Helena Buso, Davide Firinu, Renato Finco Gambier, Riccardo Scarpa, Giulia Garzi, Valentina Soccodato, Giulia Costanzo, Andrea G Ledda, Nicolò Rashidy, Ilaria Bertozzi, Stefania Nicola, Giulio Tessarin, Mauro Ramigni, Cinzia Piovesan, Fabrizio Vianello, Andrea Vianello, Stefano Del Giacco, Vassilios Lougaris, Luisa Brussino, Mark G Jones, Isabella Quinti, Carlo Agostini, Marcello Rattazzi, Cinzia Milito, Francesco Cinetto
{"title":"Lung function trajectories in Common Variable Immunodeficiencies: an observational retrospective multicenter study.","authors":"Helena Buso, Davide Firinu, Renato Finco Gambier, Riccardo Scarpa, Giulia Garzi, Valentina Soccodato, Giulia Costanzo, Andrea G Ledda, Nicolò Rashidy, Ilaria Bertozzi, Stefania Nicola, Giulio Tessarin, Mauro Ramigni, Cinzia Piovesan, Fabrizio Vianello, Andrea Vianello, Stefano Del Giacco, Vassilios Lougaris, Luisa Brussino, Mark G Jones, Isabella Quinti, Carlo Agostini, Marcello Rattazzi, Cinzia Milito, Francesco Cinetto","doi":"10.1016/j.jaci.2024.10.037","DOIUrl":null,"url":null,"abstract":"<p><strong>Background: </strong>Respiratory disease is a frequent cause of morbidity and mortality in Common Variable Immunodeficiencies (CVIDs), however lung function trajectories are poorly understood.</p><p><strong>Objective: </strong>To determine lung physiology measurements in CVID, their temporal trajectory and association with clinical and immunological parameters.</p><p><strong>Methods: </strong>Retrospective study from 5 Italian centres. CVIDs patients with longitudinal Pulmonary Function Tests (PFTs) measurements and available chest CT scan, were included. Applying the ERS/ATS 2021 standard, PFTs were expressed as percentile value (pct) within the normal distribution of healthy individuals, with the 5<sup>th</sup> pct identified the lower limit of normal (LLN). The association of lung function with clinical and immunological parameters was investigated.</p><p><strong>Results: </strong>185 CVIDs patients were included. 64% had at least one lung comorbidity (bronchiectasis 41% and Granulomatous Interstitial Lung Diseases 24%). At first spirometry, the median FEV1 was 3.07 L [IQR 2.40 - 3.80], placing at the 32<sup>nd</sup> pct [6<sup>th</sup>-61<sup>st</sup>], and the median FVC was 3.70 L [3.00 - 4.54], placing at the 29<sup>th</sup> pct [7<sup>th</sup>-49<sup>th</sup>]. 23% of patients had an FEV1 < LLN and 21% had an FVC < LLN. Switched-memory B cells <2% were associated with both FEV1<LLN (OR 7.58) and FVC<LLN (OR 3.55). In 112 patients with at least 5 years of PFT follow-up, we found no significant difference between measured and predicted annual decline of FEV1 (25.6 vs 20.7 mL/year) and FVC (15.6 vs 16.2 mL/year).</p><p><strong>Conclusion: </strong>Our findings suggest that lung volumes of the majority of CVID patients placed in the lower third of normal distribution of healthy individuals. After diagnosis the rate of lung decline was not accelerated.</p>","PeriodicalId":14936,"journal":{"name":"Journal of Allergy and Clinical Immunology","volume":" ","pages":""},"PeriodicalIF":11.4000,"publicationDate":"2024-11-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":null,"platform":"Semanticscholar","paperid":null,"PeriodicalName":"Journal of Allergy and Clinical Immunology","FirstCategoryId":"3","ListUrlMain":"https://doi.org/10.1016/j.jaci.2024.10.037","RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q1","JCRName":"ALLERGY","Score":null,"Total":0}
引用次数: 0
Abstract
Background: Respiratory disease is a frequent cause of morbidity and mortality in Common Variable Immunodeficiencies (CVIDs), however lung function trajectories are poorly understood.
Objective: To determine lung physiology measurements in CVID, their temporal trajectory and association with clinical and immunological parameters.
Methods: Retrospective study from 5 Italian centres. CVIDs patients with longitudinal Pulmonary Function Tests (PFTs) measurements and available chest CT scan, were included. Applying the ERS/ATS 2021 standard, PFTs were expressed as percentile value (pct) within the normal distribution of healthy individuals, with the 5th pct identified the lower limit of normal (LLN). The association of lung function with clinical and immunological parameters was investigated.
Results: 185 CVIDs patients were included. 64% had at least one lung comorbidity (bronchiectasis 41% and Granulomatous Interstitial Lung Diseases 24%). At first spirometry, the median FEV1 was 3.07 L [IQR 2.40 - 3.80], placing at the 32nd pct [6th-61st], and the median FVC was 3.70 L [3.00 - 4.54], placing at the 29th pct [7th-49th]. 23% of patients had an FEV1 < LLN and 21% had an FVC < LLN. Switched-memory B cells <2% were associated with both FEV1
Conclusion: Our findings suggest that lung volumes of the majority of CVID patients placed in the lower third of normal distribution of healthy individuals. After diagnosis the rate of lung decline was not accelerated.
期刊介绍：
The Journal of Allergy and Clinical Immunology is a prestigious publication that features groundbreaking research in the fields of Allergy, Asthma, and Immunology. This influential journal publishes high-impact research papers that explore various topics, including asthma, food allergy, allergic rhinitis, atopic dermatitis, primary immune deficiencies, occupational and environmental allergy, and other allergic and immunologic diseases. The articles not only report on clinical trials and mechanistic studies but also provide insights into novel therapies, underlying mechanisms, and important discoveries that contribute to our understanding of these diseases. By sharing this valuable information, the journal aims to enhance the diagnosis and management of patients in the future.
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