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Prognosis and monitoring of idiopathic pulmonary fibrosis

Authors
Christopher Ryerson, MD
Brett Ley, MD
Section Editor
Kevin R Flaherty, MD, MS
Deputy Editor
Helen Hollingsworth, MD

INTRODUCTION

Idiopathic pulmonary fibrosis (IPF)/usual interstitial pneumonia (UIP), previously known as cryptogenic fibrosing alveolitis (CFA) in Europe, is the most common type of idiopathic interstitial pneumonia (IIP). The other IIPs include idiopathic nonspecific interstitial pneumonia (NSIP), desquamative interstitial pneumonia (DIP), respiratory bronchiolitis-associated interstitial lung disease (RB-ILD), acute interstitial pneumonia (AIP), lymphocytic interstitial pneumonia (LIP), and cryptogenic organizing pneumonia (COP) [1].

An American Thoracic Society (ATS)/European Respiratory Society (ERS) consensus statement defines IPF as a spontaneously occurring (idiopathic) specific form of chronic fibrosing interstitial pneumonia limited to the lung and associated with a pattern of UIP on high resolution computed tomography (HRCT) or surgical (thoracoscopic or open) lung biopsy [2]. The importance of careful monitoring and accurate prognostication has increased with the availability of new medications to treat IPF and expanding eligibility for lung transplantation.

The prognosis and monitoring of IPF will be reviewed here. The clinical manifestations, diagnosis, and treatment of IPF are discussed separately. (See "Clinical manifestations and diagnosis of idiopathic pulmonary fibrosis" and "Treatment of idiopathic pulmonary fibrosis" and "Acute exacerbations of idiopathic pulmonary fibrosis".)

PROGNOSIS

The natural history of IPF is most often described as one of insidious decline in lung function resulting in progression to respiratory failure and death on average within about four years after the initial diagnosis [2]. However, there is great variability in disease course among individual patients with IPF, and survival is influenced by several factors including variable progression rates, occurrence of acute exacerbations, and comorbid disease (figure 1) [3].

Mortality — The median survival of IPF has been reported to range from two to five years [4,5]. This estimate reflects the range of average life expectancies observed in cohorts of IPF patients, rather than the limits of an individual patient's life expectancy. This nuance is important, as the actual range of survival of individual IPF patients is quite broad, with up to 20 to 25 percent of patients living beyond 10 years [4].

                   
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Literature review current through: Nov 2017. | This topic last updated: Nov 21, 2017.
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