Our findings that data-driven textural analysis fibrosis score is associated with baseline measures of disease severity and with change over time suggest that CT captures anatomic information that ... Purpose To evaluate associations between pulmonary function and both quantitative analysis and visual assessment of thin-section computed tomography (CT) images at baseline and at 15-month follow-u...
FIFor the Genetic Epidemiology of COPD (COPDGene) Investigators
For the Genetic Epidemiology of COPD (COPDGene) Investigators
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Presence and severity of emphysema, scored automatically according to the Fleischner system by using a deep learning algorithm, is associated with greater impairment and risk of mortality. BackgroundPattern of emphysema at chest CT, scored visually by using the Fleischner Society system, is associated with physiologic impairment and mortality risk.PurposeTo determine whether particip...
1 October 2022 in American Journal of Respiratory and Critical Care Medicine
Rationale: Reliable outcome prediction in patients with fibrotic lung disease using baseline high-resolution computed tomography (HRCT) data remains challenging. Objectives: To evaluate the prognostic accuracy of a deep learning algorithm (SOFIA [Systematic Objective Fibrotic Imaging Analysis Algorithm]), trained and validated in the identification of usual interstitial pneumonia (UIP)-like features on HRCT (UIP probability), in a large cohort of well-characterized patients with progressive fibrotic lung disease drawn from a national registry. Methods: SOFIA and radiologist UIP probabilities were converted to Prospective Investigation of Pulmonary Embolism Diagnosis (PIOPED)-based UIP probability categories (UIP not included in the differential, 0–4%; low probability of UIP, 5–29%; intermediate probability of UIP, 30–69%; high probability of UIP, 70–94%; and pathognomonic for UIP, 95–100%), and their prognostic utility was assessed using Cox proportional hazards modeling. Measurements and Main Results: In multivariable analysis adjusting for age, sex, guideline-based radiologic diagnosis, anddisease severity (using total interstitial lung disease [ILD] extent on HRCT, percent predicted FVC, DlCO, or the composite physiologic index), only SOFIA UIP probability PIOPED categories predicted survival. SOFIA-PIOPED UIP probability categories remained prognostically significant in patients considered indeterminate (n = 83) by expert radiologist consensus (hazard ratio, 1.73; P < 0.0001; 95% confidence interval, 1.40–2.14). In patients undergoing surgical lung biopsy (n = 86), after adjusting for guideline-based histologic pattern and total ILD extent on HRCT, only SOFIA-PIOPED probabilities were predictive of mortality (hazard ratio, 1.75; P < 0.0001; 95% confidence interval, 1.37–2.25). Conclusions: Deep learning–based UIP probability on HRCT provides enhanced outcome prediction in patients with progressive fibrotic lung disease when compared with expert radiologist evaluation or guideline-based histologic pattern. In principle, this tool may be useful in multidisciplinary characterization of fibrotic lung disease. The utility of this technology as a decision support system when ILD expertise is unavailable requires further investigation.
1 July 2025 in American Journal of Respiratory and Critical Care Medicine
Background: There is growing interest in identifying early stages of interstitial lung disease (ILD) to improve patient outcomes. This document reviews updated evidence on interstitial lung abnormalities (ILAs); provides suggestions for screening, evaluation, and management; proposes criteria for distinguishing ILAs from ILD; and identifies research priorities. Methods: A committee of clinical and methodology experts met by video conference to define ILAs and ILD by consensus and voted on 11 prespecified questions after reviewing synthesized evidence from a systematic literature search. Agreement of ≥70% was required to approve each suggestion. Results: ILA is defined as nondependent bilateral parenchymal abnormalities on computed tomography, including ground-glass opacities or reticulations, lung distortion, traction bronchiectasis, and/or honeycombing involving ≥5% of a lung zone. The updated definition removes the prior exclusion of high-risk populations. ILD is distinguished from ILAs by symptoms (dyspnea/cough) attributable to an interstitial process, abnormal or declining lung function, fibrotic (honeycombing and/or reticulation with traction bronchiectasis involving ≥5% of total lung volume) or progressive imaging abnormalities, and/or specific fibrotic ILD patterns on imaging or pathology. Suggestions include ILA/ILD assessment on imaging acquired for lung cancer screening, screening adults with connective tissue disease and first-degree relatives of patients with familial pulmonary fibrosis, assessing baseline symptoms and pulmonary function among those with ILAs, and monitoring ILAs with chest computed tomography every 2–3 years. Conclusions: This document presents a comprehensive literature review of ILAs with updates to the Fleischner Society ILA definition, establishes a working ILD definition, and provides evidence-based suggestions for ILA evaluation and management.
1 June 2021 in American Journal of Respiratory Cell and Molecular Biology
Idiopathic pulmonary fibrosis (IPF) is a progressive fibrotic interstitial lung disease with underlying mechanisms that have been primarily investigated in mice after intratracheal instillation of a single dose of bleomycin. However, the model has significant limitations, including transient fibrosis that spontaneously resolves and its failure to fully recapitulate the epithelial remodeling in the lungs of patients with IPF. Thus, there remains an unmet need for a preclinical model with features that more closely resemble the human disease. Repetitive intratracheal instillation of bleomycin has previously been shown to recapitulate some of these features, but the instillation procedure is complex, and the long-term consequences on epithelial remodeling and fibrosis persistence and progression remain poorly understood. Here, we developed a simplified repetitive bleomycin instillation strategy consisting of three bi-weekly instillations that leads to persistent and progressive pulmonary fibrosis. Lung histology demonstrates increased collagen deposition, fibroblast accumulation, loss of type I and type II alveolar epithelial cells within fibrotic areas, bronchiolization of the lung parenchyma with CCSP+ cells, remodeling of the distal lung into cysts reminiscent of simple honeycombing, and accumulation of hyperplastic transitional KRT8+ epithelial cells. Micro–computed tomographic imaging demonstrated significant traction bronchiectasis and subpleural fibrosis. Thus, the simplified repetitive bleomycin instillation strategy leads to progressive fibrosis and recapitulates the histological and radiographic characteristics of IPF. Compared with the single bleomycin instillation model, we suggest that the simplified repetitive instillation model may be better suited to address mechanistic questions about IPF pathogenesis and preclinical studies of antifibrotic drug candidates.
A large proportion of emphysema progression in smokers with and those without airflow limitation was not accounted for by changes in forced expiratory volume in 1 second, suggesting that CT-detecte... Background CT is used to quantify abnormal changes in the lung parenchyma of smokers that might overlap chronic obstructive pulmonary disease (COPD), but studies on the progression of expiratory ai...