The Experts below are selected from a list of 30834 Experts worldwide ranked by ideXlab platform
Luca Richeldi - One of the best experts on this subject based on the ideXlab platform.
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Pamrevlumab for the treatment of Idiopathic Pulmonary Fibrosis.
Expert Opinion on Investigational Drugs, 2020Co-Authors: Giacomo Sgalla, Claudia Franciosa, Jacopo Simonetti, Luca RicheldiAbstract:The two available therapies for Idiopathic Pulmonary Fibrosis (IPF), pirfenidone and nintedanib, slow down but do not halt IPF progression. Hence, in the last few years, several agents with specifi...
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Novel drug targets in Idiopathic Pulmonary Fibrosis
Expert opinion on orphan drugs, 2019Co-Authors: Mariarosaria Calvello, Maria Chiara Flore, Luca RicheldiAbstract:ABSTRACTIntroduction: Idiopathic Pulmonary Fibrosis (IPF) is a fatal, fibrosing interstitial pneumonia of unknown cause, that arises with progressive worsening in lung function. Several pathways we...
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Nintedanib for the treatment of Idiopathic Pulmonary Fibrosis.
Expert Opinion on Pharmacotherapy, 2018Co-Authors: Francesco Varone, Giacomo Sgalla, Bruno Iovene, Teresa Bruni, Luca RicheldiAbstract:ABSTRACTIntroduction: Idiopathic Pulmonary Fibrosis (IPF) is an interstitial lung disease characterized by the progressive loss of Pulmonary function, ultimately leading to respiratory failure and ...
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Idiopathic Pulmonary Fibrosis
Nature reviews. Disease primers, 2017Co-Authors: Fernando J Martinez, Harold R. Collard, Ganesh Raghu, Annie Pardo, Moises Selman, Luca Richeldi, Hiroyuki Taniguchi, Jeffrey J Swigris, A.u. WellsAbstract:Idiopathic Pulmonary Fibrosis (IPF) is a chronic, progressive lung disease characterized by progressive lung scarring and the histological picture of usual interstitial pneumonia (UIP). It is associated with increasing cough and dyspnoea and impaired quality of life. IPF affects ∼3 million people worldwide, with incidence increasing dramatically with age. The diagnostic approach includes the exclusion of other interstitial lung diseases or overlapping conditions and depends on the identification of the UIP pattern, usually with high-resolution CT; lung biopsy might be required in some patients. The UIP pattern is predominantly bilateral, peripheral and with a basal distribution of reticular changes associated with traction bronchiectasis and clusters of subpleural cystic airspaces. The biological processes underlying IPF are thought to reflect an aberrant reparative response to repetitive alveolar epithelial injury in a genetically susceptible ageing individual, although many questions remain on how to define susceptibility. Substantial progress has been made in the understanding of the clinical management of IPF, with the availability of two pharmacotherapeutic agents, pirfenidone and nintedanib, that decrease physiological progression and likely improve progression-free survival. Current efforts are directed at identifying IPF early, potentially relying on combinations of biomarkers that include circulating factors, demographics and imaging data. Idiopathic Pulmonary Fibrosis (IPF) is a chronic lung disease characterized by progressive lung scarring that is thought to occur in a genetically susceptible, ageing individual through aberrant reparative responses to repeated injury to the alveolar epithelium. This Primer summarizes the processes thought to underlie the development of IPF and clinical challenges faced in treating patients with this irreversible condition.
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Idiopathic Pulmonary Fibrosis
The Lancet, 2017Co-Authors: Harold R. Collard, Luca Richeldi, Mark G JonesAbstract:Summary Idiopathic Pulmonary Fibrosis is a prototype of chronic, progressive, and fibrotic lung disease. Healthy tissue is replaced by altered extracellular matrix and alveolar architecture is destroyed, which leads to decreased lung compliance, disrupted gas exchange, and ultimately respiratory failure and death. In less than a decade, understanding of the pathogenesis and management of this disease has been transformed, and two disease-modifying therapies have been approved, worldwide. In this Seminar, we summarise the presentation, pathophysiology, diagnosis, and treatment options available for patients with Idiopathic Pulmonary Fibrosis. This disease has improved understanding of the mechanisms of lung Fibrosis, and offers hope that similar approaches will transform the management of patients with other progressive fibrotic lung diseases.
Ganesh Raghu - One of the best experts on this subject based on the ideXlab platform.
