Q What causes idiopathic pulmonary fibrosis (IPF) and how do we manage patients admitted to the intensive care unit (ICU) with this disease?

A Suzanne M. Burns, RN, MSN, RRT, ACNP, CCRN, replies:

IPF is an interstitial lung disease with a progressive and typically fatal outcome. Unfortunately, the median survival rate for this disease is often fewer than 3 years.1 The cause of IPF was previously thought to be inflammation but current information suggests that this disease is instead caused by epithelial injury that results in fibrosis, progressive decline of lung function, and death.2,4 

IPF is one of a group of interstitial diseases, as defined by the American Thoracic Society/European Respiratory Society international multidisciplinary consensus committee panel.5 The committee stated the importance of identifying or excluding IPF as early as possible because of its pattern of progressive decline and bad prognosis, and further suggested...

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