Staging of idiopathic pulmonary fibrosis: past, present and future

Abstract

Idiopathic pulmonary fibrosis (IPF) is traditionally staged with terms such as ''mild'', ''severe'', ''early'' and ''advanced'' based on pulmonary function tests. This approach allows physicians to monitor disease progression and advise patients and their families. However, it is not known if the stages of this model reflect distinct biological or clinical phenotypes and the therapeutic and prognostic value of this system is limited.Novel methods of IPF staging have recently been developed. The GAP model includes four baseline variables that were found to be predictive of outcome, as identified by logistic regression. These factors are: gender (G), age (A) and two lung physiology variables (P) (forced vital capacity and diffusing capacity of the lung for carbon monoxide). The clinical utility and accuracy of staging models may be further improved in the future by the integration of dynamic parameters that can be measured over time, as well as biological data from biomarkers which may be able to directly measure disease activity. The development of an evidence-based, multidimensional IPF staging model that builds on the current staging approaches to IPF is an important objective for improving the management of IPF. @ERSpublications Development of an evidence-based, multidimensional IPF staging model is important for improving IPF management

A Real-Life Multicenter National Study on Nintedanib in Severe Idiopathic Pulmonary Fibrosis

“…The mechanism of action that influences the variations of absolute and D LCO % in this series still needs to be explored, but the trend gives an important and positive signal. The lung diffusion capacity is indeed considered, together with FVC, as one of the most important prognostic factors for IPF [26-31]. This result is even more significant given that it was recorded in a real-life study that more closely reflects the real complexity of sick patients than the selected populations in clinical trials do.…”

3D pulmospheres serve as a personalized and predictive multicellular model for assessment of antifibrotic drugs

“…More effective therapies are urgently needed, but one of the major limitations in the development of drug therapies in IPF is the lack of reliable preclinical models of the disease. Current animal models, primarily those in rodents, have been disappointing (4), and many promising drugs have failed to demonstrate efficacy in human randomized clinical trials. An ideal preclinical model will need to recapitulate the multitude of factors responsible for the disease phenotype or endotype (1,5,6).…”

Evaluating disease severity in idiopathic pulmonary fibrosis

“…This model performs equally well when substituting DLCO for visually assessed CT fibrosis extent (CT-GAP score) [8]. A strategy to improve the sensitivity of future composite measures would be to integrate independent predictors of disease progression, such as serial change in FVC, with decline in 6MWD and/or serial change in HRCT fibrosis score [18,92]. Recent advances in our understanding of the complex pathogenesis of IPF have increased endeavours aimed at identifying and validating specific biological determinants that hold greater promise for diagnostic and prognostic applicability.…”