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Idiopathic Pulmonary Fibrosis
Nature reviews. Disease primers, 2017Co-Authors: Fernando J Martinez, Harold R. Collard, Ganesh Raghu, Annie Pardo, Moises Selman, Luca Richeldi, Hiroyuki Taniguchi, Jeffrey J Swigris, A.u. WellsAbstract:Idiopathic Pulmonary Fibrosis (IPF) is a chronic, progressive lung disease characterized by progressive lung scarring and the histological picture of usual interstitial pneumonia (UIP). It is associated with increasing cough and dyspnoea and impaired quality of life. IPF affects ∼3 million people worldwide, with incidence increasing dramatically with age. The diagnostic approach includes the exclusion of other interstitial lung diseases or overlapping conditions and depends on the identification of the UIP pattern, usually with high-resolution CT; lung biopsy might be required in some patients. The UIP pattern is predominantly bilateral, peripheral and with a basal distribution of reticular changes associated with traction bronchiectasis and clusters of subpleural cystic airspaces. The biological processes underlying IPF are thought to reflect an aberrant reparative response to repetitive alveolar epithelial injury in a genetically susceptible ageing individual, although many questions remain on how to define susceptibility. Substantial progress has been made in the understanding of the clinical management of IPF, with the availability of two pharmacotherapeutic agents, pirfenidone and nintedanib, that decrease physiological progression and likely improve progression-free survival. Current efforts are directed at identifying IPF early, potentially relying on combinations of biomarkers that include circulating factors, demographics and imaging data. Idiopathic Pulmonary Fibrosis (IPF) is a chronic lung disease characterized by progressive lung scarring that is thought to occur in a genetically susceptible, ageing individual through aberrant reparative responses to repeated injury to the alveolar epithelium. This Primer summarizes the processes thought to underlie the development of IPF and clinical challenges faced in treating patients with this irreversible condition.
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Treatment of Idiopathic Pulmonary Fibrosis With Ambrisentan
Annals of Internal Medicine, 2013Co-Authors: Ganesh Raghu, Egan Jj, Jürgen Behr, Brown Kk, Kawut Sm, Flaherty Kr, Martinez Fj, Nathan Sd, Wells Au, Collard HrAbstract:Idiopathic Pulmonary Fibrosis (IPF) is a life-threatening, progressive disease with no approved therapy. Endothelin-1 is believed to be involved in its pathogenesis. This trial aimed to determine t...
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Does Chronic Microaspiration Cause Idiopathic Pulmonary Fibrosis
The American Journal of Medicine, 2010Co-Authors: Harold R. Collard, Ganesh Raghu, Matthew P. Sweet, Steven R. Hays, Guilherme M. Campos, Jeffrey A. Golden, Talmadge E. KingAbstract:Idiopathic Pulmonary Fibrosis is a diffuse fibrotic lung disease of unknown etiology with no effective treatment. Emerging data support a role for chronic microaspiration (ie, subclinical aspiration of small droplets) in the pathogenesis and natural history of Idiopathic Pulmonary Fibrosis. However, the precise relationship between chronic microaspiration and Idiopathic Pulmonary Fibrosis remains unknown. Gastroesophageal reflux, a presumed risk factor for microaspiration, has been strongly associated with Idiopathic Pulmonary Fibrosis with an estimated prevalence of up to 90%. This review aims to describe the relationship between chronic microaspiration and Idiopathic Pulmonary Fibrosis by laying out the clinical and biologic rationale for this relationship and exploring the scientific evidence available. The gaps in our current understanding of the diagnosis of chronic microaspiration and Idiopathic Pulmonary Fibrosis and the ongoing uncertainties in management and treatment will be highlighted. Defining the role of chronic microaspiration in Idiopathic Pulmonary Fibrosis is essential as it has potential clinical, pathobiological, and treatment implications for this deadly disease.
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incidence and prevalence of Idiopathic Pulmonary Fibrosis
American Journal of Respiratory and Critical Care Medicine, 2006Co-Authors: Ganesh Raghu, Derek Weycker, John Edelsberg, Williamson Z Bradford, Gerry OsterAbstract:Rationale: Idiopathic Pulmonary Fibrosis is a chronic interstitial lung disease of unknown etiology; its epidemiology in the United States has not been well characterized.Objective: To estimate the annual incidence and prevalence of Idiopathic Pulmonary Fibrosis in the United States.Methods: Retrospective cohort design utilizing a large health care claims database spanning the period January 1996 through December 2000.Measurements and Main Results: Persons with Idiopathic Pulmonary Fibrosis were identified based on diagnosis and procedure codes. Using broad case-finding criteria, prevalence was estimated to range from 4.0 per 100,000 persons aged 18 to 34 yr to 227.2 per 100,000 among those 75 yr or older; annual incidence was estimated to range from 1.2 to 76.4 per 100,000. Using narrow case-finding criteria, prevalence ranged from 0.8 to 64.7 per 100,000 persons; comparable figures for incidence were 0.4 to 27.1 per 100,000 persons. Extrapolating these rates to the overall United States' population, pre...
