/j.jaip.2025.06.016","DOIUrl":"10.1016/j.jaip.2025.06.016","url":null,"abstract":"","PeriodicalId":51323,"journal":{"name":"Journal of Allergy and Clinical Immunology-In Practice","volume":"13 9","pages":"Pages 2492-2493"},"PeriodicalIF":6.6,"publicationDate":"2025-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144621061","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Advancements in Novel Therapeutics for Chronic Spontaneous Urticaria","authors":"Joshua S. Bernstein MD ,&nbsp;Gordon Sussman MD ,&nbsp;Helena Pite MD, PhD ,&nbsp;Jonathan A. Bernstein MD","doi":"10.1016/j.jaip.2025.05.035","DOIUrl":"10.1016/j.jaip.2025.05.035","url":null,"abstract":"<div><div>Chronic urticaria (CU) is defined by pruritic wheals with or without angioedema, associated with severe itch, which persist for greater than 6 weeks. Lesions move from one part of the body to another and generally are evanescent, lasting less than 24 hours. The global prevalence of the disease ranges from 0.5% to 5% and is correlated with an annual economic burden of over $200 million in the United States. Chronic urticaria can be further divided into chronic spontaneous urticaria and chronic inducible urticaria. Whereas chronic spontaneous urticaria has no identifiable trigger, chronic inducible urticaria can be provoked by both physical and nonphysical stimuli. As many as 7% to 30% of patients with CU can have both types. Mast cells are the major effector cells in the pathogenesis of CU. When activated, mast cells characteristically release bioactive mediators including histamine that bind to specific receptors causing vasodilation and extravasation of fluid from the blood vasculature. This causes the characteristic wheals and itch. However, the pathophysiology of CU is much more complex, involving many mast cell surface receptors, cytokines, and cell activation pathways that are targets for many of the currently available and investigational therapies. Studies have demonstrated that CU causes a significant amount of distress and disruption to patients’ daily lives that can be evaluated using validated patient-reported outcomes measures. In this review, we review CU epidemiology, pathophysiology, subtypes, and diagnosis and discuss current and novel therapies.</div></div>","PeriodicalId":51323,"journal":{"name":"Journal of Allergy and Clinical Immunology-In Practice","volume":"13 9","pages":"Pages 2272-2285"},"PeriodicalIF":6.6,"publicationDate":"2025-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144188522","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Donidalorsen Treatment of Hereditary Angioedema in Patients Previously on Long-Term Prophylaxis","authors":"Marc A. Riedl MD ,&nbsp;Jonathan A. Bernstein MD ,&nbsp;Joshua S. Jacobs MD ,&nbsp;Timothy Craig DO ,&nbsp;Aleena Banerji MD ,&nbsp;Francesca Perego MD, PhD ,&nbsp;William R. Lumry MD ,&nbsp;H. James Wedner MD ,&nbsp;Selina Gierer DO ,&nbsp;Michael E. Manning MD ,&nbsp;Laura Bordone PhD ,&nbsp;Sabrina Treadwell PhD ,&nbsp;Tao Lin MS ,&nbsp;Kenneth B. Newman MD ,&nbsp;Aaron Yarlas PhD ,&nbsp;Danny M. Cohn MD, PhD","doi":"10.1016/j.jaip.2025.06.018","DOIUrl":"10.1016/j.jaip.2025.06.018","url":null,"abstract":"<div><h3>Background</h3><div>Hereditary angioedema (HAE) is a rare, potentially life-threatening disorder characterized by episodes of tissue swelling. Donidalorsen, an investigational ligand-conjugated antisense oligonucleotide, reduces plasma prekallikrein production.</div></div><div><h3>Objective</h3><div>We report an interim analysis on safety, efficacy, quality of life (QoL), and treatment preference and satisfaction from an ongoing open-label phase 3 study (OASISplus Switch cohort, NCT05392114).