Neglected evidence in idiopathic pulmonary fibrosis: from history to earlier diagnosis

Abstract

This perspective highlights some evidence that has hitherto been neglected, especially because it may not have been sufficiently explicated in the clinical respiratory medicine literature. Idiopathic pulmonary fibrosis (IPF) has appeared only in the second half of the 20th century and, like lung cancer and chronic obstructive pulmonary disease, may be a direct consequence of the cigarette smoking epidemic. It is a disease of lung ageing, with most affected patients being .70 years of age. The relationship between lung ageing and pulmonary fibrosis is further illustrated in the bleomycin mouse model, in which older males develop more fibrosis than young female mice.Earlier diagnosis of IPF is a prerequisite for significant progress to be made in the long-term outcome and prognosis. We consider that only two different yet complementary and realistic approaches could lead to earlier diagnosis of IPF and possibly to allowing more efficient disease management: 1) investigating any patients with early Velcro crackles at lung auscultation through proactive education of, and commitment from, primary care physicians; and 2) using current large-scale lung cancer screening strategies with lowdose high-resolution computed tomography in smokers for the detection of subclinical interstitial lung disease and especially early IPF. @ERSpublications Only two approaches could lead to earlier diagnosis of IPF and more efficient disease management

Practical Issues and Challenges in Idiopathic Pulmonary Fibrosis

“…Although most hot topics in IPF will be reviewed in this series, some other important aspects can be found elsewhere, including the development of biomarkers [42,43], the impact of comorbidities including emphysema (syndrome of combined pulmonary fibrosis and emphysema) [44], gastroesophageal reflux [45,46], lung cancer [47], sleep apnea, cardiovascular disease and diabetes mellitus [48], the increasing role of genetics in the pathogenesis and management of IPF [49,50], comprehensive management and supportive care [51,52], or possible approaches for earlier diagnosis and treatment [53]. We hope readers will enjoy this series on IPF, and that it will translate into better care for IPF patients.…”

Insights into idiopathic pulmonary fibrosis in the real world

“…Epidemiological studies have showed that IPF is more common than previously appreciated in the elderly population [3], with rising prevalence and mortality [4,5]. The role of tobacco smoking is better appreciated [6]. The underlying genetic predisposition to IPF is progressively unveiled [7].…”

The changing treatment landscape in idiopathic pulmonary fibrosis

“…IPF predominantly affects elderly male smokers/ex-smokers with a median age at diagnosis of 65-70 years [3]. Although it is a rare disease, with an estimated prevalence range of 1-23 cases per 100 000 European persons [4][5][6][7][8][9], IPF represents a considerable challenge for clinicians.…”