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high prevalence of abnormal acid gastro oesophageal reflux in Idiopathic Pulmonary Fibrosis
European Respiratory Journal, 2006Co-Authors: Ganesh Raghu, T D Freudenberger, Steve T Yang, J R Curtis, C Spada, J Hayes, J Sillery, C E Pope, C A PellegriniAbstract:The aim of this prospective study was to determine the prevalence and characteristics of acid gastro-oesophageal reflux (GER) in patients with Idiopathic Pulmonary Fibrosis (IPF). Sixty-five consecutive patients with well-defined IPF were subjected to 24-h pH monitoring and oesophageal manometry. A total of 133 consecutive patients with intractable asthma and symptoms of GER were used as comparisons. The prevalence of abnormal acid GER in IPF patients was 87%, with 76% and 63% demonstrating abnormal distal and proximal oesophageal acid exposures, respectively. Abnormal acid GER was significantly more common in IPF patients than asthma patients. Only 47% of IPF patients experienced classic GER symptoms. Despite treatment with standard doses of proton pump inhibitors (PPIs), 12 out of 19 patients receiving PPIs during the 24-h pH monitoring had abnormal oesophageal acid exposures by pH probe. There was no correlation between IPF severity and acid GER severity. In conclusion, abnormal acid gastro-oesophageal reflux is highly prevalent, but often clinically occult in patients with Idiopathic Pulmonary Fibrosis. Standard doses of proton pump inhibitors may not suppress the acid gastro-oesophageal reflux in this population. Therefore, further studies are needed to determine if acid abnormal gastro-oesophageal reflux represents an important risk factor for Idiopathic Pulmonary Fibrosis development or progression, and if optimal suppression of acid gastro-oesophageal reflux slows the progression of Idiopathic Pulmonary Fibrosis and/or decreases episodic exacerbations of Idiopathic Pulmonary Fibrosis.
David J Lederer - One of the best experts on this subject based on the ideXlab platform.
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Idiopathic Pulmonary Fibrosis
The New England Journal of Medicine, 2018Co-Authors: David J Lederer, Fernando J MartinezAbstract:Idiopathic Pulmonary Fibrosis Idiopathic Pulmonary Fibrosis appears to be increasing in incidence. It requires early recognition and intervention with supportive care and pharmacologic agents to forestall its progression. Lung transplantation may be curative, but the disease may recur in transplanted lungs.
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acute exacerbation of Idiopathic Pulmonary Fibrosis an international working group report
American Journal of Respiratory and Critical Care Medicine, 2016Co-Authors: Harold R. Collard, David J Lederer, A.u. Wells, Christopher J Ryerson, Tamera J Corte, Gisli Jenkins, Yasuhiro Kondoh, Toby M Maher, Katerina M Antoniou, Juergen BehrAbstract:Acute exacerbation of Idiopathic Pulmonary Fibrosis has been defined as an acute, clinically significant, respiratory deterioration of unidentifiable cause. The objective of this international working group report on acute exacerbation of Idiopathic Pulmonary Fibrosis was to provide a comprehensive update on the topic. A literature review was conducted to identify all relevant English text publications and abstracts. Evidence-based updates on the epidemiology, etiology, risk factors, prognosis, and management of acute exacerbations of Idiopathic Pulmonary Fibrosis are provided. Finally, to better reflect the current state of knowledge and improve the feasibility of future research into its etiology and treatment, the working group proposes a new conceptual framework for acute respiratory deterioration in Idiopathic Pulmonary Fibrosis and a revised definition and diagnostic criteria for acute exacerbation of Idiopathic Pulmonary Fibrosis.
