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A Journal on Diseases of the Respiratory System
Official Journal of the Italian Society of Thoracic Endoscopy
Indexed/Abstracted in: EMBASE, Scopus
Minerva Pneumologica 2008 March;47(1):17-29
Idiopathic pulmonary fibrosis: natural history and current approaches to therapy
Poston J. T., Noth I.
Section of Pulmonary and Critical Care Medicine Department of Medicine Biological Sciences Division The University of Chicago, Chicago, IL, USA
Idiopathic pulmonary fibrosis (IPF) is the most common idiopathic interstitial pneumonia, and the incidence and prevalence of this devastating disease is increasing. Despite its description decades ago, only recently has IPF been rigorously defined by clinical, radiographic, and histopathologic criteria. Strict disease characterization has allowed accurate observation and enhanced the understanding of the natural history of IPF. While chronic progressive fibrosis remains a hallmark of IPF, acute exacerbation with a precipitous decline in lung function over days to weeks is increasingly recognized, and contributes greatly to morbidity and mortality. IPF patients also have high morbidity and mortality attributable to co-morbid conditions such as coronary artery disease, gastroesophogeal reflux disease, lung cancer, and pulmonary arterial hypertension. Management of IPF is extremely challenging. Clinicians struggle to offer their patients slowed disease progression, relief of symptoms, improved quality of life, and guidance through an often fatal disease process. Medical therapy for IPF has been disappointing to date, and no therapy has been clearly shown to improve survivorship. However, the last decade has seen a resurgence of clinical trials of novel therapies, many of which are ongoing. Lung transplantation is the only therapy to demonstrate a survival benefit, and early referral for transplant evaluation should be considered in all patients with IPF.