Chest
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Idiopathic pulmonary fibrosis (IPF) is a progressive, fatal disease. Until recently, the standard therapy for this disease has been essentially supportive, with the exception of a minority of patients who were eligible for lung transplantation. The development pathway for novel medications for IPF has been complicated. ⋯ In October 2014, these two drugs became the first agents to be approved by the US Food and Drug Administration for the treatment of IPF. (Pirfenidone had already been approved in several countries outside the United States.) In November 2014, the European Medicines Agency approved the use of nintedanib for IPF. The landscape for management of IPF has markedly changed with the advent of approved therapeutic options for IPF. In this article, we review the strategies that are being used to increase the likelihood of success in clinical development programs of novel disease-modifying agents in IPF.
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In COPD, a decreased inspiratory capacity to total lung capacity ratio (IC/TLC) is associated with dynamic hyperinflation and poor exercise capacity. The association with upper-extremity force measured by handgrip strength (HGS) and 6-min walk distance (6MWD) has not been previously described. We hypothesized that IC/TLC affects muscle strength in the upper and lower extremities, affecting HGS and 6MWD. ⋯ HGS and 6MWD are reduced in patients with COPD, particularly in those with hyperinflation and evidence of longitudinal deterioration not seen in control subjects. This finding suggests that resting hyperinflation may exert a detrimental effect on cardiac function and plays a role in reduced exercise performance in patients with COPD.