Margaret L Salisbury, Cheryl Markin, Tisra Fadely, Adam R Guttentag, Stephen M Humphries, David A Lynch, Jonathan A Kropski, Timothy S Blackwell
{"title":"家族性肺纤维化高危人群的进行性早期肺间质异常：一项前瞻性队列研究","authors":"Margaret L Salisbury, Cheryl Markin, Tisra Fadely, Adam R Guttentag, Stephen M Humphries, David A Lynch, Jonathan A Kropski, Timothy S Blackwell","doi":"10.1164/rccm.202403-0524OC","DOIUrl":null,"url":null,"abstract":"<p><strong>Rationale: </strong>Relatives of patients with familial pulmonary fibrosis (FPF) are at increased risk to develop FPF. Interstitial lung abnormalities (ILAs) are a radiologic biomarker of subclinical disease, but the implications of very mild abnormalities remain unclear.</p><p><strong>Objectives: </strong>To quantify the progression risk among FPF relatives with abnormalities below the threshold for ILAs as described by the Fleischner Society and to describe the characteristics of participants with new or progressive ILAs during observation.</p><p><strong>Methods: </strong>Asymptomatic FPF relatives undergo serial screening high-resolution chest CT (HRCT). For this analysis, Early ILAs (no minimum threshold of lung involvement) were sub-classified as Mild (all interstitial abnormalities involve <5% of a lung zone) or Moderate (any abnormality involves >5%). Identification of new or progressive ILAs on HRCT, or development of Pulmonologist-diagnosed clinical FPF were defined as progression. Covariate-adjusted logistic regression identified progression-associated characteristics.</p><p><strong>Measurements and main results: </strong>From 2008-2023, 273 participants in follow-up procedures were 53.2 9.4 years old at enrollment, 95 (35%) were male, and 73/268 (27%) were ever-smokers. During a mean follow-up of 6.2 3.0 years, progression occurred among 31/211 (15%) of those with absence of ILAs at enrollment, 32/49 (65%) of Mild ILAs, and 10/13 (77%) of Moderate ILAs. Mild ILAs had 9.15 (95% CI 4.40-19.00, p<0.0001) times and Moderate ILAs had 17.14 (95% CI 4.42-66.49, p<0.0001) times the odds of progression as subjects without ILAs.</p><p><strong>Conclusions: </strong>In persons at-risk for FPF, minor interstitial abnormalities, including reticulation that is unilateral or involves <5% of a lung zone, frequently represent subclinical disease.</p>","PeriodicalId":7664,"journal":{"name":"American journal of respiratory and critical care medicine","volume":null,"pages":null},"PeriodicalIF":19.3000,"publicationDate":"2024-08-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":"{\"title\":\"Progressive Early Interstitial Lung Abnormalities in Persons At-Risk for Familial Pulmonary Fibrosis: A Prospective Cohort Study.\",\"authors\":\"Margaret L Salisbury, Cheryl Markin, Tisra Fadely, Adam R Guttentag, Stephen M Humphries, David A Lynch, Jonathan A Kropski, Timothy S Blackwell\",\"doi\":\"10.1164/rccm.202403-0524OC\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"<p><strong>Rationale: </strong>Relatives of patients with familial pulmonary fibrosis (FPF) are at increased risk to develop FPF. Interstitial lung abnormalities (ILAs) are a radiologic biomarker of subclinical disease, but the implications of very mild abnormalities remain unclear.</p><p><strong>Objectives: </strong>To quantify the progression risk among FPF relatives with abnormalities below the threshold for ILAs as described by the Fleischner Society and to describe the characteristics of participants with new or progressive ILAs during observation.</p><p><strong>Methods: </strong>Asymptomatic FPF relatives undergo serial screening high-resolution chest CT (HRCT). For this analysis, Early ILAs (no minimum threshold of lung involvement) were sub-classified as Mild (all interstitial abnormalities involve <5% of a lung zone) or Moderate (any abnormality involves >5%). Identification of new or progressive ILAs on HRCT, or development of Pulmonologist-diagnosed clinical FPF were defined as progression. Covariate-adjusted logistic regression identified progression-associated characteristics.</p><p><strong>Measurements and main results: </strong>From 2008-2023, 273 participants in follow-up procedures were 53.2 9.4 years old at enrollment, 95 (35%) were male, and 73/268 (27%) were ever-smokers. During a mean follow-up of 6.2 3.0 years, progression occurred among 31/211 (15%) of those with absence of ILAs at enrollment, 32/49 (65%) of Mild ILAs, and 10/13 (77%) of Moderate ILAs. Mild ILAs had 9.15 (95% CI 4.40-19.00, p<0.0001) times and Moderate ILAs had 17.14 (95% CI 4.42-66.49, p<0.0001) times the odds of progression as subjects without ILAs.</p><p><strong>Conclusions: </strong>In persons at-risk for FPF, minor interstitial abnormalities, including reticulation that is unilateral or involves <5% of a lung zone, frequently represent subclinical disease.