Clinical course and prediction of survival in idiopathic pulmonary fibrosis

B Ley, HR Collard, TE King Jr - American journal of respiratory and …, 2011 - atsjournals.org
B Ley, HR Collard, TE King Jr
American journal of respiratory and critical care medicine, 2011atsjournals.org
Idiopathic pulmonary fibrosis (IPF) is a progressive, life-threatening, interstitial lung disease
of unknown etiology. The median survival of patients with IPF is only 2 to 3 years, yet some
patients live much longer. Respiratory failure resulting from disease progression is the most
frequent cause of death. To date we have limited information as to predictors of mortality in
patients with IPF, and research in this area has failed to yield prediction models that can be
reliably used in clinical practice to predict individual risk of mortality. The goal of this concise …
Idiopathic pulmonary fibrosis (IPF) is a progressive, life-threatening, interstitial lung disease of unknown etiology. The median survival of patients with IPF is only 2 to 3 years, yet some patients live much longer. Respiratory failure resulting from disease progression is the most frequent cause of death. To date we have limited information as to predictors of mortality in patients with IPF, and research in this area has failed to yield prediction models that can be reliably used in clinical practice to predict individual risk of mortality. The goal of this concise clinical review is to examine and summarize the current data on the clinical course, individual predictors of survival, and proposed clinical prediction models in IPF. Finally, we will discuss challenges and future directions related to predicting survival in IPF.
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