Update on diffuse parenchymal lung disease

Abstract

In recent years, there have been important advances in the understanding of diffuse parenchymal lung diseases, especially in relation to the idiopathic interstitial pneumonias, including idiopathic pulmonary fibrosis and nonspecific interstitial pneumonia. Among other diffuse parenchymal lung diseases, the topics of particular interest centred on hypersensitivity pneumonia, parenchymal disease associated with connective tissue disease, lymphangioleiomyomatosis and pulmonary hypertension. Insights on disease prevalence, diagnosis, prognostic evaluation, and management for the granulomatous diseases have not been discussed in this review. Epidemiology Idiopathic pulmonary fibrosis (IPF) is a devastating disease, with a 5-yr survival rate of only 20–30% following diagnosis. Pulmonary fibrosis remains a significant health problem whose true incidence and prevalence has been believed to be increasing in recent years. Using a database from the National Center for Health Statistics, Olson et al. [1] clearly show for the first time that the mortality rates have increased from 1992 to 2003. Although these data do not confirm that the prevalence is increasing, they do suggest that there have been improvements in reporting and identification of fibrotic lung diseases. The age-adjusted mortality increased for both sexes, and the authors of this report suggest that this trend will continue. The most common cause of death in people with IPF is progression of their underlying lung disease, but there is evidence that the incidence of other diseases, such as lung cancer, may be increased in people with IPF [2–5]. This is interesting, because such associations may shed light on the aetiology and/or pathogenesis of IPF, and highlight the need for a comprehensive approach to the care of people with IPF [6]. A previous review by Panos et al. [2] highlighted a possible increase in cardiovascular disease in people with IPF, and evidence supporting this observation …