</div></div><div><h3>Methods</h3><div>Patients with HAE receiving stable doses (≥12 weeks) of lanadelumab, complement protein 1 inhibitor, or berotralstat switched to donidalorsen 80 mg subcutaneously every 4 weeks, using a predefined algorithm. The primary end point was the incidence and severity of treatment-emergent adverse events. Other end points included change in HAE attack rate, angioedema-QoL score, disease control (≥10 points on the Angioedema Control Test), and treatment preference and satisfaction at week 16, compared with baseline on prior treatment.</div></div><div><h3>Results</h3><div>A total of 65 patients were enrolled; 32 switched from lanadelumab, 22 from complement protein 1 inhibitor, and 11 from berotralstat. At cutoff, 58 were ongoing in the study (89%). Forty-five patients (70%) reported treatment-emergent adverse events; 62% were unrelated to donidalorsen. At week 16, total HAE attack rates had decreased by 62%. Hereditary angioedema attack rates decreased by 65%, 41%, and 73%, and mean angioedema-QoL scores improved by 8.4, 9.6, and 17.1 points for patients switching from lanadelumab, C1INH, and berotralstat, respectively. More patients reported well-controlled disease (93% vs 67%), and most patients preferred donidalorsen over their prior treatment, with improved treatment satisfaction.</div></div><div><h3>Conclusions</h3><div>Donidalorsen was well tolerated, decreased HAE attack rate, and improved QoL and disease control. Most patients preferred donidalorsen over their prior treatment. Further analyses are planned at week 52.</div></div>","PeriodicalId":51323,"journal":{"name":"Journal of Allergy and Clinical Immunology-In Practice","volume":"13 9","pages":"Pages 2381-2389.e3"},"PeriodicalIF":6.6,"publicationDate":"2025-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144651170","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Clinical and Immunological Prognostic Factors With Novel Variants in a Large Cohort of Diacylglycerol Acyltransferase 1 Deficiency","authors":"Melek Yorgun Altunbas MD ,&nbsp;Hubert Kogler MD ,&nbsp;Hassan Abolhassani MD, PhD ,&nbsp;Erkan Akkus MD ,&nbsp;Ahmet Basturk MD ,&nbsp;Emre Akkelle MD ,&nbsp;Ersin Sayar MD ,&nbsp;Esra Polat MD ,&nbsp;Altan Kara PhD ,&nbsp;Salim Can MD ,&nbsp;Alexandra Frohne MSc ,&nbsp;Anna Segarra-Roca MSc ,&nbsp;Raul Jimenez-Heredia MSc ,&nbsp;Royala Babayeva MD ,&nbsp;Asena Pınar Sefer MD ,&nbsp;Ayca Kiykim MD ,&nbsp;Sevgi Bilgic Eltan MD ,&nbsp;Elif Karakoc-Aydiner MD ,&nbsp;Ahmet Ozen MD ,&nbsp;Omer Faruk Beser MD ,&nbsp;Safa Baris MD","doi":"10.1016/j.jaip.2025.03.026","DOIUrl":"10.1016/j.jaip.2025.03.026","url":null,"abstract":"<div><h3>Background</h3><div>Biallelic variants in diacylglycerol acyltransferase 1 (<em>DGAT1</em>) genegene have been implicated congenital diarrhea and protein-losing enteropathy. Insights into the immunopathologic features of this ultrarare disorder remain scarce, with only one cohort published to date.</div></div><div><h3>Objective</h3><div>To delineate the clinical presentations, laboratory and immunologic profiles, and therapeutic responses associated with DGAT1 deficiency and identify prognostic indicators that affect survival rates.</div></div><div><h3>Methods</h3><div>In this multicenter retrospective analysis of a comprehensive cohort of nine patients carrying seven novel variants, each displaying distinct phenotypic features, we recorded clinical, immunologic, and laboratory data of patients and evaluated the impact of various factors on prognosis.