TY - JOUR T1 - Elucidating the causes and examining the latest clinical findings in pulmonary fibrosis JF - European Respiratory Review JO - EUROPEAN RESPIRATORY REVIEW SP - 105 LP - 107 DO - 10.1183/09059180.00010901 VL - 17 IS - 109 AU - T. E. King, Jr AU - R. M. du Bois Y1 - 2008/12/01 UR - http://err.ersjournals.com/content/17/109/105.abstract N2 - Idiopathic pulmonary fibrosis (IPF) is a devastating disease, with a 5-yr survival rate of only 20–30% following diagnosis [1]. Patients with IPF may experience a rapidly progressive decline in lung function from onset or a slowly progressive course of illness, during which the development of acute exacerbations can lead to rapid respiratory failure and early death [2]. Prognosis is extremely poor and no pharmacological agent improves survival in IPF. Immunosuppressive treatment regimens have been used in the treatment of IPF; however, there is little evidence to support their efficacy and safety. There has been growing interest in the diagnosis and prevention of acute exacerbations in patients with IPF [3]. It has been shown that acute exacerbations are quite prevalent among patients with IPF, that they occur at any stage of the illness and that they are frequently fatal events. Consequently, this has created additional concern that treatment should be initiated at the earliest sign of impairment. The present issue of European Respiratory Review reports the proceedings of an International Scientific Advisory Board Meeting on “Elucidating the causes and examining the latest clinical findings in pulmonary fibrosis”, which was held in London (UK), on November 16–18, 2007. The first two articles explore the current understanding in pulmonary fibrosis and are followed by six articles that analyse the mechanisms of disease in IPF and challenge the once prevalent view of IPF as a disease of unremitting, chronic inflammation. The final article focuses on the potential genetic associations with the disease. In the first article, the authors discuss the advances in the classification of the idiopathic interstitial pneumonias (IIPs), in which IPF and its corresponding histopathological pattern of usual interstitial pneumonia (UIP) are distinguished from six non-IPF (IIP) subtypes [4]. This distinction is important because … ER -