Time From First Imaging Showing Pulmonary Fibrosis to Diagnosis of Idiopathic Pulmonary Fibrosis: Data From the IPF-PRO Registry

deAndrade, Joao; Conoscenti, Craig; Flaherty, Kevin R.; Holtze, Colin; Lancaster, Lisa; Neely, Megan L.; Snyder, Laurie D.; Yow, Eric; Whelan, Timothy

doi:10.1016/j.chest.2017.08.472

Cited by 1 publication

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“…What is more interesting is the observation that relative pattern stability may be seen in some over many years of follow-up, making the supposition of so-called earlier radiologic disease based on the absence of honeycombing or more extensive fibrotic changes less reliable for delineating how long disease has been present. Delay in the recognition of IPF even when fibrosis is present was recently demonstrated in a large multicenter registry of IPF patients, with a quarter being diagnosed >13 months after an initial scan suggesting fibrotic disease 28…”

Section: Introductionmentioning

confidence: 99%

Obstacles to early treatment of idiopathic pulmonary fibrosis: current perspectives

Moua

Ryu

2019

TCRM

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Idiopathic pulmonary fibrosis is a progressive and fatal fibrotic lung disease which has seen new opportunity for drug treatment in the last several years with the approval of nintedanib and pirfenidone, two antifibrotic agents aimed at slowing decline in lung function as defined by FVC on pulmonary function testing. Despite these promising effects, delays in drug initiation have been reported undermining the premise that earlier drug initiation may sustain lung function and prolong survival. This review explores obstacles to earlier treatment, inclusive of defining so-called early idiopathic pulmonary fibrosis, difficulties in achieving a confident diagnosis in that setting, and uncertainties regarding drug-related benefits among specific patient subgroups such as those with no symptoms or advanced disease at presentation. Goals of therapy balanced with the burdens associated with antifibrotic drug therapy are negotiated on an individual basis. We review the evidence for and against earlier initiation of antifibrotic drug therapy along with its role in patient-centered outcomes.

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Section: Introductionmentioning

confidence: 99%