</div></div><div><h3>Results</h3><div>A total of 67% of patients (n = 6) exhibited symptoms during the first month of life, whereas one demonstrated symptom onset after 6 months. Moreover, 78% of patients (n = 7) presented with diarrhea, all of whom all had vomiting, failure to thrive, hypoalbuminemia, and hypogammaglobulinemia as the advent of protein-losing enteropathy. Patients with reduced CD4⁺ T-cell frequency (n = 2) exhibited severe infections with unexpected bacteria during the follow-up. Despite immunoglobulin replacement therapy, 45% of patients (n = 4) died of infective complications. A decreased CD4⁺/CD8⁺ T-cell ratio was observed in all deceased patients whose colon biopsy samples showed marked inflammation or apoptosis. Early fat-restricted nutrition extended survival, whereas early symptom onset, recurrent severe infections, and a reduced CD4⁺/CD8⁺ T-cell ratio were associated with less favorable outcomes.</div></div><div><h3>Conclusions</h3><div>Our findings advocate early fat restriction as a critical therapeutic strategy. Given the heightened risk of severe infections, antibiotic prophylaxis can be recommended in addition to immunoglobulin replacement therapy for DGAT1-deficient patients exhibiting lymphopenia or diminished CD4⁺ T cells.</div></div>","PeriodicalId":51323,"journal":{"name":"Journal of Allergy and Clinical Immunology-In Practice","volume":"13 9","pages":"Pages 2449-2459.e7"},"PeriodicalIF":6.6,"publicationDate":"2025-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143744374","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Management of Chronic Spontaneous Urticaria Made Practical: What Every Clinician Should Know","authors":"","doi":"10.1016/j.jaip.2025.08.009","DOIUrl":"10.1016/j.jaip.2025.08.009","url":null,"abstract":"","PeriodicalId":51323,"journal":{"name":"Journal of Allergy and Clinical Immunology-In Practice","volume":"13 9","pages":"Pages 2270-2271"},"PeriodicalIF":6.6,"publicationDate":"2025-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145050641","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Exploring mepolizumab use in chronic spontaneous urticaria: An open-label, single-arm, exploratory study","authors":"Jordan Phillipps MD ,&nbsp;Arveen Bhasin MD ,&nbsp;Jason Sluzevich MD","doi":"10.1016/j.jaip.2025.06.014","DOIUrl":"10.1016/j.jaip.2025.06.014","url":null,"abstract":"","PeriodicalId":51323,"journal":{"name":"Journal of Allergy and Clinical Immunology-In Practice","volume":"13 9","pages":"Pages 2503-2505.e1"},"PeriodicalIF":6.6,"publicationDate":"2025-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144337236","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Continuing Medical Education Calendar","authors":"","doi":"10.1016/S2213-2198(25)00784-6","DOIUrl":"10.1016/S2213-2198(25)00784-6","url":null,"abstract":"","PeriodicalId":51323,"journal":{"name":"Journal of Allergy and Clinical Immunology-In Practice","volume":"13 9","pages":"Pages A25-A26"},"PeriodicalIF":6.6,"publicationDate":"2025-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145044451","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Benralizumab for Acute Asthma and COPD","authors":"Adithi Y. Reddy MD,&nbsp;James T. Li MD, PhD","doi":"10.1016/j.jaip.2025.06.026","DOIUrl":"10.1016/j.jaip.2025.06.026","url":null,"abstract":"","PeriodicalId":51323,"journal":{"name":"Journal of Allergy and Clinical Immunology-In Practice","volume":"13 9","pages":"Pages 2532-2533"},"PeriodicalIF":6.6,"publicationDate":"2025-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145049953","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Health-Related Quality of Life in Chronic Urticaria: A Systematic Review and Meta-Analysis","authors":"Gabriel Berberi BSc ,&nbsp;Mahan Maazi MEng ,&nbsp;Connor Prosty MD ,&nbsp;Eric P. McMullen MD ,&nbsp;Michael Fein MD, MSc ,&nbsp;Hermenio Lima MD ,&nbsp;Elvis Martinez-Jaramillo MD, PhD ,&nbsp;Moshe Ben-Shoshan MD, MSc ,&nbsp;Farhad Pourpanah PhD ,&nbsp;Elena Netchiporouk MD, MSc","doi":"10.1016/j.jaip.2025.03.049","DOIUrl":"10.1016/j.jaip.2025.03.049","url":null,"abstract":"<div><h3>Background</h3><div>Chronic urticaria<span> (CU), including chronic spontaneous urticaria<span><span> (CSU) and chronic inducible urticaria (CIndU), significantly affect patients’ health-related quality of life (HRQOL). To date, no </span>systematic reviews or meta-analyses have summarized the HRQOL tools used in CU and the magnitude of HRQOL impact in CU.</span></span></div></div><div><h3>Objective</h3><div>To determine the HRQOL tools used in CU, the burden of CU regarding HRQOL in adults and children, and whether CU subtypes differentially affect HRQOL.</div></div><div><h3>Methods</h3><div>We searched MEDLINE, Embase, Web of Science, Cochrane, CINAHL, and PsycINFO from inception to August 20, 2023 for original articles including populations of adult or pediatric<span> patients with CU or any of its subtypes in which HRQOL was reported using any quality-of-life instrument. We conducted a series of meta-analyses to pool and compare data on HRQOL outcomes by CU subtype, primarily using the Dermatology Life Quality Index.</span></div></div><div><h3>Results</h3><div>We identified 123 publications reporting on 23,508 unique patients with CU and/or its subtypes. The meta-analysis revealed a moderate to large impact of CU and its subtypes on HRQOL. In adults, pooled mean Dermatology Life Quality Index score was 8.9 (95% CI, 7.7-10.1) for CU, 11.0 (95% CI, 9.3-12.7) for CSU, 8.3 (95% CI, 6.8-9.8) for CIndU, and 10.0 (95% CI, 7.7-12.4) for concomitant CSU/CIndU. In children with CSU, mean Children Dermatology Life Quality Index score was 6.9 (95% CI, 1.9-12.0). The analysis revealed high heterogeneity, likely owing to differences in patient population and study design.</div></div><div><h3>Conclusions</h3><div>Chronic urticaria and its subtypes significantly impair HRQOL. The clinical implication of the differences among CU subtypes needs to be further explored, along with additional research in pediatric populations.</div></div>","PeriodicalId":51323,"journal":{"name":"Journal of Allergy and Clinical Immunology-In Practice","volume":"13 9","pages":"Pages 2317-2328"},"PeriodicalIF":6.6,"publicationDate":"2025-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144008385","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Long-term effectiveness and safety of dupilumab in severe asthma: A 5-year real-world multicenter study","authors":"Astrid Chauvin MD ,&nbsp;Drifa Belhadi PhD ,&nbsp;Clairelyne Dupin MD ,&nbsp;Laurent Guilleminault MD, PhD ,&nbsp;Amel Boudjemaa MD ,&nbsp;Nouha Ghourabi MSc ,&nbsp;Anne-Sophie Gamez MD ,&nbsp;Patrick Berger MD, PhD ,&nbsp;Naji Khayat MD ,&nbsp;Philippe Bonniaud MD, PhD ,&nbsp;Christophe Leroyer MD ,&nbsp;Guillaume Mahay MD ,&nbsp;Stéphanie Fry MD ,&nbsp;Pierre-Olivier Girodet MD, PhD ,&nbsp;Geneviève Le Bourdellès MD ,&nbsp;Alain Proust MD ,&nbsp;Lise Rosencher MD ,&nbsp;Gilles Garcia MD, PhD ,&nbsp;Arnaud Bourdin MD, PhD ,&nbsp;Cécile Chenivesse MD, PhD ,&nbsp;Camille Taillé MD, PhD","doi":"10.1016/j.jaip.2025.05.050","DOIUrl":"10.1016/j.jaip.2025.05.050","url":null,"abstract":"","PeriodicalId":51323,"journal":{"name":"Journal of Allergy and Clinical Immunology-In Practice","volume":"13 9","pages":"Pages 2515-2517.e2"},"PeriodicalIF":6.6,"publicationDate":"2025-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144250797","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}