Clinical Course and Prediction of Survival in Idiopathic Pulmonary Fibrosis

Abstract

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.

Staging of idiopathic pulmonary fibrosis: past, present and future

“…As mentioned previously, a number of studies have shown that selected features of IPF, which are commonly observed in clinical practice, are associated with an increased risk of mortality [3]. Incorporation of these factors into a multidimensional staging model may enhance the validity and clinical applicability of staging in IPF, but requires validation.…”

“…However, the clinical course of disease can vary considerably, from slow progression over many years to acute exacerbation, rapid loss of lung function and early death [2][3][4]. A number of studies have shown that selected features commonly observed in clinical practice are associated with an increased risk of mortality.…”

“…A number of studies have shown that selected features commonly observed in clinical practice are associated with an increased risk of mortality. These include clinical history and physical examination, as well as radiographic, physiological, pathological and biomarker findings [3]. However, there is no widely accepted, standardised method to combine such predictors in order to define prognosis or stage of disease.…”

Validation of the COPD Assessment Test (CAT) in patients with idiopathic pulmonary fibrosis

“…Disease progress is often slow and occurs gradually over a few years. However, some patients experience an accelerated decline, with acute respiratory worsening and exacerbations [12,13]. It is the type of lung fibrosis with the highest mortality rate, as the median length of survival is 3–5 years [8–11,14], which means that IPF has a poorer prognosis than a number of malignant diseases [7,15].…”

Current approaches to the diagnosis and treatment of idiopathic pulmonary fibrosis in Europe: the AIR survey

“…The clinical course of IPF is largely unpredictable and, despite extensive research, the precise molecular and cellular mechanisms underlying its pathogenesis remain to be elucidated [1][2][3][4][5]. The classification, nomenclature, diagnosis and treatment of IPF have undergone numerous changes in the past.…”