Idiopathic Pulmonary Fibrosis: Epidemiology, Clinical Features, Prognosis, and Management

Lynch, Joseph P.; Huynh, Richard; Fishbein, Michael C.; Saggar, Rajan; Belperio, John A.; Weigt, S. Sam

doi:10.1055/s-0036-1582011

Cited by 27 publications

(12 citation statements)

References 306 publications

(623 reference statements)

Supporting

Mentioning

Contrasting

Order By: Relevance

“…CPI was developed in a British study and has the advantage of relying on PFT data to predict IPF prognosis. Additionally, CPI was developed by considering the severity of emphysema, which could lead to overestimation of lung function 19 . However, CPI also has disadvantages: it does not consider clinical data such as age, gender, smoking history, presence of desaturation, or 6-minute walk distance.…”

Section: Discussionmentioning

confidence: 99%

Comparison of CPI and GAP models in patients with idiopathic pulmonary fibrosis: a nationwide cohort study

Lee

Kim

et al. 2018

Sci Rep

View full text Add to dashboard Cite

The clinical course of idiopathic pulmonary fibrosis (IPF) is difficult to predict, partly owing to its heterogeneity. Composite physiologic index (CPI) and gender-age-physiology (GAP) models are easy-to-use predictors of IPF progression. This study aimed to compare the predictive values of these two models. From 2003 to 2007, the Korean Interstitial Lung Disease (ILD) Study Group surveyed ILD patients using the 2002 ATS/ERS criteria. A total of 832 patients with IPF were enrolled in this study. CPI was calculated as follows: 91.0 − (0.65 × %DLCO) − [0.53 × %FVC + [0.34 × %FEV1. GAP stage was calculated based on gender (0–1 points), age (0–2 points), and two physiologic lung function parameters (0–5 points). The two models had similar significant predictive values for patients with IPF (p < 0.001). The area under the curve (AUC) was higher for CPI than GAP for prediction of 1-, 2-, and 3-year mortality in this study. The AUC was higher for surgically diagnosed IPF patients than for clinically diagnosed patients. However, neither CPI nor GAP yielded good predictions of outcomes; the AUC was approximately 0.61~0.65. Although both CPI and GAP stage are significantly useful predictors for IPF, they have limited capability to accurately predict outcomes.

show abstract

Section: Discussionmentioning

confidence: 99%

Comparison of CPI and GAP models in patients with idiopathic pulmonary fibrosis: a nationwide cohort study

Lee

Kim

et al. 2018

Sci Rep

View full text Add to dashboard Cite

show abstract

“…Patients with IPF have a median survival of only 2–5 years from diagnosis. The prevalence of IPF in the United States is ~40 cases per 100,000, and accounts for over 16,000 deaths per year (Lynch et al, 2016 ; Raimundo et al, 2016 ). The health burden of IPF in other developed and developing countries is no less significant.…”

Section: Fibrotic Disease Of the Lungsmentioning

confidence: 99%

The Processes and Mechanisms of Cardiac and Pulmonary Fibrosis

et al. 2017

View full text Add to dashboard Cite

Fibrosis is the formation of fibrous connective tissue in response to injury. It is characterized by the accumulation of extracellular matrix components, particularly collagen, at the site of injury. Fibrosis is an adaptive response that is a vital component of wound healing and tissue repair. However, its continued activation is highly detrimental and a common final pathway of numerous disease states including cardiovascular and respiratory disease. Worldwide, fibrotic diseases cause over 800,000 deaths per year, accounting for ~45% of total deaths. With an aging population, the incidence of fibrotic disease and subsequently the number of fibrosis-related deaths will rise further. Although, fibrosis is a well-recognized cause of morbidity and mortality in a range of disease states, there are currently no viable therapies to reverse the effects of chronic fibrosis. Numerous predisposing factors contribute to the development of fibrosis. Biological aging in particular, interferes with repair of damaged tissue, accelerating the transition to pathological remodeling, rather than a process of resolution and regeneration. When fibrosis progresses in an uncontrolled manner, it results in the irreversible stiffening of the affected tissue, which can lead to organ malfunction and death. Further investigation into the mechanisms of fibrosis is necessary to elucidate novel, much needed, therapeutic targets. Fibrosis of the heart and lung make up a significant proportion of fibrosis-related deaths. It has long been established that the heart and lung are functionally and geographically linked when it comes to health and disease, and thus exploring the processes and mechanisms that contribute to fibrosis of each organ, the focus of this review, may help to highlight potential avenues of therapeutic investigation.

show abstract

“…Idiopathic pulmonary fibrosis (IPF) is the most frequent form of fibrotic interstitial lung diseases (ILDs), which presents a variable outcome and is generally fatal within 2–4 years from diagnosis [ 1 ]. Histologically, IPF is characterized by an usual interstitial pneumonia (UIP) pattern; where honeycombing areas with collagen deposition and fibroblast foci are situated next to structurally preserved areas [ 2 ]. Advances in the knowledge of the pathogenesis have been focused in integrating the different factors involved in IPF with the purpose of improving the diagnosis, management and treatment [ 3 ].…”

Section: Introductionmentioning

confidence: 99%

Serum AGE/RAGEs as potential biomarker in idiopathic pulmonary fibrosis

et al. 2018

View full text Add to dashboard Cite

BackgroundThe soluble receptor for advanced glycation end-products (sRAGE) has been suggested that it acts as a decoy for capturing advanced glycation end-products (AGEs) and inhibits the activation of the oxidative stress and apoptotic pathways. Lung AGEs/sRAGE is increased in idiopathic pulmonary fibrosis (IPF). The objective of the study was to evaluate the AGEs and sRAGE levels in serum as a potential biomarker in IPF.MethodsSerum samples were collected from adult patients: 62 IPF, 22 chronic hypersensitivity pneumonitis (cHP), 20 fibrotic non-specific interstitial pneumonia (fNSIP); and 12 healthy controls. In addition, 23 IPF patients were re-evaluated after 3-year follow-up period. Epidemiological and clinical features were recorded: age, sex, smoking habits, and lung function. AGEs and sRAGE were evaluated by ELISA, and the results were correlated with pulmonary functional test values.ResultsIPF and cHP groups presented a significant increase of AGE/sRAGE serum concentration compared with fNSIP patients. Moreover, an inverse correlation between AGEs and sRAGE levels were found in IPF, and serum sRAGE at diagnosis correlated with FVC and DLCO values. Additionally, changes in serum AGEs and sRAGE correlated with % change of FVC, DLCO and TLC during the follow-up. sRAGE levels below 428.25 pg/ml evolved poor survival rates.ConclusionsThese findings demonstrate that the increase of AGE/sRAGE ratio is higher in IPF, although the levels were close to cHP. AGE/sRAGE increase correlates with respiratory functional progression. Furthermore, the concentration of sRAGE in blood stream at diagnosis and follow-up could be considered as a potential prognostic biomarker.Electronic supplementary materialThe online version of this article (10.1186/s12931-018-0924-7) contains supplementary material, which is available to authorized users.

show abstract

Idiopathic Pulmonary Fibrosis: Epidemiology, Clinical Features, Prognosis, and Management

Cited by 27 publications

References 306 publications

Comparison of CPI and GAP models in patients with idiopathic pulmonary fibrosis: a nationwide cohort study

Comparison of CPI and GAP models in patients with idiopathic pulmonary fibrosis: a nationwide cohort study

The Processes and Mechanisms of Cardiac and Pulmonary Fibrosis

Serum AGE/RAGEs as potential biomarker in idiopathic pulmonary fibrosis

Contact Info

Product

Resources

About