Familial Pulmonary Fibrosis: Natural History Of Preclinical Disease

El–Chemaly, Souheil; Ziegler, Shira G.; Wilson, Kirkland; Gahl, William A.; Moss, Joel; Gochuico, Bernadette R.

doi:10.1164/ajrccm-conference.2010.181.1_meetingabstracts.a2980

Cited by 3 publications

(6 citation statements)

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“…Although radiologic progression has been demonstrated in small numbers of subjects with subclinical rheumatoid arthritis and ILD (RA-ILD) (17) and smokers (z50 and 25-44%, respectively) (5,19), it is important to note that the clinical significance of radiologic subtypes is unclear and that progression of subclinical ILD to clinically significant ILD has been demonstrated only in a small number of studies (5,11). Longitudinal studies will be required to identify which radiologic abnormalities most commonly precede the development of ILD in at-risk populations.…”

Section: Detection and Assessment Of Subclinical Ild Visual And Autommentioning

confidence: 99%

“…Several studies have identified subclinical ILD in subjects with genetic mutations demonstrated to be associated with IPF and FPF (11,24,34,35). Diaz de Leon and colleagues assessed a variety of pulmonary, blood, skin, and bone parameters in 20 asymptomatic subjects with heterozygous TERT mutations and radiologic evidence of subclinical ILD (24).…”

Section: Genetic and Genomic Associationsmentioning

confidence: 99%

“…More recent studies demonstrate that screening affected FPF kindreds with high-resolution computed tomography (HRCT) scans leads to detection of subclinical ILD in asymptomatic relatives (8,9). This suggests that some individuals at risk for ILD (e.g., members of families affected with pulmonary fibrosis, smokers, and individuals with connective tissue diseases) have mild radiologic changes on CT scan, and a subset of these patients over time could develop ILD (10,11).…”

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confidence: 99%

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Subclinical Interstitial Lung Disease

Doyle

Hunninghake

Rosas

2012

Am J Respir Crit Care Med

142

114

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The widespread use of high-resolution computed tomography in clinical and research settings has increased the detection of interstitial lung abnormalities (ILA) in asymptomatic and undiagnosed individuals. We reported that in smokers, ILA were present in about 1 of every 12 high-resolution computed tomographic scans; however, the long-term significance of these subclinical changes remains unclear. Studies in families affected with pulmonary fibrosis, smokers with chronic obstructive pulmonary disease, and patients with inflammatory lung disease have shown that asymptomatic and undiagnosed individuals with ILA have reductions in lung volume, functional limitations, increased pulmonary symptoms, histopathologic changes, and molecular profiles similar to those observed in patients with clinically significant interstitial lung disease (ILD). These findings suggest that, in select at-risk populations, ILA may represent early stages of pulmonary fibrosis or subclinical ILD. The growing interest surrounding this topic is motivated by our poor understanding of the inciting events and natural history of ILD, coupled with a lack of effective therapies. In this perspective, we outline past and current research focused on validating radiologic, physiological, and molecular methods to detect subclinical ILD. We discuss the limitations of the available cross-sectional studies and the need for future longitudinal studies to determine the prognostic and therapeutic implications of subclinical ILD in populations at risk of developing clinically significant ILD.

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Section: Detection and Assessment Of Subclinical Ild Visual And Autommentioning

confidence: 99%

Section: Genetic and Genomic Associationsmentioning

confidence: 99%

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confidence: 99%

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Subclinical Interstitial Lung Disease

Doyle

Hunninghake

Rosas

2012

Am J Respir Crit Care Med

142

114

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“…According to the 2013 ILD consensus statement by the American Thoracic Society/European Respiratory Society (ATS/ERS), the most frequent ILD is idiopathic interstitial pneumonia (IIP) [ 4 , 5 , 6 ], the most common of which is idiopathic pulmonary fibrosis (IPF) (40%) [ 7 , 8 , 9 ]. In recent years, the incidence of IPF has been on the rise [ 10 , 11 ], and a study carried out in the United Kingdom from 1968 to 2008 determined that the incidence of IPF is growing at a rate of 5% per year [ 12 ].…”

Section: Introductionmentioning

confidence: 99%

“…It is important to know the family history of patients with IPF, as up to 20% of patients have a history of a family member with this pathology [ 4 ]. Other etiological factors that have been associated with the development of this disease are smoking, gastroesophageal reflux disease (GERD) associated with hiatal hernia in up to 80% and autoimmunity [ 1 , 7 ].…”

Section: Introductionmentioning

confidence: 99%

Clinical Profile of Patients with Idiopathic Pulmonary Fibrosis in Real Life

Morena

Fernández

Campos

et al. 2023

JCM

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Objective: The objective of this study is to define the real-life clinical profile and therapeutic management of patients with idiopathic pulmonary fibrosis using artificial intelligence. Methods: We have conducted an observational, retrospective, non-interventional study using data from the Castilla-La Mancha Regional Healthcare Service (SESCAM) in Spain between January 2012 and December 2020. The Savana Manager 3.0 artificial intelligence platform was used to collect information from electronic medical records by applying natural language processing. Results: Our study includes 897 subjects whose diagnosis was compatible with idiopathic pulmonary fibrosis; 64.8% were men, with a mean age of 72.9 years (95% CI 71.9–73.8), and 35.2% were women, with a mean age of 76.8 years (95% CI 75.5–78). Patients who had a family history of IPF (98 patients; 12%) were younger and predominantly female (53.1%). Regarding treatment, 45% of patients received antifibrotic therapy. Patients who had undergone lung biopsy, chest CT, or bronchoscopy were younger than the patient population in whom these studies were not completed. Conclusions: This study has used artificial intelligence techniques to analyze a large population over a 9-year period and determine the situation of IPF in standard clinical practice by identifying the patient clinical profile, use of diagnostic tests and therapeutic management.

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Clinical Course and Prediction of Survival in Idiopathic Pulmonary Fibrosis

Ley

Collard

King

2011

Am J Respir Crit Care Med

1,391

1,091

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Idiopathic pulmonary fibrosis (IPF) is a progressive, life-threatening, interstitial lung disease of unknown etiology. The median survival of patients with IPF is only 2 to 3 years, yet some patients live much longer. Respiratory failure resulting from disease progression is the most frequent cause of death. To date we have limited information as to predictors of mortality in patients with IPF, and research in this area has failed to yield prediction models that can be reliably used in clinical practice to predict individual risk of mortality. The goal of this concise clinical review is to examine and summarize the current data on the clinical course, individual predictors of survival, and proposed clinical prediction models in IPF. Finally, we will discuss challenges and future directions related to predicting survival in IPF.

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Familial Pulmonary Fibrosis: Natural History Of Preclinical Disease

Cited by 3 publications

References 0 publications

Subclinical Interstitial Lung Disease

Subclinical Interstitial Lung Disease

Clinical Profile of Patients with Idiopathic Pulmonary Fibrosis in Real Life

Clinical Course and Prediction of Survival in Idiopathic Pulmonary Fibrosis

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