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delayed access and survival in Idiopathic Pulmonary Fibrosis a cohort study
American Journal of Respiratory and Critical Care Medicine, 2011Co-Authors: Daniela Lamas, Steven M. Kawut, Selim M. Arcasoy, Emilia Bagiella, Nisha Philip, David J LedererAbstract:Rationale: Idiopathic Pulmonary Fibrosis is often initially misdiagnosed. Delays in accessing subspecialty care could lead to worse outcomes among those with Idiopathic Pulmonary Fibrosis. Objectives: To examine the association between delayed access to subspecialty care and survival time in Idiopathic Pulmonary Fibrosis. Methods: We performed a prospective cohort study of 129 adults who met American Thoracic Society criteria for Idiopathic Pulmonary Fibrosis evaluated at a tertiary care center. Delay was defined as the time from the onset of dyspnea to the date of initial evaluation at a tertiary care center. We used competing risk survival methods to examine survival time and time to transplantation. Measurements and Main Results: The mean age was 63 years and 76% were men. The median delay was 2.2 years (interquartile range 1.0–3.8 yr), and the median follow-up time was 1.1 years. Age and lung function at the time of evaluation did not vary by delay. A longer delay was associated with an increased risk of death independent of age, sex, forced vital capacity, third-party payer, and educational attainment (adjusted hazard ratio per doubling of delay was 1.3, 95% confidence interval 1.03 to 1.6). Longer delay was not associated with a lower likelihood of undergoing lung transplantation. Conclusions: Delayed access to a tertiary care center is associated with a higher mortality rate in Idiopathic Pulmonary Fibrosis independent of disease severity. Early referral to a specialty center should be considered for those with known or suspected interstitial lung disease.
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Titrated oxygen requirement and prognostication in Idiopathic Pulmonary Fibrosis
European Respiratory Journal, 2011Co-Authors: Jaime L. Hook, Steven M. Kawut, Selim M. Arcasoy, David Zemmel, Matthew N. Bartels, David J LedererAbstract:The supplemental oxygen flow rate is a common bedside measure of gas exchange impairment. We aimed to determine whether a titrated oxygen requirement predicted mortality in Idiopathic Pulmonary Fibrosis. We examined 104 adults with Idiopathic Pulmonary Fibrosis enrolled in a prospective cohort study and a validation cohort of 151 adults with a variety of interstitial lung diseases. The titrated oxygen requirement was defined as the lowest oxygen flow rate required to maintain an oxyhemoglobin saturation of 96% while standing. Cox proportional hazards models and time-dependent receiver operating characteristic curves were used to examine survival time. A higher titrated oxygen requirement was associated with a greater mortality rate independent of forced vital capacity and six-minute walk test results in Idiopathic Pulmonary Fibrosis (adjusted hazard ratio per 1 L·min−1=1.10, 95% confidence interval 1.01 to 1.20). The titrated oxygen requirement was at least as accurate as Pulmonary function and six-minute walk testing at predicting 1-year mortality. Findings were similar in other interstitial lung diseases. The titrated oxygen requirement is a simple, inexpensive bedside measurement that aids prognostication in Idiopathic Pulmonary Fibrosis.
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Pulmonary hypertension in Idiopathic Pulmonary Fibrosis
Chest, 2007Co-Authors: Nina Patel, David J Lederer, Alain C. Borczuk, Steven M. KawutAbstract:Idiopathic Pulmonary Fibrosis (IPF) is an untreatable diffuse parenchymal lung disease with a median survival of
Toby M Maher - One of the best experts on this subject based on the ideXlab platform.
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Incidence, Prevalence, and Survival of Patients with Idiopathic Pulmonary Fibrosis in the UK.
Advances in Therapy, 2018Co-Authors: Helen Strongman, Imran Kausar, Toby M MaherAbstract:Introduction Recent developments in the care of patients with Idiopathic Pulmonary Fibrosis have the potential to improve survival rates. Population-based estimates of the current disease burden are needed to evaluate the future impact of newly approved therapies. The objective of this study is to describe incidence, prevalence, and survival of Idiopathic Pulmonary Fibrosis patients in the UK.
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acute exacerbation of Idiopathic Pulmonary Fibrosis an international working group report
American Journal of Respiratory and Critical Care Medicine, 2016Co-Authors: Harold R. Collard, David J Lederer, A.u. Wells, Christopher J Ryerson, Tamera J Corte, Gisli Jenkins, Yasuhiro Kondoh, Toby M Maher, Katerina M Antoniou, Juergen BehrAbstract:Acute exacerbation of Idiopathic Pulmonary Fibrosis has been defined as an acute, clinically significant, respiratory deterioration of unidentifiable cause. The objective of this international working group report on acute exacerbation of Idiopathic Pulmonary Fibrosis was to provide a comprehensive update on the topic. A literature review was conducted to identify all relevant English text publications and abstracts. Evidence-based updates on the epidemiology, etiology, risk factors, prognosis, and management of acute exacerbations of Idiopathic Pulmonary Fibrosis are provided. Finally, to better reflect the current state of knowledge and improve the feasibility of future research into its etiology and treatment, the working group proposes a new conceptual framework for acute respiratory deterioration in Idiopathic Pulmonary Fibrosis and a revised definition and diagnostic criteria for acute exacerbation of Idiopathic Pulmonary Fibrosis.
