Interstitial lung disease · How IPF progresses
The clinical course of patients with idiopathic pulmonary fibrosis
| Design | Prospective longitudinal analysis within a randomised controlled trial |
|---|---|
| Treatment | Serial physiological monitoring |
| Control | None — observational analysis of trial participants |
| Population | Patients with mild to moderate idiopathic pulmonary fibrosis enrolled in a randomised trial |
| Follow-up | Duration of the parent trial |
| Primary endpoint | Change in physiological variables and clinical events over time |
| Result | Physiological variables changed minimally during the study, yet 23% of patients required hospitalisation for a respiratory disorder and 21% died. IPF was the primary cause of death in 89% of those who died, and an apparent acute clinical deterioration preceded death in 47% of them. |
| Secondary endpoints | Cause of death, frequency of acute deterioration |
Recognition of the common occurrence of acute fatal deterioration in patients with mild to moderate IPF has important implications for monitoring patients and supports early referral for lung transplantation.
The trial authors, in the published abstract
The observation that reshaped how IPF is followed up: stable spirometry does not mean a stable patient, because death is frequently preceded by acute deterioration rather than gradual decline — in 47% of deaths here. It is the reason transplant referral is made early rather than when the numbers fall, and the reason acute exacerbation became a defined entity.
Martinez FJ, Safrin S, Weycker D, et al. The clinical course of patients with idiopathic pulmonary fibrosis. Ann Intern Med 2005 Jun 21;142(12 Pt 1):963-7.