Neglected evidence in idiopathic pulmonary fibrosis and the importance of early diagnosis and treatment

• Published in: European respiratory review Vol. 23; no. 131; pp. 106 - 110
• Main Authors: Cottin, Vincent, Richeldi, Luca
• Format: Journal Article
• Language: English
• Published: England European Respiratory Society 01.03.2014
• Subjects: Early Diagnosis; Human health and pathology; Humans; Idiopathic Pulmonary Fibrosis - diagnosis; Idiopathic Pulmonary Fibrosis - mortality; Idiopathic Pulmonary Fibrosis - therapy; Life Sciences; Patient Care Team; Predictive Value of Tests; Pulmonology and respiratory tract; Reviews; Time Factors; Treatment Outcome; idiopathic Pulmonary Fibrosis/diagnosis/mortality/therapy; traitement; treatment; maladie pulmonaire; patient Care Team; predictive value of tests; treatment outcome; fibrose pulmonaire; early diagnosis; time factors; respiratory disease; pulmonary fibrosis; tomodensitométrie; diagnostic; humans; respiratory system diseases; maladie respiratoire
• ISSN: 0905-9180; 1600-0617
• DOI: 10.1183/09059180.00008613

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.