Journal
AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE
Volume 183, Issue 4, Pages 431-440Publisher
AMER THORACIC SOC
DOI: 10.1164/rccm.201006-0894CI
Keywords
idiopathic pulmonary fibrosis; survival; prognosis; predictors; clinical course
Categories
Funding
- CV Therapeutics
- Genzyme
- Gilead Science
- Nektar
- Actelion
- Amira
- Bayer
- Fibrogen
- NIH
- ASP/Chest Foundation
- Boehringer Ingelheim
- InterMune
- ImmuneWorks
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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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