TY - JOUR T1 - Velcro crackles: the key for early diagnosis of idiopathic pulmonary fibrosis? JF - European Respiratory Journal JO - Eur Respir J SP - 519 LP - 521 DO - 10.1183/09031936.00001612 VL - 40 IS - 3 AU - Vincent Cottin AU - Jean-François Cordier Y1 - 2012/09/01 UR - http://erj.ersjournals.com/content/40/3/519.abstract N2 - Idiopathic pulmonary fibrosis (IPF), affecting individuals mostly aged 60–70 yrs, is the most common and the most severe of idiopathic interstitial pneumonias, with a median survival of only 3 yrs. The incidence and mortality of IPF has risen dramatically in the last decade [1]. Furthermore, IPF is one of the most challenging diseases for therapy developments, due to its complex and unresolved pathogenic processes, the possible inadequate drug diffusion in fibrotic lungs, and difficulties in designing clinical trials [2]. Up until now, no treatment could prevent the relentless progression of IPF to end-stage lung and ensuing death. However, drug therapy in IPF has recently regained hope with the publication of clinical trials using pirfenidone or the triple tyrosine kinase inhibitor BIBF 1120 that demonstrated an effect in limiting the decline in lung function [3–5].Given these therapeutic advances, diagnosing IPF at an earlier stage becomes a relevant healthcare issue. Indeed, pirfenidone has been approved in Japan and in the European Union, and is indicated in mild-to-moderate IPF, which in the absence of a consensus definition may correspond to patients with forced vital capacity (FVC) >50% predicted and diffusing capacity of the lung for carbon monoxide (DL,CO) >35% pred (i.e. pulmonary function inclusion criteria for the recent phase III pirfenidone trials) [3]. Similarly, in a phase II study, BIBF 1120 has demonstrated a trend toward a reduction in lung function decline in patients with FVC >50% pred and DL,CO >30% pred [4], and is currently evaluated in a phase III study (clinical trial identifier number NCT01335464). The criteria chosen for these trials … ER -