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combination therapy the future of management for Idiopathic Pulmonary Fibrosis
The Lancet Respiratory Medicine, 2014Co-Authors: Wim Wuyts, Toby M Maher, Katerina M Antoniou, Keren Borensztajn, Ulrich Costabel, Vincent Cottin, Bruno Crestani, Jan C Grutters, Venerino Poletti, Luca RicheldiAbstract:Summary Findings from recently published placebo-controlled trials in Idiopathic Pulmonary Fibrosis have established that pirfenidone and nintedanib prevent about 50% of the decline in forced vital capacity typically seen in this disease; future trials are therefore unlikely to use placebo as a control group for ethical reasons. Future clinical assessment will probably include add-on trials in which a new drug is combined with an intervention with established efficacy; this development is in turn likely to herald the use of combination regimens in clinical practice. Personalised medicine (the selection of monotherapies on the basis of individualised biomarker signal) is an intrinsically attractive alternative approach, but is unlikely to be useful in routine management of Idiopathic Pulmonary Fibrosis in the medium-term future because of the complex nature of the disease's pathogenesis. In this Personal View, we review the pleiotropic nature of disease pathogenesis in Idiopathic Pulmonary disease, the use of combination regimens in other selected chronic lung diseases, and the conceptual basis for combination therapies in interstitial lung disorders other than Idiopathic Pulmonary Fibrosis. On the basis of these considerations, and the emergence of data from add-on trials, we believe that the future of management for Idiopathic Pulmonary Fibrosis lies in the development of combination regimens.
Harold R. Collard - One of the best experts on this subject based on the ideXlab platform.
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Idiopathic Pulmonary Fibrosis
Nature reviews. Disease primers, 2017Co-Authors: Fernando J Martinez, Harold R. Collard, Ganesh Raghu, Annie Pardo, Moises Selman, Luca Richeldi, Hiroyuki Taniguchi, Jeffrey J Swigris, A.u. WellsAbstract:Idiopathic Pulmonary Fibrosis (IPF) is a chronic, progressive lung disease characterized by progressive lung scarring and the histological picture of usual interstitial pneumonia (UIP). It is associated with increasing cough and dyspnoea and impaired quality of life. IPF affects ∼3 million people worldwide, with incidence increasing dramatically with age. The diagnostic approach includes the exclusion of other interstitial lung diseases or overlapping conditions and depends on the identification of the UIP pattern, usually with high-resolution CT; lung biopsy might be required in some patients. The UIP pattern is predominantly bilateral, peripheral and with a basal distribution of reticular changes associated with traction bronchiectasis and clusters of subpleural cystic airspaces. The biological processes underlying IPF are thought to reflect an aberrant reparative response to repetitive alveolar epithelial injury in a genetically susceptible ageing individual, although many questions remain on how to define susceptibility. Substantial progress has been made in the understanding of the clinical management of IPF, with the availability of two pharmacotherapeutic agents, pirfenidone and nintedanib, that decrease physiological progression and likely improve progression-free survival. Current efforts are directed at identifying IPF early, potentially relying on combinations of biomarkers that include circulating factors, demographics and imaging data. Idiopathic Pulmonary Fibrosis (IPF) is a chronic lung disease characterized by progressive lung scarring that is thought to occur in a genetically susceptible, ageing individual through aberrant reparative responses to repeated injury to the alveolar epithelium. This Primer summarizes the processes thought to underlie the development of IPF and clinical challenges faced in treating patients with this irreversible condition.