</p>\",\"PeriodicalId\":7664,\"journal\":{\"name\":\"American journal of respiratory and critical care medicine\",\"volume\":null,\"pages\":null},\"PeriodicalIF\":19.3000,\"publicationDate\":\"2024-08-13\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"American journal of respiratory and critical care medicine\",\"FirstCategoryId\":\"3\",\"ListUrlMain\":\"https://doi.org/10.1164/rccm.202403-0524OC\",\"RegionNum\":1,\"RegionCategory\":\"医学\",\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"\",\"PubModel\":\"\",\"JCR\":\"Q1\",\"JCRName\":\"CRITICAL CARE MEDICINE\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"American journal of respiratory and critical care medicine","FirstCategoryId":"3","ListUrlMain":"https://doi.org/10.1164/rccm.202403-0524OC","RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q1","JCRName":"CRITICAL CARE MEDICINE","Score":null,"Total":0}
Progressive Early Interstitial Lung Abnormalities in Persons At-Risk for Familial Pulmonary Fibrosis: A Prospective Cohort Study.
Rationale: Relatives of patients with familial pulmonary fibrosis (FPF) are at increased risk to develop FPF. Interstitial lung abnormalities (ILAs) are a radiologic biomarker of subclinical disease, but the implications of very mild abnormalities remain unclear.
Objectives: To quantify the progression risk among FPF relatives with abnormalities below the threshold for ILAs as described by the Fleischner Society and to describe the characteristics of participants with new or progressive ILAs during observation.
Methods: Asymptomatic FPF relatives undergo serial screening high-resolution chest CT (HRCT). For this analysis, Early ILAs (no minimum threshold of lung involvement) were sub-classified as Mild (all interstitial abnormalities involve <5% of a lung zone) or Moderate (any abnormality involves >5%). Identification of new or progressive ILAs on HRCT, or development of Pulmonologist-diagnosed clinical FPF were defined as progression. Covariate-adjusted logistic regression identified progression-associated characteristics.
Measurements and main results: From 2008-2023, 273 participants in follow-up procedures were 53.2 9.4 years old at enrollment, 95 (35%) were male, and 73/268 (27%) were ever-smokers. During a mean follow-up of 6.2 3.0 years, progression occurred among 31/211 (15%) of those with absence of ILAs at enrollment, 32/49 (65%) of Mild ILAs, and 10/13 (77%) of Moderate ILAs. Mild ILAs had 9.15 (95% CI 4.40-19.00, p<0.0001) times and Moderate ILAs had 17.14 (95% CI 4.42-66.49, p<0.0001) times the odds of progression as subjects without ILAs.
Conclusions: In persons at-risk for FPF, minor interstitial abnormalities, including reticulation that is unilateral or involves <5% of a lung zone, frequently represent subclinical disease.
期刊介绍：
The American Journal of Respiratory and Critical Care Medicine focuses on human biology and disease, as well as animal studies that contribute to the understanding of pathophysiology and treatment of diseases that affect the respiratory system and critically ill patients. Papers that are solely or predominantly based in cell and molecular biology are published in the companion journal, the American Journal of Respiratory Cell and Molecular Biology. The Journal also seeks to publish clinical trials and outstanding review articles on areas of interest in several forms. The State-of-the-Art review is a treatise usually covering a broad field that brings bench research to the bedside. Shorter reviews are published as Critical Care Perspectives or Pulmonary Perspectives. These are generally focused on a more limited area and advance a concerted opinion about care for a specific process. Concise Clinical Reviews provide an evidence-based synthesis of the literature pertaining to topics of fundamental importance to the practice of pulmonary, critical care, and sleep medicine. Images providing advances or unusual contributions to the field are published as Images in Pulmonary, Critical Care, Sleep Medicine and the Sciences.
A recent trend and future direction of the Journal has been to include debates of a topical nature on issues of importance in pulmonary and critical care medicine and to the membership of the American Thoracic Society. Other recent changes have included encompassing works from the field of critical care medicine and the extension of the editorial governing of journal policy to colleagues outside of the United States of America. The focus and direction of the Journal is to establish an international forum for state-of-the-art respiratory and critical care medicine.
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