What can biomarkers tell us about the pathogenesis of acute exacerbations of idiopathic pulmonary fibrosis?

摘要

Idiopathic pulmonary fibrosis (IPF) is a chronic, fibrosing interstitial lung disease of unknown etiology. An estimated 200,000 Americans are presently living with IPF, and the prevalence appears to be increasing. Mean survival from the time of diagnosis is only 2-3 years, and unfortunately there is no effective therapy. Historically, the clinical course of IPF was considered to be one of relentless, progressive deterioration culminating in respiratory failure and death. More recent data demonstrate that many IPF patients have prolonged periods of clinical stability punctuated by episodes of acute respiratory decline (6). These abrupt declines result in death in up to one-half of patients with IPF. When no cause for the decline can be identified, these episodes are termed an acute exacerbation of IPF (AE-IPF). Similar exacerbations occur in other fibrosing lung diseases (3). The pathophysiology of AE-IPF is unknown, and there is no known therapy.