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Idiopathic Pulmonary Fibrosis
The Lancet, 2017Co-Authors: Harold R. Collard, Luca Richeldi, Mark G JonesAbstract:Summary Idiopathic Pulmonary Fibrosis is a prototype of chronic, progressive, and fibrotic lung disease. Healthy tissue is replaced by altered extracellular matrix and alveolar architecture is destroyed, which leads to decreased lung compliance, disrupted gas exchange, and ultimately respiratory failure and death. In less than a decade, understanding of the pathogenesis and management of this disease has been transformed, and two disease-modifying therapies have been approved, worldwide. In this Seminar, we summarise the presentation, pathophysiology, diagnosis, and treatment options available for patients with Idiopathic Pulmonary Fibrosis. This disease has improved understanding of the mechanisms of lung Fibrosis, and offers hope that similar approaches will transform the management of patients with other progressive fibrotic lung diseases.
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acute exacerbation of Idiopathic Pulmonary Fibrosis an international working group report
American Journal of Respiratory and Critical Care Medicine, 2016Co-Authors: Harold R. Collard, David J Lederer, A.u. Wells, Christopher J Ryerson, Tamera J Corte, Gisli Jenkins, Yasuhiro Kondoh, Toby M Maher, Katerina M Antoniou, Juergen BehrAbstract:Acute exacerbation of Idiopathic Pulmonary Fibrosis has been defined as an acute, clinically significant, respiratory deterioration of unidentifiable cause. The objective of this international working group report on acute exacerbation of Idiopathic Pulmonary Fibrosis was to provide a comprehensive update on the topic. A literature review was conducted to identify all relevant English text publications and abstracts. Evidence-based updates on the epidemiology, etiology, risk factors, prognosis, and management of acute exacerbations of Idiopathic Pulmonary Fibrosis are provided. Finally, to better reflect the current state of knowledge and improve the feasibility of future research into its etiology and treatment, the working group proposes a new conceptual framework for acute respiratory deterioration in Idiopathic Pulmonary Fibrosis and a revised definition and diagnostic criteria for acute exacerbation of Idiopathic Pulmonary Fibrosis.
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Models of disease behavior in Idiopathic Pulmonary Fibrosis
BMC Medicine, 2015Co-Authors: Kerri A. Johannson, Harold R. CollardAbstract:Idiopathic Pulmonary Fibrosis is a diffuse parenchymal lung disease of unknown cause. The natural history of disease can vary considerably, making it difficult to predict the clinical trajectory for an individual patient. Accurate prognostication is desirable for clinical management as well as for cohort enrichment in clinical trials of therapeutics. Clinical and biomarker models of disease behavior have been developed to improve prognostication in Idiopathic Pulmonary Fibrosis, with moderate predictive capabilities. Integrated prediction models that combine both clinical and biomarker variables will improve prognostication for patients and improved cohort enrichment strategies for clinical trials. This goal may be best achieved through collaborative patient registries with prospectively collected biological samples that allow for characterization of disease behavior in Idiopathic Pulmonary Fibrosis.
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acute exacerbation of Idiopathic Pulmonary Fibrosis associated with air pollution exposure
European Respiratory Journal, 2014Co-Authors: Kerri A. Johannson, Eric Vittinghoff, John R Balmes, Wonjun Ji, Gilaad G Kaplan, Harold R. CollardAbstract:Acute exacerbations of Idiopathic Pulmonary Fibrosis are associated with high mortality and are of unknown cause. The effect of air pollution on exacerbations of interstitial lung disease is unknown. This study aims to define the association of air pollution exposure with acute exacerbation of Idiopathic Pulmonary Fibrosis. Patients with Idiopathic Pulmonary Fibrosis and corresponding air pollution data were identified from a longitudinal cohort. Air pollution exposures were assigned to each patient for ozone, nitrogen dioxide, particulate matter, sulfur dioxide and carbon monoxide based on geo-coded residential addresses. Cox proportional hazards models were used to estimate the association of air pollution exposures and acute exacerbations. Acute exacerbation was significantly associated with antecedent 6-week increases in mean level, maximum level and number of exceedances above accepted standards of ozone (hazard ratio (HR) 1.57, 95% CI 1.09–2.24; HR 1.42, 95% CI 1.11–1.82; and HR 1.51, 95% CI 1.17–1.94, respectively) and nitrogen dioxide (HR 1.41, 95% CI 1.04–1.91; HR 1.27, 95% CI 1.01–1.59; and HR 1.20, 95% CI 1.10–1.31, respectively). Increased ozone and nitrogen dioxide exposure over the preceding 6 weeks was associated with an increased risk of acute exacerbation of Idiopathic Pulmonary Fibrosis, suggesting that air pollution may contribute to the development of this clinically meaningful event. Acute exacerbation of Idiopathic Pulmonary Fibrosis is associated with increased ozone and nitrogen dioxide exposure
