title: Neglected evidence in idiopathic pulmonary fibrosis and the importance of early diagnosis and treatment.
abstract: In idiopathic pulmonary fibrosis (IPF), some facts or concepts based on substantial evidence, whilst implicit for learned subspecialists, have previously been neglected and/or not explicitly formulated or made accessible to a wider audience. IPF is strongly associated with cigarette smoking and is predominantly a disease of ageing. However, its cause(s) remain elusive and, thus, it is one of the most challenging diseases for the development of novel effective and safe therapies. With the approval of pirfenidone for patients with mild-to-moderate IPF, an earlier diagnosis of IPF is a prerequisite for earlier treatment and, potentially, improvement of the long-term clinical outcome of this progressive and ultimately fatal disease. An earlier diagnosis may be achieved in IPF by promoting thin-slice chest high-resolution computed tomography screening of interstitial lung disease as a "by-product" of large-scale lung cancer screening strategies in smokers, but other techniques, which have been neglected in the past, are now available. Lung auscultation and early identification of "velcro" crackles has been proposed as a key component of early diagnosis of IPF. An ongoing study is exploring correlations between lung sounds on auscultation obtained using electronic stethoscopes and high-resolution computed tomography patterns.
authors.affiliation: Hospices Civils de Lyon, Hôpital Louis Pradel, Service de pneumologie - Centre de référence national des maladies pulmonaires rares, Lyon, and 2 Université de Lyon, Université Claude Bernard Lyon 1, INRA, UMR754 INRA-Vetagrosup EPHE IFR 128, Lyon, France. 3 Academic Unit of Clinical and Experimental Sciences, Faculty of Medicine, University of Southampton, Southampton, and 4 Southampton NIHR Respiratory Biomedical Research Unit, University Hospital Southampton, Southampton, UK.
authors.forename: Vincent
authors.initials: V
authors.lastname: Cottin
authors.forename: Luca
authors.initials: L
authors.lastname: Richeldi
mesh_terms: D042241:Early Diagnosis; D006801:Humans; D054990:Idiopathic Pulmonary Fibrosis / Q000175:diagnosis* / Q000401:mortality / Q000628:therapy*; D010348:Patient Care Team; D011237:Predictive Value of Tests; D013997:Time Factors; D016896:Treatment Outcome